Degenerative disorders

PARKINSON’S DISEASE
DEFINITION:
It is a chronic, progressive disease of the nervous system
characterized by cardinal features of rigidity, bradykinesia, tremor and
postural instability.
ETIOLOGY:
Idiopathic
Viral inflammation
Trauma
Stroke
Smocking
alcohol
Poison intake
Genetic
Drug induced
Environmental toxins
PATHOLOGY:
Etiological cause
↓
Affects the substantia nigra
↓ ↓↓
Destuction of dopamine producing neurons within the basal ganglia
↓ ↓
Reduces the amount of available straital dopamine
(inhibitory effects)
↓
There is increase in acetylcholine (excitatory effects )
↓
Excitatory activity of Ach is inadequately balanced
↓
Difficulty in controlling and initiating voluntary movements

CLINICAL FEATURE:
Rigidity
Bradykinesia
Tremor
Postural instability
Hesitation
Masked face
Fatigue
Contracture
Deformities
Impaired gait
Kyphotic posture
Increase fall
Akathisia
Dysarthria
Dhysphagia
Decreased arm swing
Dementia
Dyspnea
Visuosaptial deficits
Depression
 Bradyphrenia
Excessive sweating
Constipation
Low bp
Impaired respiratory function
Urinary bladder dysfunction

DIAGNOSIS:
Neurological examination
Ct scan
MRI
Autopsy of brain
Blood test

MANAGEMENT:
Medical management
Mono amine oxidase inhibitors
Levodopa
Carbidopa
Anticholineric agents
Anti histamine
Dopamine agonist
Nutritional management
Low protein diet
High fiber
Hydrated fruits
Surgical management
Thalamatomy
Pallidatomy
Fetal tissue implantation
Deep brain stimulation
Neural transplantation
 Dementia
DEFINITION:

RISK FACTORS FOR DEMENTIA

• Age
• Sex (male)
• Genetic
• Over weight
• Drug
• Smoking
• Alcohol
• Blood pressure
• Blood sugar level
• Cholesterol
• Depression
• Stress
• Heart diseases
• Diabetes
• Vascular diseases
• Head injury
• Inflammatory stress
• Tumor
• HIV infection
• Some immunological disorders
• Poor education
 • Low physical activity
• Poor nutrition
CLASSIFICATION -BASED ON CAUSE
1)ALZHEIMER’S DISEASE
2)CEREBRO VASCULAR DISEASES
3)NEURO DEGENERATIVE
4)INFECTIONS
5)NORMAL PRESSURE HYDROCEPHALUS
6)NUTRITIONAL
7)METABOLIC
8)CHRONIC INFLAMMATORY
9)TRAUMA
10)TUMOR
ALZHEIMER’S DISASE:
Most common type of dementia; accounts for an estimated 60 to
80 percent of cases.
Symptoms:
Difficulty remembering names and recent events
apathy and depression.
impaired judgment, disorientation, confusion, behavior changes
and difficulty speaking, swallowing and walking.
CEREBROVASCULAR ACCIDENT:
Previously known as multi-infarct or post-stroke dementia,
vascular dementia is the second most common cause of dementia
after Alzheimer's disease.(20% Of all cases)
Symptoms:
Impaired judgment or ability to plan steps needed to complete a
task
Occurs because of brain injuries such as microscopic bleeding
and blood vessel blockage.
thinking and physical functioning are affected
NEURO DEGENERATIVE DEMENTIA
Pick’s disease
presents with impairment of recent memory associated with
entorhinal cortex and hippocampal dysfunction, Pick disease
typically affects the frontal and/or anterolateral temporal lobes.
A TYPE OF FRONTO-TEMPORAL DEMENTIA.
Emotional & behavioral changes, mutism, aphasia,
echolalia(repeating whatever spoken to them), rigidity, apraxia,
weakness, memory loss

CREUTZFELD-JACOB DISEASE
CJD is the most common human form of a group of rare, fatal
brain disorders affecting people and certain other mammals.
Variant CJD (“mad cow disease”) occurs in cattle, and has been
transmitted to people under certain circumstances.
Symptoms:
Rapidly fatal disorder that impairs memory and coordination and
causes behavior changes.
Normal Pressure Hydrocephalus
Symptoms:
Symptoms include difficulty walking, memory loss and inability to
control urination

Mixed Dementia
In mixed dementia abnormalities linked to more than one type of
dementia occur simultaneously in the brain.
Clinical features:
Progressive and frequent memory loss (mostly short-term)
Confusion
Personality changes
Apathy and withdrawal
 Loss of functional abilities to perform activities of daily living
Diagnosis:
History including cognitive, behavioural and psychological
symptoms
Physical examination
Blood and urine tests
Cognitive testing
Examining cerebrospinal fluid
MRI
CT
Management:
pharmacological
Anti depresents
Anti psychotics
Hypnotics
Anxiety relieving drugs
Anticonvulasants
Cholinesterase inhibitors
Non pharmalogical
Cognitive stimulation therapy.
Reminiscence therapy for mild to moderate dementia.
Cognitive rehabilitation or occupational therapy
Lifestyle Modifications
Regular exercise and an active lifestyle. Very effective in the
management of the depression component of dementia.
 Stimulating, personalised daily activities

Infections
PYOGENIC MENINGITITS SEQUALE
DEFINITION:
• It is potentially life threatening neurological disorder.
• Inflammation of meninges of brain and spinal cord due to bacteril
infection
• Age – 6 to 12 months are more prone to meningitis
CAUSES:
AGE CAUSATIVE AGENT
0 – 2 months • STAPHYLOCOCCUS
AURES
• E.COLI
• GROUP B STREPTOCOCCI
2months – 2 years • STREPTOCOCCUS
PNEUMONEA
• NISSERIA MENINGITIS
Above 2 years • HAEMOPHILUS
INFLUENZA
• STAPHYLOCOCCUS
AURES
• STREPTOCOCCUS
PNEUMONEA
 • NISSERIA MENINGITIS

EPIDEMIOLOGY:
• 170,000 die every year.
• 1/3 deaths due to meningococcal disease.
• In developing countries incidence is 10 times than developed
countries in children.
• Mortality 5-15% developed countries
• Mortality 15-50% developing countries
PATHOLOGY:
• Those causative agent enters CNS via either direct invasion or
through blood stream
• Produce inflammation of meninges
• Meninges initiated during organisim disrupts blood – brain
barrier
• Release of cytokinis and chemokines in CNS stimulated by
bacteria
• Results, meninges will be swollen and damaged
• Early in illness cerebral edema present and ventricles reduced in
sizes
 • Pressure on peripheral nerves may lead to motor or sensory
deficit
• Communicating hydrocephalus due to adhesive thickening of
arachnoid in basal cisterns
• Cerebral vessels and cranial nerves can be involved and may lead
to permanent neurological deficit
CLINICAL FEATURES:
• Fever
• Sleepiness
• Bulging soft spot
• Vomiting
• Blotchy skin
• Chillness of hand
• Dyspnea
• Irriatibility
• Confussion
• Diarrhea
• Pink prick marks
• Cranial nerve palsy
• Neck stiffness

DIAGNOSIS:
• CSF ANALYSIS- increased / normal protein
• CSF lactate, ferritin level increased
• Serology – IgM antibody level
 • MRI Scan – diffuse brain swelling , localized areas of
inflammation
• EEG – diffuse slow wave activity
DIFFERENTIAL DIAGNOSIS:
• Confusion/dementia
• Cervical arthritis (stiff neck)
• Subarachnoid haemorrhage
• Pneumonia
• Otitis media
• Pharyngitis
• Gastroenteritis
• Upper Respiratory Infection
• Retropharyngeal Abscess
• Brain/Subdural/Epidural Abscess
• Encephalitis
MANAGEMENT:
Medical management:
 TB INECTION OF CNS
INTRODUCTION:
• M. tuberculosis is:
1. Free-living organisms that lack a cell wall.
2. Gram-negative intracellular organisms
3. Strictly aerobic, non-sporing, non-motile, weakly Gram-positive
bacilli.
4. Aerobic, oxidase positive Gram Negative bacilli.
5. M. tuberculosis belongs to the genus Mycobacterium.
6. They are strictly aerobes, nonmotile slender rods, and don’t form
spores.
DEFINITION:
Central nervous system tuberculosis occurs in approximately 1% of
all patients with active Tb. The CNS involvement includes:
• Meningitis
• Tuberculoma
• Abscess
• Spinal tuberculous arachnoiditis
EPIDEMIOLOGY:
• M>F
• 60-80 years age
 • 100% fatal if untreated
TYPES:
INTRACRANIAL –
• Miliary spread
• Encephalopathy
• Meningtis
• Tuberculoma
• Brain abcess
SPINAL TB –
• Potts spine
• Potts Paraplegia
• Tuberculous arachnoiditis
• Spinal tuberculoma
• Spinal meningitis

PATHOLOGY:
Step 1- Entrance of the bacilli into the host
Step 2- Lung invasion
Step 3- Regional lymph node dissemination
Step 4- Primary complex formation
• In case of CNS involvement the characteristic lesions known as
Rich’s foci tuberculous subpial or subependymal foci about 1
mm in diameter are formed
• CNS tuberculosis is secondary to disease elsewhere in the body.
• Mycobacteria reach the brain by hematogenous route.
• Initial small tuberculous lesions (Rich foci) develop in meninges,
subpial or subependymal surface of the brain or the spinal cord,
and may remain dormant for years.
• Reactivation may be due to endogenous factors:
• Innate immunological and non immunological defences Level of
function of cell mediated immunity.
• Tumour necrosis factor may have a role.
STAGING OF TBM:
• TBM is classified into 3 stages according to the British Medical
Research Council (MRC) criteria
• Stage I: Prodromal phase with no definite neurologic symptoms.
• Stage II: Signs of meningeal irritation with slight or no clouding
of sensorium and minor (cranial nerve palsy) or no neurological
deficit.
• Stage III: Severe clouding of sensorium, convulsions, focal
neurological deficit and involuntary movements.
INVESTIGATIONS:
• CSF Smear examination: Zeihl Nelson’s, Gram’s and India Ink
stain.
• Predominantly lymphocytic pleocytosis, with increased proteins
and low CSF/ blood glucose ratio.
• CSF protein (> 150 mg/dl) should always raise the suspicion of
tuberculosis or fungal infection, rarely seen in viral
meningitis.Smear is +ve in 10%, can be increased by examining
large volume of CSF.Culture is +ve in 25-70%.
• CT/ MRI confirm the presence and extent of basal arachnoiditis,
cerebral oedema, infarction, ventriculitis and hydrocephalus.
TREATMENT:
• Same as pulmonary TB
• CNS tuberculosis is categorised under TB treatment category I by
WHO.Initial phase therapy ( 2 mths) with isoniazid, rifampicin,
 pyrazinamide and streptomycin or ethambutol followed by
continuation phase (7 mths) with isoniazid and rifampicin.
Therapy should be extended to 18 months in patients who do not
tolerate pyrazinamide. Short duration therapy (6 mths) might be
sufficient if the likelihood of drug resistance is low. However as
the emergence of neurological deficit has been seen in some of the
studies so a minimum of 12 months treatment would be
worthwhile.
POOR PROGNOSTIC FACTORS:
• Stage of disease.
• Presence of miliary disease
• Severe disease on admission
• Delay in initiation of treatment
• Extremes of age
• Very abnormal CSF (very low glucose or elevated protein)

POLIOMYELIOTIS
INTRODUCTION
A viral disease which may affect the spinal cord causing muscle
weakness and paralysis
The words polio (grey) and myelin (marrow) indicating the spinal
cord are derived from the greek
Poliomyelitis is a disease of the anterior horn motor neurons of
the spinal cord and brain stem caused by poliovirus
 Poliomyelitis, literally meaning “grey” spinal cord inflammation
It is a viral infection
DEFINITION
It is an acute viral infectious disease caused by enterovirus
The word myelitis meams an inflammation in the spinal cord,
which often targets insulting material covering nerve cell fibres
EPIDEMIOLOGY
Incidence:
2011 india – cases -1wild polio
ETIOLOGY
Age: infants and elderly
Living with infected person
Compromised immune system
Lack of immunization against polio
Extreme stress or sternous activity
Travel to an area that has experienced polio outbreak
LIFE CYCLE OF POLIO VIRUS
Enters through mouth
↓
Intestine if the virus finds a cell with the correct receptor in the intestine
↓
Infection begins
 ↓
The polio virus genoma enters the intestinal cell
↓
The viral RNA takes over the cell
↓
Replicates in the intestinal cell
↓
New RNA + new capsids = new polio virus
↓
Thousands of polio virus burst out of cell and enters the blood stream
PATHOPHYSIOLOGY
Portal of entry (feco – oral, inhalational, infects the pharynx and
intestinal mucosa)
↓
Local multiplication (epithelial cells of GIT and lymphatic tissue)
↓
Minor/ primary viremia (spreads through regional lymph nodes)
↓
Major/ secondary viremia (multiplie in reticulo endothelial systems)
↓
To the spinal cord and brain (blood stream)
↓
Multiplies in neurons
↓
Degeneration of nissil’s body
↓
Lesions are mostly in anterior horns of the spinal cord causing flaccid
paralysis and Destroys them
CLINICAL MANIFESTATIONS
Inapparent/ assymptomatic (90 – 95%)
Accounts for approximately 95% of cases
Virus stays in intestinal tract and does not attack the nerves
Virus is shed in the stool so infected individual is still able to
infect others
Apparent/ symptomatic (5% - 10%)
Abortive polio
Non paralytic aseptic meningitis
Paralytic poliomyelitis
Polio encephalitis
Abortive polio
4% - 8% infections
Does not lead to paralysis
Minor illness
Symptoms;
Low grade fever
Sore throat
Vomiting
Abdominal pain
Loss of appetite
Malaise
Non paralytic aseptic meningitis
Occurs in 1% - 2% of polio infections
Symptoms;
Headache
Nausea
Vomiting
Pain and stiffness of the neck, back and legs
Complete recovery after 2 – 10 days of symptoms
Signs;
Tripod sign
Kiss the knee test
Head drop sgn
Neck rigidity
Paralytic poliomyelitis
0.5% - 1% of those infected develop this type
2 phases:
Minor – same as absorptive polio
Major – muscle pain, spasam and return of fever
Followed by rapid onset flaccid paralysis, complete within 72
hours
It is of three types
Spinal paralytic poliomyelitis
Bulbar poliomyelitis
Bulbo – spinal poliomyelitis
1. Spinal paralytic poliomyelitis
Attacks motor neurons in the spinal cord and causes
paralysis in arms and legs and breathing problems
Most common 79% - 80% of cases
Results from lower motor neuron lesion of anterior horn
cells of spinal cord
Affect muscles of legs, arms and trunk
Severe cases – quadriplegia, paralysis of trunk, abdominal
and thoracic muscles
Muscles – floppy
Reflexes – diminished, deep tendon reflex lost before onset of
paralysis
Sensation – normal
Residual paralysis – after 60 days
2. Bulbar poliomyelitis
Affects neurons responsible for sight, vision, taste,
swallowing and breathing
Accounts for 2% of paralytic polio
Life threatening
Virus attacks motor neurons in brain stem
Affects cranial nerve function
Cranial nerve lesion – vagus
Primarily inhibits ability to breathe, speak and shallow
effectively
Facial asymmetry present
Symptoms:
nasal twang and hoarseness of voice
nasal regurgitation
dyspnea
dysphagia
child refuses to feed
secretions accumulate in pharynx – aspiration
respiratory centre – shallow, irregular respiration
vasomotor centre – BP rises and then fall
pulse – rapid, weak thread
skin – dusky and mottled
restless, confused and comatose
3. bulbo – spinal poliomyelitis
accounts for 19% of paralytic cases
affects extremities and cranial nerves
leads to severe respiratory involvement
combination of spinal paralytic and bulbar polio
DIAGNOSIS
history
clinical examination
stool examination
CSF examination
Serological test
Stool examination
TREATMENT
Symptomatic and supportive
The goal of the treatment is to control symptoms while the
infection runs its course as there is no specific treatment for the
viral infection
Treatment may includes:
Hospitalization
If the respiratory is involved, long – term ventilation is necessary
Catheterization
Antibiotic for UTI
Moist heat
Pain killer to reduce muscle pain, headache
Physical therapy, braces or corrective shoes or orthopedic
surgery to recover muscle strength and function
Reconstructive surgery
Bed rest
Pain relief
Physiotherapy
 Occupational therapy
Recreational therapy
Speech therapy
COMPLICATION
Myocarditis
Hypertention
Pulmonary edema
Pneumonia
Urinary tract infection
Compression neuropathy
Scoliosis
Osteoporosis
Bone fractures
Skeletal deformities
Equines foot
PROGNOSIS
non paralytic cases – compete recovery
paralytic polio – permanent weakness in 2/3rd cases
worse – older children, sudden onset of illness with high fever

TABES DORSALIS
DEFINITION:
• Posterior spinal root and posterior column dysfunction
• Tabes dorsalis is a late consequence of neurosyphilis,
characterized by the slow degeneration (specifically,
 demyelination) of the neural tracts primarily in the dorsal root
ganglia of the spinal cord ( nerve root ).
• These patients have lancinating nerve root pain which is
aggravated by coughing, and features of sensory ataxia with
ocular involvement.
EPIDEMIOLOGY:
• The disease is more frequent in males than in females.
• Onset is commonly during mid-life.
• The incidence of tabes dorsalis is rising, in part due to co-
associated HIV infection
• It is common in males than in females and manifests, 8–12
years after primary infection.
• The age of onset is mostly 35–50 years.
CAUSES:
• Demyelination by advanced syphilis infection
• Infection by the causative spirochete bacterium, Treponema
pallidum
• Syphilis is a bacterial infection that is spread sexually
• Transfusion of contaminated blood or blood products
(contaminated by the syphilitic bacteria)
PATHOLOGY:
• It manifests as atrophy of the dorsal spinal roots,(especially
lower thoracic/lumbosacral regions) the axons of dorsal root
ganglia, and compression of dorsal root fibres by meningeal
constrictions.
 1. Optic atrophy
2. Argyll-Roberts on pupil(Pupillary abnormality)
3. The sensory fibres of V and IXth cranial nerves may be
involved.

CLINICAL MANIFESTATIONS:
• Pain and hyperpathia due to irritation of nerve roots(lightning
pain)
• Sensory loss (interruption of reflex arc) and sensory ataxia due to
posterior column involvement.
• Loss of deep sensations (JPS, VS and Rombergism)Abadie’s sign
is loss of painful sensation on squeezing the tendoAchilles or testis
 • Argyll-Robertson pupil(impaired response to light)
• Optic atrophy
• Areflexia.
• Lightning pains/thunder bolt pain
• Abdominal pain/diarrhoea
• Hypoesthesias (abnormally diminished cutaneous, especially
tactile, sensory modalities)
• Tabetic gait (locomotor ataxia)
• Tabes dorsalgia" is a related lancinating back pain
SIGNS AND SYMPTOMS:
• Abnormal sensations (paresthesia), often called “lightning pains”
• Problems walking such as with the legs far apart
• Loss of coordination and reflexes
• Joint damage, especially of the knees
• Muscle weakness
• Vision changes
• Bladder control problems
• Sexual function problems
• Ambulation difficultie
• Muscle weakness
 • Wide based gait
• Degeneration of joints
INVESTIGATION:
• Physical exam with evaluation of medical history characteristic
features of a myelopathy
• Exam of cerebrospinal fluid, via a lumbar puncture
• CT scan of cranium
• Cerebral angiogram
• MRI scan of brain, spinal cord
• Blood test, for detecting antibodies against Syphilis
• Nerve damage will be suggested by the presence of areflexia
• Serum rapid plasma reagin for screening the infection
MANAGEMENT:
• Intravenouly administered penicillin G
• Ceftriaxone IV
• For pain analegics like :opiates, valproate, or carbamazepine
• Mild form of narcotics
• Physiotheraphy :strenthening ,mobility,balance training and
frenkel’s exercise
• Prevention of complication
PROGNOSIS:
• Left untreated, tabes dorsalis can lead to paralysis, dementia,
and blindness. Existing nerve damage cannot be reversed.
• Death may result from respiratory, neurological, cardiovascular,
or liver complications
• The outcomes are poorer in individuals diagnosed with AIDS/HIV
infection, or other opportunistic infections

Disease of the muscle

Myopathy
DEFINITION:
Myopathies are disorders with structural changes or functional
impairment of muscle.
It does not include upper motor neuron lesions, lower motor
neuron lesions, myasthenia gravis which also causes muscle
weakness.
TYPES OF MYOPATHIES:
Inflammatory Myopathies
Polymyositis
Dermatomyositis
Inclusion body myositis
Muscular dystrophies
X-linked
Limb-girdle(ar/d)
Congenital
Fasioscapulohumeral (ad)
Oculopharyngeal
Myotonic Syndromes
Myotonic dystrophy (ad)
Inherited
Schwarz-Jampel
Drug-induced
Congenital myopathies
Central core disease
Nemaline myopathy
Myotubular
Metabolic myopathies
Glycogen storage disorders
Mitochondrial
Periodic paralysis
Endocrine myopathies
Thyroid
Parathyroid
Adrenal/steroid
Pituitary
Drug-induced/toxic
CLINICAL FEATURE:
Symentric muscle weakness
Increase difficulty in daily life activities
Ocular muscles are spared
Head drop sign
Dysphagia
Respiratory muscle weakness
Muscle wasting
Sensation is normal
Dtr preserved
Malaise
Dark color urine
Fever
 Fatigue
dermatoyositis
DIAGNOSIS:
criterion
myopathic muscle weakness
electromyogram
muscle enzymes
muscle biopsy
rash
TREATMENT:
glucoorticoids
immunosuppressive drugs
IVIg
IBM
MUSCULAR DYSTROPHY
DEFINITION:
• A general term for a number of hereditary, progressive
degenerative disorder affecting skeletal muscles and often other
organ systems as well.
• Muscular dystrophy is a heterogeneous group of inherited
disorders recognized by progressive degenerative muscle
weakness and loss of muscle tissue (started in childhood).
• Affect muscles strength and action.
• Generalized or localized.
• Skeletal muscle and other organs may involve
CAUSES OF MUSCULAR DYSTROPHY:
• Inheritance
 • Dominant genes
• Recessive gene
• Depends on the age when symptoms appear, and the types of
symptoms that develop.
Risk factors:
• Because these are inherited disorders, risk include a family history
of muscular dystrophy
INCIDENCE:
• It is estimated that between 50,000 -250,000 are affected annually.
1 per 3500 live male births
TYPES OF MUSCULAR DYTROPHY:
• Duchenne’s Muscular Dystrophy
• Becker’s Muscular Dystrophy
• Emery- Dreifuss Dystrophy
• Facioscapulohumeral Dystrophy
• Scapuloperoneal Syndrome
• Oculopharyngeal Dystrophy
• Congenital Muscular Dystrophies
• Kearns-Sayre Syndrome
• Myotonic Dystrophy
• Limb-Girdle Muscular Dystrophies
CLASSIFICATION:
• Sex-linked: DMD, BMD, EDMD
• Autosomal recessive: LGMD, infantile FSHD
 • Autosomal dominant: FSHD, distalMD, ocular MD,
oculopharyngeal MD.

Duchenne’s Muscular Dystrophy:

• Also called Pseudohypertrophic muscular dystrophy.
• X linked recessive disorder

Incidence : 30 per 1,00,000 live born males

• Most common male, Turner syndrome
• 1/3 new mutation
• 65% family history
Etiology:
• single gene defect
• Xp21.2 region
• absent dystrophin
Clinical manifestation:
• Shoulders and arms are held back awkwardly when walking
• Sway back
• Belly sticks out due to weak belly buttons
• Weak butt buttons
• Thin weak thigh muscles
• Knees may bend back to take weight
• Poor balance
• Thick lower leg muscles
• Tight heel cord
• Weak muscles in front of the leg cause foot drop and tip toe
contractures
• Progressive weakness
• Spinal deformity
• Cardiopulmonary involvement
• Mild – moderate MR
Diagnosis:
• Gait
• Absent DTR
• Ober test
• Thomas test
• Meyeron sign – child slips through truncal grasp
• Macroglossia
• Myocardial deterioration
• IQ ~ 80
• Increase CPK (200x)
• Myopathic change in EMG
• Bx: m. degeneration
• Immunoblotting: Absence dystrophin
• DNA mutation analysis

Treatment:
Physical Therapy:
aim : to keep joints as loose as possible.
Commenced at 3-4 yrs of age , when parents are taught to stretch
child’s heel cords, hip flexors, iliotibial bands on daily basis.
• Night splints can be considered
• Bracing : appropriate use of bracing – delay child’s progression
to wheelchair by approx 2yrs
Surgery : Reconstructive surgery of the leg often accompanies bracing.
The purpose : to keep leg extended & prevent contractures of iliotibial
bands & hip flexors .
• Percutaneous tenotomies of Achilles tendon, knee flexors, hip
flexors and iliotibial bands.
• Pharmacological : Prednisolone improves muscle strength & fn (
3 yrs . Deflazacort – synthetic steroid .
Becker’s Muscular Dystrophy
Less severe form of X-linked recessive muscular dystrophy results from
allelic defects of the same gene responsible for Duchenne dystrophy
Incidence:
• 3 per 1,00,000 live born males.
• The pattern of muscle wasting in Becker muscular dystrophy
closely resembles that seen in Duchenne.

Etiology:
• single gene defect
• short arm X chromosome
• altered size & decreased amount of dystrophin

Clinical feature:
• Less common
o 1: 30000 live male birth
• Less severe
• Family history: atypical MD
• Similar & less severe than DMD
• Onset: age > 7 years
• Pseudohypertrophy of calf
• Equinous and varus foot
• High rate of scoliosis
• Less frequent cardiac involvement
Diagnosis:
• The same as DMD
• Increase CPK (<200x)
• Decrease dystrophin and/or altered size
Treatment:
• the same as in DMD
• forefoot equinous: plantar release, midfoot dorsalwedge
osteotomy
Emery – Dreifuss Dystrophy:
Emerin deficiency( a lamina associated structural protein )
X linked recessive disease
Responsible gene – on long arm of X chromosome, close to
centromere
Etiology
• X-linked recessive
• Xq28
• Emerin protein (in nuclear membrane)
Epidemiology
• Male: typical phenotype
• Female carrier: partial
Clinical Features
• Muscle weakness
• Contracture
• Neck extension, elbow, achillis tendon.
• Scoliosis: common, low incidence of progression
• Bradycardia, 1st degree AV block
• sudden death
Diagnosis:
• Gower’s sign
• Mildly/moderately elevated CPK
• EMG: myopathic
• Normal dystrophin
Treatment:
• Physical therapy
Prevent contracture: neck, elbow, paravertebral muscles
For slow progress elbow flexion contracture
• Soft tissue contracture
Achillis lengthening, posterior ankle capsulotomy + anterior
transfer of tibialis posterior
• Spinal stabilization
 For curve > 40 degrees
• Cardiologic intervention
• Cardiac pacemaker

Facioscapulohumeral Dystrophy
• Autosomal dominant disease
• Responsible gene – end of long arm of chromosome 4
• Genetic abnormality – deletion
• Severity of illness – related to size of deletion: smallest fragments
– severe disease
• Onset : childhood / young adulthood

Etiology
• Autosomal dominant
• Gene defect (FRG1)
• Chromosome 4q35
Epidemiology: Female > male

Clinical feature:
• Facial weakness is the initial manifestation – inability to
smile,whistle,fully close the eyes
• Weakness of shoulder girdles usually brings pt to medical
attention
• Loss of scapular stabilizer muscles makes arm elevation difficult.
• Scapular winging – with attempts at abduction & forward
movement of arms.
• Biceps & triceps severely affected with relative sparing of deltoid
muscles.
• Weakness worst for wrist extension
• Weakness of ant.compartment muscles of legs – footdrop
• Weakness of hip flexors , quadriceps , ankle dorsiflexors +
• But plantar flexors strength is preserved
 • Children might lose ability to walk by 9-10 yrs.

Diagnosis:
• DNA studies
• EMG
• Muscle Biopsy
Treatment:
• Supportive
• If pt unable to lift arms above head – surgical stabilization of
scapula
• Ankle-foot orthoses – footdrop
• Surgical transposition of posterior tibial tendon to dorsum of foot
– for pts with marked intorsion of foot while walking.

Oculopharyngeal Dystrophy
• Autosomal dominant disorder
• Hallmark of illness “ presence of small intranuclear
tubulofilaments. These occur as palisading filamentous
inclusions”
Clinical Features :
• Begins at 30-40 yrs with weakness of eye muscles & mild ptosis
• Ptosis – asymmetrical initially, as muscles weaken, both lids
become severe ptotic , eye movements are diminished in all
directions.
• Later , pt develops difficulty in swallowing.
• Death – starvation , emaciation – pneumonia following aspiration
Diagnosis :
• Muscle Biopsy
• EMG – typical myopathic
• S.CK – Elevated
• Tensilon test & repetitive nerve stimulation test for abn fatigue of
evoked potential – to differentiate from myasthenia gravis
Treatment:
• Supportive
• Swallowing difficulties : initially managed by taking soft diet.
Later – nasogastric tube , gastrostomy.

Congenital Muscular Dystrophies

• A group of diseases that often appear at birth with hypotonia &
severe trunk & limb weakness.
• Contractures of joints are prominent – particularly at ankle, knees
& hips
• MR may be present
• MRI brain – increase in signal from the white matter in many pts.
• All are autosomal recessive
• They are 1. Merosin deficiency
2. Fukuyama type muscular dystrophy
3. Walker-Warburg disease
4. Muscle-eye-brain disease of Santavuori

Merosin Deficiency
• Laminin alpha 2 , formerly known as merosin – found in basement
membrane
• It is found in muscle as well as skin & nerve.
• Onset in infancy
• Severe weakness of trunk & limbs and hypotonia at birth.
• Extraocular muscles & face – spared
• Prominent contractures of feet & hips
Diagnosis:
• MRI
• Lab invg : S.CK – Elevated
EMG – Slowed nerve conduction velocities
Management:
• demonstration of alteration of laminin alpha 2 in muscle
 • muscle / skin biopsy
• demonstration of abn gene on chr 6
Fukuyama Type Muscular Dystrophy
• Autosomal recessive disease
• Responsible gene – chr 9q31-33
Clinical features :
• Normal at birth
• Some are floppy, joint contractures in 70% pts by age of 3 months
– hip & knee
• Children – severely mentally retarded
• Weakness is diffuse & disabling – child never learns to walk
Diagnosis :
• S.CK – elevated
• Muscular biopsy : dystrophic changes with variability in size of
fibres and fibrosis
• CT Scan – presence of lucencies in frontal areas

Kearns-Sayre Syndrome
• Belongs to the group “ Mitochondrial myopathies “
• Due to large deletion in mitochondrial DNA.
• Severity depends on ratio of mitochondria with deletions to
normal mitochondria
• It is almost always a sporadic disease
Clinical features :
• Progressive external ophthalmoplegia
• May start in childhood / adult life – progress to total immobility of
eyes.
• Associated with retinitis pigmentosa
• Presence of mitochondrial abn in striated muscle & other tissues
• Cerebellar incoordination & nerve deafness
• MR + short stature
• Cardiac conduction defects– sudden cardiac death
Diagnosis :
• Muscle biopsy : numerous ragged-red fibres in trichrome stain
against relatively normal muscle
• CSF protein – elevated
• CSF folate – reduced
Treatment :
• Thiamine, folate, riboflavin, carnitine, ubiquinone, methionine

Myotonic Dystrophy
• Also known as dystrophia myotonica
• Composed of 2 clinical disorders with overlapping phenotypes &
distinct molecular genetic defects :
1. DM1- the classic disease
2. DM2- proximal myotonic myopathy
• Autosomal dominant disease
• Responsible gene – chr 19q13.3
• CTG trinucleotide repeats

Clinical feature:
• Hatchet-faced appearance d/t temporalis, masseter, facial muscle
atrophy & weakness
• Frontal baldness
• Neck muscles, sternocleidomastoids & distal limb muscles –
involved early
• Weakness of wrist & finger extensors, intrinsic muscles of hand
• Ankle dorsiflexors weakness – foot drop
• Proximal muscles remain strong all throughout the course of
disease
• Palatal,pharyngeal & tongue inv produce a dysarthric speech,
nasal voice & swallowing problems
• Diaphragm & intercostal muscle weakness – resp insuff
 • Myotonia appears by age 5 yrs – percussion of thenar eminence ,
tongue, wrist extensor muscles
• Myotonia causes slow relaxation of hand grip after a forced
voluntary closure
• Cardiac disturbances – common in DM1
• 1st degree heart block, complete heart block, sudden cardiac death
• MVP is common
• Intellectual impairment, hypersomnia, posterior subcapsular
cataract, gonadal atrophy, insulin resistance, decreased
esophageal & colonic motility
• Congenital myotonic dystrophy – more severe form of DM1
- severe facial & bulbar weakness, transient neonatal
respiratory insufficiency & mental retardation
• DM2 – distinct pattern inv of muscles – proximal muscles
Diagnosis:
• S.CK – Normal / mildly elevated
• EMG
• Muscle Biopsy
Treatment:
• Supportive treatment – ankle foot orthoses to treat foot drop
• Breathing exercises & postural drainage – in severe myotonia to
ward off frequent respiratory infections.
• Quinine, phenytoin, procainamide, mexiletine & acetazolamide –
to treat myotonia
• Cardiac pacemaker- in pts with unexplained syncope, conduction
system defects.

Limb-Girdle Muscular Dystrophies

Etiology
• Autosomal recessive at chromosome 15q
• Autosomal dominant at 5q
Epidemiology
• Common
• More benign
Clinical manifestation
• Age of onset: 3rd decade
• Initial: pelvic/shoulder m. (proximal to distal)
• Similar distribution as DMD
Classification
• Pelvic girdle type - common
• Scapulohumeral type - rare
Diagnosis
• Same clinical as DMD/BMD carriers
• Moderately elevated CPN
• Normal dystrophin
Treatment
• Similar to DMD
• Scoliosis: mild, no Rx.
SPINAL MUSCULAR ATROPHIES(SMAs)
DEFINITION:
• It is a group of inherited neuromuscular disease that attacks motor
neurons, which control the movement of voluntary muscles.
• It cause lower motor neurons in the base of the brain and spinal
cord degenerate gradually.
• It is also the second most common fatal, autosomal recessive
disease in Caucasians (after cystic fibrosis).
• Incidence of 1/10000
• M>F
CAUSES:
• SMN 1 (survival of motor neuron)gene is missing or mutated with
chromosome 5
• Programmed cell death of neuroblasts .
PATHOLOGY:
• The mutations affects the gene what has been termed the survival
of motor neurons

TYPES:
Depending on the age of onset, degree of muscular involvement and
length of survival.. there are 5 types
1. Infantile progressive SMA type 1
2. Intermediate SMA type 2
3. Juvenile SMA type 3
4. Adult SMA type 4
 5. SMARD 1
6. Congenital SMA with Arthrogryposis
Type I – Werdnig Hoffman disease (Acute Infantile SMA)
• This is an autosomal recessive disorder.
• Incidence 1:25 000 births
Clinical features:
• Reduced fetal movements in late pregnancy with weakness and
hypotonia at birth.
• All motor milestones are delayed; 95% of all patients are dead by
18 months.
• Hypotonia
• Bulbar dysfunction
• Prolonged cyanosis
• Loss of tendon reflex
• Fasciculation ( fine trembling)
• Tremors
• Swallowing and breathing difficulty.
• Scoliosis
Type II – Kugelberg Welander disease (Late infantile or juvenile SMA)
• Pathological features similar to Werdnig Hoffman disease.
 • Begin between 6-18 months
• Common form of SMA
Clinical features:
• Motor delay, failure to sitting independently or failure to stand by
1 year of age.
• Some of the childrens learn to sit but will never be stand or walk.
• Limb girdle muscles affected.
• Pseudohypertrophy of gastrocnemius muscle.
• Fasciculation
• Scoliosis ,contracture
• It is slowly progressive with great variability even within the same
family.
• Median age at death 12 years. Mostly due to respiratory
infections.
Type III (Adult onset SMA) kugelberg – walender
• Onset between 2nd and 5th decade with progressive limb girdle
weakness. (since 18 months)
• Distinction from progressive muscular atrophy form of ALS is
difficult. A benign course supports the former.
• Bulbar dysfunction in the later stage
• Able to stand and walk but difficulty to climbing up & down stairs.
 • Pseudohypertrophy
• Fine tremor of fingers
• Abnormal gait
• Scoliosis
• Joint contractures
• Respiratory infections
Type IV
• Adult form
• age onset- 35 years
• Autosomal recessive and dominant
• Symptoms get worse slowly
• Initially not able to find the condition in later stage can find with
symptoms
Spinal muscular atrophy with respiratory distress type 1 (SMARD1)
• Severe respiratory distress due to paralysis of diaphragm
• Symptoms onset 1-6 months
• Respiratory failure with progressive muscle weakness
predominantly in lower limb.
Congenital SMA with Arthrogryposis
 • Persistent contracture of joints with fixed abnormal posture of
limbs
• Rare condition
• Clinical features:
• Severe contracture
• Curvature of the spine
• Chest deformity
• Respiratory problems
• Small jaw drooping of eyelids
Kennedy’s disease
• Progressive spinobulbar muscular atrophy
• Age- 15 to 60 years
• Enlargement of male breasts / develop of NI-DDM type 2
• Clinical features:
• Weakness and atrophy of facial, tongue and jaw muscles
• Difficulty in swallowing , chewing and speech
• Pain and muscle fatigue – early
• Fasciculations
• Sensory loss in feet and hands
DIAGNOSIS:
 • Blood test- CK level typically normal
• Genetic testing – SMN1 deletion is 95% sensitive {definitive
diagnose of SMA}
• EMG – to determine the motor neurons has affected
• Muscle biopsy- neuromuscualar disease
• NCS – Nerves ability to send a signal
DIFFERENTIAL DIAGNOSIS:
• Amyotrophic lateral sclerosis
• Congenital muscular dystrophy
• Congenital myopathies
• Carbohydrate metabolism disorders
TREATMENT:
• Riluzole
• Gabapentin
• Valproic acid
• Muscle relaxants- baclofen
• Botulinum toxin – treat jaw spasm / drooling
• Excess saliva- amitriptyline,atropine
• antidepressants
 Peripheral nerve disorders
PERIPHERAL NERVES INJURIES
INTRODUCTION
Peripheral nerve injuries are formed by the bundles or axons,
each with a neurilemmal tube
Peripheral nerves are formed from nerve and spinal cord 31 pairs
or peripheral nerves represent segment of spinal cord as a direct
brachial plexus
Mixed nerves →motor, sensory and autonomic
Dermotome :
is an area of skin supplied by a single spinal root
Myotome :
Represents a muscle unit supplied by a single spinal root

CLASSIFICATION
Seddon's classification
Neurapraxia –
Temporary paralysis of a nerve caused by lack of blood flow or by
pressure on the affected nerve with no loss of structural continuity.
Axonotmesis –
Neural tube intact, but axons are disrupted.
Nerves are likely to recover.
Neurotmesis –
The neural tube is severed.
Injuries are likely permanent without repair.
Sunderland`s classification
Grade I
Same as Seddon's neuropraxia.

Grade II
Same as Seddon's axonotmesis.
Grade III
Neurotmesis with preservation of the perineurium.
Grade IV
Neurotmesis with preservation of th epineurium.
Everything else is disrupted.
Nerve grossly appear edematous.
Nerve grafting is required.
Grade V
Complete transection of the nerve trunk.

TYPICAL DEFORMITIES
Wrist drop - radial nerve injury
Claw hand - ulnar nerve injury
Foot drop - lateral popliteal nerve injury
Ape thumb - median nerve injury
Winging of scapula - thoracodorsal nerve injury
Pointing index - median nerve injury

Ulnar nerve injury

Causes :
General causes : metabolic diseases , collagen diseases , malignancies ,
endogenous or exogenous toxins , chemical or mechanical trauma , etc.
Local causes :
Causes in the axilla :
Crutch pressure
 Aneurysm of the axillary vessels
Causes in the arm :
# shaft of humerus
Gunshot and penetrating injuries
Causes at the elbow :
Compression by the accessory muscles
# lateral epicondyle of humerus
Repeated occupational strains
Recurrent subluxation of the nerve
Compression by the osteophytes as in rheumatoid and
osteoarthritis
Causes in the forearm :
# both bones forearm
Incised wounds , gunshot wounds and penetrating injuries of
the forearm
Causes at the wrist :
Compression by osteophytes
# hook of the hamate
Compression by ganglion
Wrist injuries
Causes in the hand:
Blunt trauma
Penetrating injuries
Ulnar nerve injuries gives rise to claw hand Deformity
Claw hand deformity
It is a deformity with hyperextension of the MCP joints and flexion
of the IP joints of the fingers ( loss of flexon at MCP and extension
at IP joints )
Clinical features
Loss of sensation along the ulnar nerve distribution and
 Wasting of the hypothenar muscles , intrinsic muscles of the hand
leading to hollow intermetacarpal spaces on the dorsum of the
hand of the lesion
High : above the level of elbow , entire nerve function is lost
Low : Below the elbow at the junction of the middle and lower
third of forearm :
Spared : - function of FDP and FUC
Lost :
Motor : HTM ,Its , Lum ,PB
Sensory : dorsal aspect of hand and one and half Fingers
Proximal to Guyon`s canal :
Spared : FDP , FCU and dorsal sensation
Lost : same as above + loss of volar sensation
Distal to Guyon`s canal : -
Spared : FDP , FCU , HTM , PB, dorsal and volar sensation
Lost : interossei and lumbricals
FCU – flexor carpi ulnaris
FDP – flexor digitorum profundus
HTM – hypothenar muscles
PB – palmaris brevis
Lum – lumbricals
Its – interossei
Clinical tests :
Froment's sign.
When the patient attempts to pinch with the thumb and index
finger, the long flexor of the thumb is used to substitute for the
thumb adductor, resulting in flexion of the thumb at the
interphalangeal joint.
This characteristic appearance is present in this patient's left
hand, caused by an ulnar nerve lesion at the elbow
Card test
 Inability to hold a card or paper in between fingers due to loss of
adduction by the palmar interossei
Pen test
Unable to touch the pen due to the loss of action of abductor
pollicic brevis
Egawa test
With palm flat on the table the patient is asked to move the middle
finger sideways( test for the dorsal interossei of middle finger )
In total clawing median nerve is also injuried
Pointing index or oschner`s clasp test :
When both the hands are clapsed together , index and middle
fingers , fail to flex due to the loss of action of long finger flexors
of the index and middle fingers which are supplied by the median
nerve . of ulnar nerve injury
Unless there is a lot of muscle wasting, (nonsurgical treatment )
Prevention
Avoid frequent use of the arm with the elbow bent
If you use a computer frequently, make sure that your chair is not
too low. Do not rest the elbow on the armrest.
Avoid putting pressure on the inside of the arm (do not drive with
the arm resting on the open window ).
Keep the elbow straight at night when you are sleeping (done by
wrapping a towel around the straight elbow, wearing an elbow
pad backwards, or using a special brace )
Nonsurgical Treatment
If symptoms have only just started, Anti – inflammatory drugs,
ibuprofen,( to reduce swelling around the nerve ).
Steroid (cortisone) injections around the ulnar nerve are not
generally used because there is a risk of damage to the nerve.
Exercises ( prevents arm and wrist from stiffness ).
Surgical Treatment
If the nerve is very compressed; or if there is muscle wasting
Surgery:
Around the elbow and the wrist or both
More commonly, the nerve is moved from its place behind the
elbow to a new place in front of the elbow. This is called an
anterior transposition of the ulnar nerve.
The nerve can be moved : - under the skin and fat (subcutaneous
transposition),
within the muscle (intermuscular transposition) or
under the muscle (submuscular transposition).
ulnar paradox
Median nerve:
The higher the lesion of the median and ulnar nerve injury , the
less prominent is the deformity and vice versa, because in higher
lesions the long finger flexors are paralysed .
The loss of finger flexion makes the deformity look less obvius
Radial nerve injury
Causes:
General causes : metabolic diseases , collagen diseases ,
malignancies , endogenous or exogenous toxins , chemical or
mechanical trauma , etc.
Local causes : -
In the axilla :
Aneurysm of the axillary vessels
Crutch palsy
In the shoulder:
Proximal
humeral #
Shoulder dislocation
In the spiral groove ( 5 `s )
Shaft #
Saturday night #
 Syringe palsy
`S ` march`s tourniquet palsy
Between spiral groove and lateral epicondyle :
# shaft humerus
Supracondylar # humerus
Lateral epicondyle # of humerus
Penetrating and gunshot injuries
Cubitus valgus deformity
At the elbow :
Posterior dislocation of elbow
# head of radius
Monteggia #
Causes in the forearm :
# both bones of forearm
Penetrating and gunshot injuries
Levels of lesion
High above spiral groove---- total palsy
Low :
Type 1 (Between the spiral groove and the lateral epicondyle ) : -
Spared : - elbow extensor
Lost : -
Motor : wrist extensor , thumb extensor , finger extensor
Sensory : dorsum of first web space
Low
Type 2 (below the elbow) :
Spared :
Elbow extensor
Wrist extensor
Lost :
Motor : thumb extensor , finger extensor
Sensory : First web space
Clinical features
 Depend upon the site of the injury: -
Lesions in or above the axilla :
Paralysis and wasting of all the muscles innervated.
Clinically, this is manifest as:
weakness of forearm extension and flexion - triceps and
brachioradialis
wrist drop and finger drop - paralysis of the extensors of the
wrist and digits
weakness of the long thumb abductor and extensor muscles
Sensory loss on the dorsum of hand and forearm appropriate to
the cutaneous distribution
Lesions around the humerus spare brachioradialis and extensor
carpi radialis longus.
Posterior interosseous palsy (due to a dislocation or fracture of the
elbow ).
weakness of finger extension, and of thumb extension and
abduction.
little or no wrist drop, and usually, no sensory loss.
Diagnosis
Tests
Muscles supplied by the radial nerve and how to test each:
C7,8: triceps - ask patient to extend elbow against resistance.
C5,6: brachioradialis - ask patient to flex elbow with forearm half
way between pronation and supination.
C6,7: extensor carpi radialis longus - ask patient to extend wrist
to radial side with fingers extended.
C5,6: supinator - with arm by side, ask patient to resist hand
pronation.
C7,8: extensor digitorum - ask patient to keep fingers extended at
MCP joint.
C7,8: extensor carpi ulnaris - ask patient to extend wrist to ulnar
side.
 C7,8: abductor pollicis longus - ask patient to abduct thumb at
90° to palm.
C7,8: extensor pollicis brevis - ask patient to extend thumb at
MCP joint.
C7,8: extensor pollicis longus - ask patient to resist thumb flexion
at IP joint.
Sensation:
The cutaneous branches of the radial nerve supply the dorsal
aspect of the forearm from below the elbow down over the lateral
part of the hand to include the thumb to the interphalangeal joint
and the fingers to the distal interphalangeal joint.
Exams and Tests
An examination of the arm, hand, and wrist identify radial nerve
dysfunction.
Decreased ability to extend the arm at the elbow
Decreased ability to rotate the arm outward (supination)
Difficulty lifting the wrist or fingers (extensor muscle weakness)
Muscle loss (atrophy) in the forearm
Weakness of the wrist and finger
Wrist or finger drop
Tests for nerve dysfunction :
EMG
MRI of the head, neck, and shoulder
Nerve biopsy
Nerve conduction tests
Treatment
Closed fracture
CONTROL OF SYMPTOMS
Analgesics ( to control pain neuralgia)
Phenytoin, carbamazepine, or tricyclic antidepressants
(amitriptyline) to reduce stabbing pain
Steroids (prednisone) to reduce swelling
 Other treatments include:
Braces, splints,
Physical therapy to help maintain muscle strength
Occupational therapy, or job counseling
Surgery : -
Failure of conservative by 12 to 18 months
Surgery ( open #)
Clean wound :
Primary repair , splint , physiotherapy
Contaminated wound :
Delayed primary repair and secondary repair
Late cases :
Tendon transfers
Arthrodesis
Splints
Complications
Mild to severe deformity of the hand
Partial or complete loss of feeling in the hand
Partial or complete loss of wrist or hand movement
Recurrent injury to the hand
Sciatic nerve injury
Thickest nerve in the body
Leprosy is the commonest cause
High stepping gait is the characterisic
Conservative treatment is indicated up to one year
Foot drop
Causes
General causes: metabolic diseases, collagen diseases , malignancies ,
endogenous or exogenous toxins , chemical or mechanical trauma , etc.
Local :
At the spine :
 Spina bifida
Tumors
Disc prolapsed

At the hip :
Posterior dislocation of the hip
# around the hip
# acetabulum
At the gluteal region :
Deep I.M injections
At the thigh :
# shaft femur
Penetrating injury and gunshot Injury
At the knee ( common causes )
Forcible inversion of the knee
Dislocation of knee
# lateral condyle of tibia
Tight plaster casts around the knee
Surgical damage during application of skeletal traction
Gunshot injuries , incised and penetrating Injuries
Levels of lesion
High lesion (above knee ) :
Both tibial and common peroneal nerve are paralysed
Low lesion ( below knee )
Type 1 ( anterior tibial nerve injury )
Lost : tibialis anterior , extensor hallucis longus , extensor
digitorium longus
Sensation : over first web space is lost
Type 2 ( musculocutaneous nerve injury ):
Spared : all the above muscles innervated by anterior tibial nerve
Lost : peroneous longus and brevis
Sensation : over outer leg and foot
Clinical features
Foot drop :
Complete (sciatic or lateral popliteal nerve injury)
Incomplete (superficial or deep peroneal nerve )
High lesions ------total foot drop
Low lesions ------ incomplete foot drop
Low lesions
Type 1 :
Dorsiflexion and inversion is not possible
Front of the leg is wasted
Sensation over the dorsal web space is lost
Type 2 :
Cannot evert but can dorsiflex and invert the foot
Wasting of the outer half of the leg
Sensation lost over outer leg and foot
Gait : - high stepping gait is characteristic .
Treatment
Braces or splints.
Physical therapy.
Nerve stimulation :
In some cases, a small, battery-operated electrical stimulator is
strapped to the leg just below the knee.
In other cases, the stimulator is implanted in the leg.
Surgery.
Tendon transfer ( for mobile foot drop )
Tendon – Achilles lengthening ( in fixed )
Treatment
Different types of braces (also known as ankle-foot orthotics or AFOs)
are used .
Two standard motions thatoccur at the ankle joint –
“dorsiflexion” and “plantarflexion”.
Plantarflexion (toes point downward ).
 Dorsiflexion ( foot points upward ).
Dropfoot ( partial or complete weakness of the muscles that
dorsiflex the foot at the ankle joint ).
Types of AFOs
Short leg fixed AFOs
Dorsiflexion assist short leg AFOs
Solid ankle AFO (with or without posterior stop). Also available
with dorsiflexion assist.
Full leg posterior leaf spring AFO
Short Leg AFO with Fixed
Hinge (doesn’t flex at ankle joint)
Dorsiflexion Assist AFO (dorsiflex the ankle)
Plantarflexion Stop AFO
Solid AFO (stops plantarflexion and also stops or limits
dorsiflexion).
Posterior Leaf Spring AFO
Patients who have instability of the knee along with their dropfoot.
Brachical plexus Injuries
Causes
Closed injury :
Due to birth or
Due to bike trauma
Open injury :
Due to penetrating or gunshot injuries
Others ( less common )
Traction injuries
Tumor removal
Shoulder dislocations
Surgical excision of cervical ribs
Abnormal pressures due to faulty posture
Types of lesions
Supraclavicular lesion:
Preganglionic lesion :
Cause could be either birth or bike trauma
Characteristic feature :
Presence of Horner`s syndrome.
Postganglionic lesion : -
absence of Horner`s syndrome
prognosis is slightly better than the preganglionic lesion
positive Tinel`s sign ( tapping above the clavicle , produces
tingling sensation in the anaesthetic limb )
Horner`s syndrome
Remember ( 5 P`s ) : -
Ptosis of the eyelid
Pupils which are small and constricted
Protrusion of the eyeball which is slight
Pain even at rest
Poor prognosis
Assessment of brachial plexus injury
In preganglionic lesion
Horner`s syndrome – present
Unable to elevate scapula
In postganglionic lesion
Horner`s syndrome – absent
Able to elevate scapula
Tinel`s sign - present in the later stages
Investigation
X – ray ( to rule out # )
CT scan ( study cross – section anatomy )
MRI ( study the soft tissue damages )
Electromyogram (EMG or electromyography)
Nerve conduction study
Physical Therapy
Intervention
1 . Splinting
A-Aeroplane splint
B-Shoulder sling : to protect shoulder joint in peripheral nerve
injuries as( axillary nerve)
C-cook up splint : in Radial nerve injuries.
D-Ankle foot orthosis : in Common peroneal nerve lesion.
E-Finger splint : in Ulnar nerve lesion to correct hyperextension
of MCPjoints and correct flexion in IPJ joints.
2 . For pain control :
1-Electro therapy:
A-TENS
B- Interferntial current.
C- Continuous Ultrasound : for proximal affection.(5 min)
D- Deep cold laser (Infra red laser)(3 min)
E-Hot pack & Infrared : to maintain skin visibility ( must have
intact superfacial sensation to avoid burn).
3-Motor retraining
A-Passive movement for the affected joints.
B-Facilitation for paralysed muscles by
Tapping on the muscles.
Quick stretch.
Breif ice application.
Squeezing the muscles.
P.N.F techniques : Resist strong proximal muscles to
facilitate waek distal muscles using quick stretch.
Jendrassic maneuveur : Firing of all motor neuron pool.
C- Electrical stimulation : Faradic stimulation, used for muscle
re-education ,nerve stimulation .
4- Sensory re education :
Protection of desensitized area to avoid burn & injuries.
B-Brushing skin with different materials as :cotton –silk ….
 C-Occlouded vision : ask to recognize different objects ( sharp –
smooth )
D- Occlouded vision : ask to recognize quantity of material by
touch.
Surgical measures
Types of surgery
Nerve graft : -
the damaged part of the brachial plexus is removed and replaced
with sections of nerves cut from other parts of body
Nerve transfers
Done in the most serious types of brachial plexus injuries, called
avulsions, when the nerve root has been torn out of the spinal
cord.
Muscle transfers
Needed if arm muscles have Atrophied from lack of use.
Erb's palsy
paralysis of the muscles in a baby's arm, caused by injury of the
nerves in the shoulder at birth (during delivery).
The baby lies with one arm and hand twisted backward and does
not move the arm as much as the other.
If the full range of motion of the arm is not kept through regular
exercise, contractures will develop.
Clinical features
At the shoulder :
Loss of shoulder abduction and external rotation ( due to
paralysis of the deltoid , supra and infraspinatus and teres
minor muscles )
At the elbow :
Loss of flexion of the elbow joint ( due to paralysis of the
biceps and brachialis )
At the forearm :
 Loss of supination of the forearm
May be sensory loss on the outer aspects of the arm and
forearm both in the front and back .
Policeman or Waiter`s tip
Shoulder --- internally rotated
Elbow ----- extension
Forearm --- pronated
Wrist ------ flexion
Treatment
1 . Splinting
Aeroplane splint
2 . For pain control :
TENS method
Types of surgery
- Nerve graft .
- Nerve transfers .
- Muscle transfers .
- release of soft tissue contractures .
With the baby, start range-of-motion exercises 2 times a day.
When the child is old,, have him do exercises himself,, for range
off motion and to increase strength..
Entrapment neuropathies
INTRODUCTION
The nerve is injured by
1. chronic direct compression,
2. angulations
3. stretching forces causing mechanical damage to the nerve.
DEFINITION
Entrapment Neuropathy is defined as: Pressure or Pressure induced
injury to a segment of a peripheral nerve secondary to anatomical or
pathological structures

TYPES
NERVES SITE
Median N.(wrist) Carpal tunnel
(elbow) Btwn heads of pronator teres
Ulnar N. (wrist) Guyon’s canal( ulnar tunnel)
(elbow) Bicipital groove,cubital tunnel
Lower trunk or medial cord of Cervical rib or band at thoracic
branchial plexus outlet
Suprascapular N Spinoglenoid notch
Post.interosseous N Radial tunnel—at point of
entrance into supinator
Muscle (arcade of Frohse)
Common Peroneal nerve Fibular tunnel
Lateral femoral cutaneous Inguinal ligament
(meralgia paresthetica)
Posterior tibial Tarsal tunnel; medial
malleolus–flexor
Retinaculum
Interdigital plantar (Morton Plantar fascia: heads of third
metatarsalgia) and fourth metatarsals
Obturator Obturator canal

PATHOPHYSIOLOGY
Focal slowing of Nerve conduction is the principal
electrophysiological feature of entrapment neuropathy
 Mild degrees of pressure(suprasystolic) applied to the nerve for
short periods produce reversible dysfunction d/t
ischemia(entrapped nerve more sensitive to ischemia than normal
nerve)
Acute ischemia may be responsible for paresthesias and
dysethesias
Prolonged ischemia may l/t neural tissue infarction
Relevance
Epineurium protects against compression
Epineurium and perineurium protect against stretch
NEUROPRAXIA:Segmental axonal conduction block
CONDUCTION SLOWING:(in the absence of histological
change)
Myelin is slightly damaged,widening of nodal areas(NOT
destruction of internodal segment)-longer time to activate
Conduction is slowed,but not completely blocked
Characteristic of Entrapment Neuropathies(Old
term:Axonostenosis)
DOUBLE CRUSH SYNDROME
A proximal level of nerve compression could cause more distal
sites to be susceptible to compression.
The summation of compression along the nerve would result in
alterations of axoplasmic flow
The possibility of a distal site of compression making the more
proximal nerve susceptible to secondary compression: A reverse
double crush.
Systemic diseases such as obesity, diabetes, thyroid disease,
alcoholism, rheumatoid arthritis and neuropatthies lower the
threshold for the occurrence of a nerve compression and alter
axoplasmic transport rendering that nerve more susceptible to
develop compression neuropathy and act as a ‘crush’.
CLINICAL FEATURES
Either or all
Pain
Numbness
Tingling
Burning
Weakness
Muscle wasting(severe cases) in respective anatomical areas
DIAGNOSIS
Electro diagnosis: mainstay
Nerve Conduction studies(NCS)
Electromyography(EMG)
NCS assess integrity of sensory and motor neurons
EMG assess electrical activity of a muscle from a needle inserted
into a muscle
CARPEL TUNNEL SYNDROME
Median Nerve: Position and Morphology
Round or oval at distal radius level
Elliptical at the pisiform and hamate
Morphology changes with flexion and extension
Wrist flexion :elliptical shape flattens
Wrist extension :least morphological change
Frictional forces btwn the median N.adjacent tendons and the
transverse carpal lig compounded by morphologic changes
irritate nerve
Mechanism:
demyelination f/b axonal degeneration.
Sensory and autonomic fibers affected before motor
Epidemiology:
F:M::3-10:1,Age peak 45-60yrs
Etiology:
Aging,female,Increased BMI,Square shaped wrist,short
stature,dominant hand ,white race,caffeine,alcohol, nicotine
Linked to body morphology,DM,thyroid disease,hereditary
neuropathies,RA,Acromegaly,Amyloidosis
High amounts of repititive wrist movements and exposure to
vibration/cold
Lack of aerobic exercise,preg,BF,Use of wheelchairs,walking
aids,recent menopause,renal dialysis(elbow positioning during
dialysis, upper extremity vascular-access, and underlying disease
is one cause of ulnar entrapment.)
Clinical features:
PAIN: aching over ventral wrist extending distally to finger and
proximally to forearm
SENSORY: hyperasthesias, parasthesias
Muscle atrophy and weakness are late findings
Autonomic changes: Increase sensitivity to temp changes
Intermittent sym and increase with driving,reading the paper,
crocheting, painting
Diagnosis:
SPECIAL:
Hoffman Tinel,Phalen,Reverse Phalen,carpal compression
test,square wrist sign
USG more cost effective and non invasive-may detect minute
details which Electrophysiology may miss
Lacks standardization
ELECTRODIAGNOSIS
1st LINE INVESTIGATION
Prognosticates severity and used to follow disease process over
time
Positive in >90 % pts. with clinical CTS
Treatment
Physical therapy
Aerobic exercise, Modalities (iontophoresis, phonophoresis,
ultrasound)
Occupational therapy
Work site ergonomic assessment (posture)
Wrist-hand orthosis(worn at night for 3-4 wks)
Stretching/strengthening
Pharmacotherapy:
NSAIDS, diuretics, steroids, Vit B6/12-no proven benefit, reduce
caffeine, nicotine, alcohol intake
Local 40mg methyl pred inj results in significant improvement in
mild CTS

Surgery:
release of transverse carpal lig
Indicated for failure of conservative care or severe category at
presentation
Open vs endoscopic
OTHER MEDIAN NERVE SYNDROME
Pronator syndrome
Clinical presentation:
The patients complain of forearm pain. There is usually no history
of trauma. However, there is often a history of repetive pronation
(rotating forearm palm up to palm down) and wrist flexion such as
incurred by carpenters, frequent computer users eith mouse,
weight lifters, athelets – especially yoga, golf and tennis
Cause:
Compression of the median nerve, which can occur in severe sites
Bicipital aponeurosis
Between the two heads of pronator teres
Compression is often due to hypertrophy
Development of fibrous tissue due to inflammatory process
Treatment:
Rest
Ice
Therapy
Elevation
Manual therapy
Increase fluid flow
Decrease tension
Decrease inflammation
Breakup of fibrous blockage and adhesions
Rebalancing up of muscle, tendons and ligaments
 Reducation and exercise
Anterior interosseous nerve syndrome
Site of compression essentially same for both Pronator
syndrome(PS) and AIN
PS: Vague volar forearm pain, Median nerve parasthesias,
minimum motor findings
AIN: Pure motor palsy of any or all three 1.FPL, 2.FDP of index
and middle fingers, 3.PQ.
Surgical indications for nerve decompression include persistent
symptoms for >6 months in patients with PS or for a minimum of
12 months with no signs of motor improvement in those with AIN
syndrome cubital tunnel syndrome
Mechanism:
Repititive bending or leaning on elbow for long periods
Fluid build up in the elbow
Trauma
All of these cause narrowing and constriction of the nerve
Symptoms:
Aching pain on the inside of elbow
Numbness, tingling ring and index finger esp when bending the
elbow
Weakening of grip,difficulty in finger coordination,muscle
wasting- when more severe compression
Diagnosis:
Electromyography
Motor conduction velocities
Treatment:
In situ or simple decompression
Incising the aponeurotic arch between the olecranon and medial
epicondyle if conservative treatment fails
In situ decompression is simple and does not influence the blood
supply of the ulnar nerve
Second, it is also effective because it addresses the primary focus
of the lesion, the cubital tunnel.
Third, it has lower rate of postoperative complications and more
opportunities for quicker rehabilitations
Simple decompression, however, is not appropriate in a poor bed,
severe cubitus valgus, or a subluxing nerve
GUYON’ S CANAL ENTRAPMENT
Typically in cycling,wt lifters,jackhammers
Seen also in hook of hamate compression of ulnar nerve at
Guyon’s canal
Symptoms may be motor or sensory:
Feeling of pins and needles in the ring and little fingers, which is
often noticed in the early morning
This may progress to a burning pain in the wrist and hand
followed by decreased sensation in the ring and little fingers.
The hand may become clumsy when the muscles controlled by the
ulnar nerve become weak.
Treatment:
Proper bicycle fitting, handlebar adjustments, frequent change in
hand position, handle bar and glove padding
Wrist splints
Surgical decompression from failed non-op mgmt., especially with
structural lesions such as hook of hamate fracture
RADIAL NERVE ENTRAPMENT SYNDROME
Radial nerve entrapment at one of 5 sites
Anatomy:
posterior cord to emerge between long and lateral heads of
triceps, spiral groove of humerus proceeding medially to laterally
to emerge between brachialis and brachioradialis on lateral elbow
to enter the radial tunnel
Susceptible: Racquet sports, rowing and wt. Lifting
Symptoms:
Sensory and motor complaints, although typically less weakness
than with Posterior interosseous Nerve entrapment
Dull, deep lateral elbow pain
Tenderness over extensor muscle group
Pain reproduced with resisted forearm supination with elbow
flexed
May mimic or coexist with lateral epicondylitis
Treatment:
Conservative neural mobilization techniques
Neural mobilization is a manipulative technique by which neural
tissues are moved, relative to their surroundings
Surgery for persistent symptoms usually involves releasing the
entrapped location
POSTERIOR INTEROSSEOUS NERVE SYNDROME
PIN is a branch of the radial nerve, originating in the lateral
intermuscular septum
 Purely motor function
Innervates the supinator
Most common in racquet sports, bowlers, rowers, discus throwers,
golfers, swimmers
All involve repetitive supination and Pronation
Specifically, pain with resisted supination;
EMG/NCS may be helpful to differentiate between lateral
epicondylitis and PIN
Rx:minimize supination during Rehabilitation
SUPRASCAPULAR NERVE ENTRAPMENT
Throwers, other overhead athletes and weight-lifters
Arises from superior trunk of brachial plexus
Innervates supraspinatus and infraspinatus
Compression most commonly suprascapular or spinoglenoid notch
Etiology:
Notch narrowing
Ganglion cyst from intraarticular defect
Often indicative of a labral (SLAP) tear
Nerve kinking or traction from excessive infraspinatus
motion
Superior or inferior (spinoglenoid) transverse scapular
ligament hypertrophy causing compression
Clinical features:
Vague posterior shoulder pain, weakness and fatigability
Weakness/atrophy without pain often suggests compression at
spinoglenoid notch (nerve purely motor beyond this)
Symptoms may mimic rotator cuff pathology or instability
Exam reveals rotator cuff weakness and possibly supra- and/or
infraspinatus atrophy
Diagnosis:
MRI may exclude rotator cuff tears, demonstrate atrophy and/or
reveal a ganglion or space-occupying lesion- if present, strongly
consider surgical excision
NCS/EMG may assist with the diagnosis
Typically begin with non-operative mgmt.
Treatment:
Rest from repetitive hyperabduction
NSAIDs and corticosteroid injections considered
Nonresponders may benefit from a spinoglenoid notchplasty,
transverse scapular ligament release, nerve decompression or
surgical exploration
THORACIC OUTLET SYNDROME
The thoracic outlet syndrome is a group of symptoms arising not
only from the upper extremity, but also from the chest, neck and
shoulders. The symptoms are produced by a positional,
interminnent compression of the brachial plexus anterior
subclavian artery and vein
Soft tissue lesions:
Scalene mucle
Subclavicular muscle and cstoclavicular ligament
Pectoralis minor muscle
Body lesions:
Cervical rib
First rib anomalies
Long c7 transverse process
Clavicular anomalies
Symptoms:
Neural symptoms:
Peripheral
Pain
Paresthesia
 Motor weakness
Atrophy
Sympathetic
Raynauds phenomenon
Pain
Color and temperature
Ischemia
Trophic changes
Vascular symptoms:
Subclavian artery
Loss of pulse
Motor weakness
Claudication
Thrombosis
Peripheral emboli
Subclavian vein
Pain
Edema
Venous distension
Cyanosis
Collateral vein
Paget schroetter syndrome
Investigations:
Plain films may reveal a cervical rib or exuberant callus from a
clavicle/upper rib fx
MRI and MRA can reveal brachial plexus anatomy, subclavian
vein anatomy or vascular occlusion/compression
MRA with the arm in abduction can demonstrate subclavian vein
obstruction in baseball pitchers
Treatment:
Nonoperative treatment focuses on rest, stretching of the nearby
soft tissue structures and posture mechanics; gradual
improvement
Injection of botulinum toxin into the muscles of the thoracic outlet
(scalenes, pectoralis minor, subclavius) has potential for
obtaining long-term symptom relief, but further research is
needed.
Surgical treatments:
Rib resection
Brachial plexus neurolysis and sympathectomy
Effort thrombosis also treated with clot lysis with urokinase or
heparin
MERALGIA PARASTHESIA
Mechanism of injury:
Compression (entrapment)may occur at the point where it passes
between the two prongs of attachment of the inguinal ligament.
Clinical features:
numbness,mild sensitivity of the skin,or occasionally persistent
burning
Perception of touch and pinprick are reduced in the territory of
the nerve; there is no weakness of the quadriceps or diminution of
the knee jerk.
The symptoms are characteristically worsened in certain
positions and after prolonged standing or walking
Diagnosis:
The sensory response is absent in 71% of patients with meralgia
paresthetica and is prolonged in 24%
Electromyographic test results with needle are normal which may
help to differentiate it from an upper lumbar radiculopathy
Treatment:
Weight loss
 Adjustment of restrictive clothing or correction of habitual
postures
Neurectomy of the nerve, Hydrocortisone
PIRIFORMIS SYNDROME
Piriformis syndrome (false sciatica)because instead of actual
nerve irritation, it is caused by referral pain. caused by tight knots
of contraction in the piriformis muscle,
Sciatica refers to irritation of the sciatic nerve, that arises from
nerve roots in the lumbar spine. The most common cause of “true”
sciatica is compression of one or more of its component nerve
roots due to disc herniation or spinal degeneration in the lower
lumbar region
OBTURATOR NERVE ENTRAPMENT
Etiology
During delivery as a result of compression of the nerve between
the head of the fetus and the bony structures of the pelvis,
As a consequence of compression of the nerve between a tumor
and the bony pelvis. in the obturator canal during surgery or with
total hip arthroplasties.
Malposition of the lower limb for prolonged periods, entrapment
in the adductor magnus in athletes,
Clinical symptoms:
Difficulty with ambulation and the development of an unstable leg.
Diagnosis:
Membrane instability (positive sharp waves and fibrillation
potentials) will occur within 3 weeks of the nerve injury, and
needle examination should be performed on patients with groin
pain of longer than 3 months
Treatment
With physical therapy, cryotherapy or a transcutaneous electrical
nerve stimulation (TENS) unit may be tried.
 "TENS" is the acronym for Transcutaneous Electrical Nerve
Stimulation. A "TENS unit" is a pocket size, portable, battery-
operated device that sends electrical impulses to certain parts of
the body to block pain signals. The electrical currents produced
are mild, but they can prevent pain messages from being
transmitted to the brain and may raise the level of endorphins
(natural pain killers produced by the brain).
TARSEL TUNNEL SYNDROME
Mechanism of injury:
Thickening of the tendon sheaths,or connective tissue or
osteoarthritic changes
Clinical features
Tingling pain and burning over the sole of the foot develop after
standing or walking for a long time
Diagnosis:
EMG and NCV testing values include the following:
Prolonged distal motor latency: Terminal latencies of the
abductor digiti quinti muscle (lateral plantar nerve) longer than
7.0 ms are abnormal.
Terminal latencies of the abductor hallucis muscle (medial plantar
nerve) longer than 6.2 ms are abnormal.
Fibrillations in the abductor hallucis muscle may be present.
Treatment:
Rest, NSAIDs, corticosteroid injection
Footwear adjustments, including a medial arch support
Surgical release ~75% success rate
MORTONS METATARSALGIA
Mechanism of injury:
perineural fibrosis and nerve degeneration due torepetitive
irritation
Incidence:
occurs most frequently in women (F:M 8:1) aged 40-50 who wear
high-heeled, pointed-toe shoes
Clinical features:
Common digital nerve to the third/fourth metatarsal spaces is
most often affected pain is only felt when the patient wears shoes.
There is localized tenderness over the site of the neuroma
Diagnosis:
USG is the modality of Choice
Treatment:
If there is no relief from symptomatic padding then the neuroma
may be excised
PERIPHERAL NEUROPATHY
DEFINITION
Generalized term including disorders of any cause affecting PNS
May involve sensory nerves, motor nerves, or both
May affect one nerve (mononeuropathy), several nerves together
(polyneuropathy) or several nerves not contiguous
(Mononeuropathy multiplex)
TYPES OF PERIPHERAL NEUROPATHY
Type – I
Neuropathies might be acute or chronic
Mononeuropathy – affecting a single nerve
Polyneuropathy – diffuse, symmetrical disease usually starting
peripherally.
Mononeuritis multiplex – affects several or multiple nerves.
Radiculopathy – disease affecting nerve roots
Peripheral Neuropathy can affect:
Sensory pathways
Motor pathways
 Autonomic pathways
Type – II
Neuropraxia
Axonotmesis
Neurotmesis

MECHANISM OF PERIPHERAL NERVE INJURY

There are 6 possible mechanisms of peripheral nerve degeneration
Demyelination – e.g. Guillain-Barre Syndrome
Axonal degeneration - e.g. toxic neuropathies
Wallerian degeneration
Compression – e.g. carpal tunnel syndrome
Infarction – e.g. diabetes
Infiltration – e.g. leprosy and granulomas
CAUSES OF PERIPHERAL NEUROPATHY
The causes of peripheral neuropathy are often unknown but the two
main causes are:
Diabetic Neuropathy
Nutritional, including alcohol (B1 deficiency)
Other causes
Infection – HIV, leprosy, diptheria, tetanus, botulism
Heavy metal poisoning e.g. Lead and mercury
Malignancy
Metabolic – hypothyroidism, liver failure, renal failure
Postinfective polyneuritis – Guillain-Barre Syndrome
Sarcoidosis
Drugs – isoniazid, vincrinstine, phenytoin, gold, excess vitamin B6
Congenital – Charcot-Marie Tooth syndrome
MONONEUROPATHIES
Peripheral nerve compression and entrapment
Carpal tunnel syndrome is a common mononeuropathy – Median
nerve entrapement
Clinical presentation
Pain, tingling and paraesthesia on palmar aspect of hand and
fingers
Weakness of thenar muscles and wasting of abductor pollicis
brevis
Nocturnal
Pain may extend to arm and shoulder
Tinel’s and Phalen’s tests are positive.
SENSORY NEUROPATHY
Paraesthesia
Numbness
Burning pain
Loss of vibration sense and position sense
Difficulty using small objects e.g. needles
Subacute with ataxia due to loss of sense of posture
Feet are usually affected first – Sock and Glove
AUTONOMIC NEUROPATHY
Clinical Presentation:
Postural hypotension
Urinary retention
Erectile dysfunction
Diarrhoea/constipation
Diminished sweating
Impaired pupillary response
Cardiac arrhythmias
Might occur in:
Diabetes
Amyloidosis
Guillain-Barre syndrome
MOTOR NEUROPATHY
Clinical presentation:
Progressive weakness or clumsiness
Difficulty walking (falling or stumbling)
Respiratory difficulties (falling vital capacity)
Wasting
Foot or wrist drop might be seen
Reflexes absent or reduced
DIABETIC NEUROPATHY
Diabetic neuropathies are nerve disorders associated with diabetes,
which affect approximately half of all diabetes patients. The most
common complication of diabetes is caused by hyperglycemia which
can damage nerve fibers throughout the body.
Types of Diabetic neuropathy
Symmetrical mainly sensory neuropathy
Acute painful neuropathy
Mononeuropathy and mononeuritis multiplex
Diabetic amyotrophy
Autonomic Neuropathy
Factors
Total cholesterol
Triglyceride
Body mass index
HbA1c change degree
Smoking
Duration of diabetes mellitus
HbA1c level
Hypertension
Pathological mechanism
The pathological mechanism of diabetic neuropathy cannot be
explained with a single cause, and various hypotheseshave been
proposed below.
 Activation of polyol pathway.
Down-regulation of intracellulary myoinositol.
Dysfunction of protein kinase C.
Down-regulation of intracellular cyclic AMP.
Inhibition of Na+/K+/ATPase.
Degradation of nitric oxide.
Advance of protein glycation.
Increase of free radical.
Disorder of polyunsaturated fatty acid synthesis.
Disorder of prostaglandin synthesis.
Action attenuation of a nerve growth factor
Nerve blood flow degradation, nerve vascular resistance
enhancement
Symptoms
Sensory symptoms accompanying diabetic neuropathy, such as pain and
numbness, distress patients, and subsequent hypoesthesia leads to the
primary cause of lower limb amputation, diabetic gangrene. Diverse
symptoms of autonomic neuropathy markedly reduce the Quality of Life
(QOL) of patients.
Constipation, diarrhoea, gastric hypokinesia (dull feeling in the
stomach)
Dizziness (orthostatic hypotension)
Silent myocardial infarction: Myocardial infarction or angina
without chest pain
Dysuria
Erectile dysfunction
Non-symptomatic hypoglycaemia
Clinically, there are several disease types of diabetic neuropathy based
on the distribution of disorders and developmental pattern.
1) Hyperglycaemic neuropathy
Sensory / autonomic neuropathy.
Acute painful diabetic neuropathy.
 Focal and multifocal neuropathy
2) Symmetric polyneuropathy
Cranial neuropathy.
Thoraco-abdominal neuropathy
Focal limb neuropathy
Diabetic amyotrophy
Mixed forms
Tests and diagnosis:
Diabetic neuropathy is usually diagnosed based on your symptoms,
your medical history and a physical exam. During the exam, your
doctor is likely to check your muscle strength and tone, tendon reflexes,
and sensitivity to touch, temperature and vibration. Your doctor may
also conduct tests that include:
Filament test
Nerve conduction studies
Electromyography (EMG
Quantitative sensory testing
Autonomic testing
Treatment
Early-stage diabetic neuropathy can be improved by blood glucose
control alone, but it becomes intractable after progression to a certain
stage. Aldose reductase inhibitors are being developed for treatment
based on the metabolic disorder hypothesis of diabetic neuropathy, but
treatment with these drugs alone may be insufficient .
Blood glucose control
Antioxidants
Incretin
Regeneration therapy

VASCULITIC NEUROPATHY
Vasculitis is a systemic illness with inflammation in the blood
vessels.
 The inflammation may lead to occlusion of blood vessels and
subsequent ischemia in the organs and tissues.
When the inflammation is in the blood vessels supplying
peripheral nerves, patients may develop vasculitic neuropathy.
Symptoms
The symptoms of vasculitic neuropathy depend on the type and
location of the nerve fiber involved.
It often causes both sensory and motor dysfunction, which
manifest itself as unusual sensations (paresthesias), numbness,
pain and weakness of the muscles in the limbs.
Unlike many of the other peripheral neuropathies, vasculitic
neuropathy can be very asymmetric and affect one limb more than
the rest.
Diagnosis
Diagnosis of vasculitic neuropathy is based on history, clinical
examination and supporting laboratory investigations.
These include electromyography with nerve conduction studies,
skin biopsies to evaluate cutaneous nerve innervation, and nerve
and muscle biopsies for histopathological evaluation.
Treatment
Treatment of vasculitic neuropathies depends on controlling the
underlying inflammation in the blood vessels.
Since the underlying blood vessel inflammation is an autoimmune
disorder, vasculitic neuropathy often responds to
immunomodulatory therapies.
The patients are initially treated with corticosteroids. However,
long-term use of corticosteroids is associated with multiple
 complications and patients are often switched to “corticosteroid-
sparing” agents.
Acute exacerbations of symptoms can sometimes be treated with
intravenous immune globulins (IVIG) or plasma exchange.
Neuropathic pain due to vasculitic neuropathy can be treated with
anti-seizure medications, antidepressants, or analgesics including
opiate drugs.
In severe painful conditions patients may be referred to the
Blaustein Chronic Pain Clinic for a multidisciplinary approach to
pain management.
ALCHOHOLIC NEUROPATHY
Chronic alcohol abuse leads to polyneuropathy
Calf pain is common
Deficiency in thiamine due to alcoholism also causes neuropathy
Can lead to Wernicke-Korsakoss syndrome
Common presentation
Eye signs
Ataxia
Cognitive change
Delirium tremens
Hypothermia and hypotension

COMMON INVESTIGATIONS
Blood analysis
Urine analysis
CSF analysis
Nerve conduction study
EMG
Sensory threshold
Autonomic function test
Nerve biopsy
COMMON MANAGEMENT
Slow progression
Treat causative factors if possible
If rapidly progressing
IVIG
Immunomodulating agents
Symptom Management
Tricyclic antidepressants
Amitryptilin, nortryptilin
Calcium channel alpha-2-delta ligands
Gabapentin, pregabalin
SNRI’s
Duloxetine, venlafaxine
Topical Agents
Lidocaine, Capsaicin
Tricyclic antidepressants
Amitryptilin, nortryptilin
Calcium channel alpha-2-delta ligands
Gabapentin, pregabalin
SNRI’s
Duloxetine, venlafaxine
Topical Agents
Lidocaine, Capsaicin
Antiepileptic Drugs
Carbamazepine, phenytoin, lacosamide
SSRI’s
Opioid analgesics
Tramadol
Miscellaneous
Botulinum toxin
Mexiletine
Alpha lipoic acid
 Physical Therapy
Gait and balance training
Assistive devices
Safe environment
Footwear at all times
Foot hygiene
Spinal cord lesions
Paraplegia and quadriplegia
DEFINITION
Originates from Greek language
Para + plēssein means ‘strike at side’
Impairment in motor function of the lower extremities
With or without involvement of sensory system
Paraplegia - Complete paralysis
Paraparesis - Partial paralysis
CAUSES OF PARAPLEGIA
Cerebral
Spinal
Infection
Immuno-allergic
Inflammatory
Demyelinating
Heredofamilial
Toxic myelopathy
Vascular
Metabolic/nutritional
Tropical
Para neoplastic syndromes
 Physical agents
CEREBRAL
1. Causes in the Parasagittal Region
Traumatic
Depressed fracture of the vault of the skull
Subdural hematoma
Vascular
Superior sagittal sinus thrombosis
Inflammatory
Encephalitis
Meningo-encephalitis
Neoplastic
Parasagittal meningioma
Degenerative
Cerebral palsy
2. Causes in the Brain Stem
Syringobulbia and midline tumours.
These lesions arise in the midline
Involves the innermost fibers
Which supplies lower limbs
SPINAL
1. Intramedullary
Syringomyelia
Hematomyelia
Ependymoma
Glioma
Astrocytoma
2. Extramedullary
Intradural
Meningioma
 Neurofibroma
Arachnoiditis
Extradural
Extradural
Vertebral
Fracture or fracture-dislocation of the vertebra
Disc prolapse and spondylosis
Deformity of the vertebral (e.g. kyphoscoliosis)
Infective - Pott's disease
Congenital - Spina bifida
Neoplastic diseases - Primary(myeloma)/metastatic(leukemia)
Paravertebral – Abscess/hematoma/aortic aneurysm
INFECTIVE CAUSES
Bacterial
Acute - Staphylococcal (extradural or intradural)
Chronic - Tuberculosis/Syphilis
Parasitic
Hydatid
Cysticercosis
Schistosomiasis
Viral
Polio/Rabies/Herpes zoster/HIV
OTHERS
Inflammatory
Transverse myelitis
Myelomeningitis/Myeloradiculitis
Immuno Allergic causes
Post vaccinal – Rabies/tetanus/polio
Post exanthematous - Chicken pox/Herpes zoster
 Demyelinating
Multiple sclerosis/Neuromyelitis optica/SACD
CLASSIFICATION
Based upon nervous system involved and tone
Paraplegia is classified into
Spastic paraplegia
Flaccid paraplegia.
SPASTIC PARAPLEGIA
Weakness of muscles + increased tone
Occurs in UMN diseases
Loss of inhibition of contraction
Increased muscle tone
Exaggerated deep tendon reflexes
Plantar extension with or without clonus
Classified into
Paraplegia in extension
Paraplegia in flexion
Paraplegia in extension
Occurs in initial stages of lesion
Partial transection of spinal cord with Involvement of pyramidal
tract
Hypertonia is more in extensor group of muscles
Paraplegia in flexion
Occurs as the disease or lesion progresses further or
With complete transection of spinal cord
Extra pyramidal tracts get involved
More hypertonia in flexor group of muscles
Resulting in flexed posture of limbs
FLACCID PARAPLEGIA
Decreased tone and contractility of muscles + weakness.
It occurs in lower motor neuron diseases
Occurs due to
Loss of stimulatory innervation to muscle
Decreased muscle tone
Atrophied muscle
Absent deep tendon reflexes
Flexor or equivocal plantar with or without fasciculations
SEGMENTS/FUNCTION
C1-C6 - Neck flexors
C1-T1 - Neck extensors
C3-C5 - Supply diaphragm (mostly C4)
C5-C6
Raise arm(deltoid)
Flexion of elbows(biceps)
C6 - Externally rotates the arm(supinator)
C6-C7
Extends elbow and wrist
Triceps and wrist extensor
Pronates arm
C7-T1 - Flexes wrist
C7-T1 - Supply small muscles of hand
T1-T6 - Intercostal muscles
T7-L1 - Abdominal muscles
L1-L4 - Thigh flexion
L2-L4 - Thigh adduction
L4-S1 - Thigh abduction
L5-S2 - Extension of leg at hip (gluteus maximus)
L2-L4 - Extension of legs at knee(quad. femoris)
L4-S2 - Flexion of leg at knee (hamstrings)
 L4-S1 - Dorsiflexion of foot/Extension of toes
L5-S2 - Plantar flexion of foot/Flexion of toes
CLINICAL FEATURES
In cerebral paraplegia
Weakness of upper limbs and along with that
Mental retardation
Delayed milestones
Seizures
Altered sensorium
In Spinal paraplegia
Spasticity
Exaggerated DTR
Radicular pain
Depending upon level of spine
Dermatomal sensory involvement +
Specific motor weakness present
If peripheral nerve involvement occurs
Distal weakness
Sensory loss
Muscle atrophy
Absent tendon reflexes
EXAMINATION
Neurological examination
Higher mental function status
Affected in cerebral and degenerative diseases
Cranial nerve examination
Affected in brain stem leisons
Tone
Increased in UMN disease
Decreased in LMN disease
 DTR
Exaggerated in UMN leisons
Absent in LMN leisons and spinal shock
Sensory examination
To asses particular sensory level
To find the extent of sensory loss
Proper examination of skull and spine
To look for any localized tenderness
Depressed fracture
Deformity
SUPERFICIAL REFLEXES
D7 lesion
Abdominal reflexes lost in all four quadrants
Cremastric reflexes B/L lost
Plantar B/L extensor
D10 lesion
Abdominal reflexes lost in lower 2 quadrants
Cremastric reflexes B/L lost
Plantar B/L extensor
L1 lesion
Abdominal reflexes present in all four quadrants
Cremastric reflexes B/L lost
Plantar B/L extensor
SEGMENTS/SIGNS/SYMPTOMS
Foramen magnum & Upper cervical region
Severe pain in the occiput &neck
In hands
Loss of post. column sensation
Severe tingling/Numbness
Pain/weakness in the limbs/wasting may occur in the upper limb
 Decreased diaphragm movements
Compression of phrenic nerve
Lower cranial nerve involvement/medullary involvement can
occur
Descending tract of trigeminal can be involved
C5C6 segment lesion
Inverted supinator reflex
Wasting of muscles supplied by C5C6
Deltoid/biceps/brachioradialis/rhomboids
C8T1 Level
Wasting of small muscle of hands
Wasting of flexors of wrist & fingers
Horner’s syndrome
DTR of upper limbs preserved
Spastic paralysis of trunk & lower limbs
Cervical spondylosis never involves C8&T1
So small muscle wasting rules out cervical spondylosis
Mid Thoracic region of spinal cord
Upper limb normal
Wasting of intercostal muscles (supplied by involved segments)
Movements of diaphragm normal
Spastic paralysis of abdominal muscles &lower limbs
9th &10th thoracic segments
Lower abdominal muscles are weaker
Upper abdominal muscles are intact
BEEVER’S SIGN positive
when patient raises the head against resistance
umbilicus is drawn upwards
T12L1 segments
Abdominal reflexes preserved
 Cremastric reflex lost
Wasting of internal oblique & transverse abdominal muscle
L3 L4 segmental lesion
Flexion of hip is preserved
Cremastric reflex preserved
Quadriceps & adductors of hip are wasted
knee jerk is lost or diminished
But ankle jerk is present
Plantar - Foot drop
S1S2 segments
Wasting & paralysis of intrinsic muscles of feet
Wasting & paralysis of calf muscles, Plantarflexion impaired
But dorsiflexion of foot is preserved
In the hip all muscles of hip are preserved
Except flexors & adductors
In the knee flexors of knee are wasted
Knee jerk is preserved, ankle jerk is lost
Plantar reflex is lost. No foot drop
Anal & Bulbocavernous reflexes are preserved
S3S4 segments
Large bowel & bladder are paralysed.
There is retention of urine and feces due to
Unopposed action of internal sphincters
The external sphincters are paralyzed
Anal and bulbo cavernous reflex are lost
Saddle shaped anaesthesia occurs
No paraplegia
INVESTIGATIONS
Routine blood tests (CBC, PS, CRP & C/S)
Blood chemistry (blood urea, creatinine, electrolytes etc.)
 Routine urine exam, urine for culture and sensitivity
Plain X-ray Spine (Lateral and oblique view)
CSF Analysis
To R/O infection-bacterial/tubercular/viral meningitis
CSF culture and sensitivity testing
C.S.F.-Electrophoresis to show oligoclonal bands of multiple
sclerosis
CT Cranium/Brain
MRI brain is more informative than CT
It helps in diagnosing
Degenerative/neoplastic/vascular/infective lesions
Spinal MRI
Sagittal views - differentiates
Syringomyelia from intramedullary tumours/transverse myelitis
It also shows cord compression whether internal or external
Myelogram
CONDITIONS A/W PARAPLEGIA
Compressive myelopathy
Dorsal nerve root
U/L or B/L pain aggravated by sudden rapid body movement
Ventral nerve root
LMN type of paraparesis
Corticospinal tract
Produce weakness(asymmetrical)
Stiffness of lower limbs
Posterior column
Produce loss of position/vibration
Syringomyleia
Cystic dilatation of the spinal cord
Obliteration of the flow of CSF
 From within spinal canal to its point of absorption
Hematomyelia
Haemorrhage into the substance into spinal cord occurs due to
Trauma/vascular malformations/bleeding disorders/neoplasms/
Epidural abscess
Form anywhere along spinal canal
Two third from haematogenous spread of infection
One third from direct extension of local infections
Pott’s disease
Also known as spinal TB/tubercular spondylitis
Haematogenous spread in most cases
Small no. of cases from adjacent paravertebral lymph nodes
Anterior spinal artery infarction
It supplies the ant. two thirds of the spinal cord
Its infarction causes anterior cord syndrome
Paraplegia or quadriplegia
Dissociated sensory loss
Affecting pain and temp sensation
But sparing vibration and position sense
Loss of sphincter control
Subacute combined degeneration
AV malformation of spinal cord
Hereditary spastic paraplegia
Hydrocephalus
Parasagittal meningioma
Guillain barre syndrome
Transverse myelitis
MANAGEMENT OF PARAPLEGIA
1. General
Frequent change of posture to guard against bedsores
Care of skin
Frequent washing with alcohol and
Applying talc powder
Care of the bladder
If there is retention,
Use parasympathomimetic drugs
If this fails, use a catheter to evacuate the bladder
In case of urinary incontinence
Frequent change of bed-sheets
2. Physiotherapy
3. Symptomatic Treatment
Analgesics and sedatives for pain
Muscle relaxants for the spasticity
Vitamins and mineral supplementation
4. Specific Treatment (treatment of the cause)
ATT + supportive measures in Pott's disease
Drainage of paraspinal abscess
Traumatic spine stabilisation
Surgical management of some tumors
5. Rehabilitation
Management of complications
Occupational therapy
Gait retaining
Community re- integration
COMPLICATIONS
Bed sores
Contractures
 Urinary tract infection
Pneumonia
Deep venous thrombosis

NEUROGENIC BLADDER
INTRODUCTION:
Functions of the bladder;
• Storage - at low pressure until such time as it is convenient and
socially acceptable to void
• Voiding - initiated by inhibition of the striated sphincter and pelvic
floor, followed some seconds later by a contraction of the detrusor
muscle.
Control of micturition;
1. Cortical micturition centre
2. Pontine micturition centre
3. Spinal micturition centre – sympathetic (t11 – l2), parasympathetic
(s2, s3, s4)
4. Peripheral nerves – (s3, s4)

Cortical micturation centre(CMC)

Location: Paracentral lobule in the medial aspect of the frontoparietal
cotex
Function: Inhibitory to PMC
Dysfunction – loss of social control of bladder. The brain’s control of
the PMC is part of the social training that children experience at age 2 -
4 years
Pontine Micturition Centre (PMC)
Also called Barrington’s nucleus
• Lateral region
Function - continence, storage urine stimulation results in a powerful
contraction of the urethral sphincter
• Medial region
Function - micturition center stimulation results in decrease in urethral
pressure and silence of pelvic floor EMG signal, followed by a rise in
detrusor pressure.
Sacral reflex or Sacral/Primitive micturition centre (SMC/PMC)
1. Sacral parasympathetic nucleus
(SPN): S234- pelvic splanchnic nerves (nervi erigentes) arise from
2. Somatic – Onufoid nuclei
Collection of external urethral sphinter motoneurones
3. Levator Ani Motoneurones

Peripheral innervations

Stimulation
Parasympathetic (S 2-4)
Sympathetic (T11- L2)
Somatic ( S2 - 4)
response
Excitatory to detrusor, relaxes
sphincter - void
Inhibitory to detrusor, ↑trigone
& Urethral tone
Excitatory to the external sphincter
MICTURATION REFLEX:
Internal sphincter – no important role in micturition, prevents leakage
during filling and prevents reflux of semen into bladder during
ejaculation
Sympathetic nerves – no part in micturition

FLOW CHART:

Sensation of bladder via Frontal lobe decides social

pelvic puddendal nerve to s2, appropriateness
s3, s4

Periaqueductal gray matter

Medial Pontine micturition center

Detrussor center (S Onuf’s nucleus to

2,3,4) to pelvic pudendal nerves
nerves

RECIPROCAL ACTIVITY BETWEEN SPHINCTER & DETRUSOR

micturation
SYMPTOMOLOGY
Neuropathy
• Long history of neuropathic symptoms,
• Stocking glove anesthesia
• Absent knee and ankle jerks will be absent
• Small fiber sensory impairment demonstrable to the level of the
ankles
• Other features of autonomic involvement
• Sexual dysfunction
Cauda equina
• Bladder, sexual & bowel dysfunction
• S 2, 3, 4 sensory loss
• Lax anal sphincter
• Bulbocavernosus (sacral reflexes) reflex lost
 • +/- Foot deformities, lower limb abnormalities
• Cutaneous markers over the back & sacrum
Spinal Cord
• Signs of upper motor neuron lesion in the lower limbs (unless the
lesion is central intramedullary and small)
• Erectile dysfunction in men
• +/- Paraparesis
Brainstem
• Marked neurological deficits dorsal and discreet lesion defect of
bladder function
• MLF lesion
• Internuclear
• Ophthalmoplegia
Extrapyramidal diseases
• Extrapyramidal features
• MSA, Parkinsons disease
• Autonomic dysfunction
• Cerebellar signs
Suprapontine
• Frontal lobe disorders
• Dementia, personality change
• Aware about incontinence unless extensive lesions
• •Severe urgency, frequency & urge incontinence without dementia,
socially aware and embarrassed by incontinence
• Urinary retention

Types according to the level of bladder dysfunction

a) Suprapontine/cortical lesion –
“Uninhibited /Cortical bladder”
Severe urgency, frequency & urge incontinence
with dementia – incontinent and inappropriate voiding
without dementia- socially aware & embarrassed by their incontinence.
b) Pontine lesion – “ Reflex / Automatic bladder”
DH, Arreflexia in pts with INO
c) Spinal (subpontine/suprasacral) “ Spastic Bladder”
Disorders of storage and emptying DSD (true only if above T6 level),
DH
d) Sacral and subsacral lesions
I) Afferent fibres involved only –
“Atonic /Areflexic bladder”
• Overflow incontinence
• Straining for micturition
• No DSD, no DH
II) Both afferent and efferent involved –
“Autonomous bladder”
• Small capacity , acting of its own. No DSD/DH
Causes of various levels of dysfunction
a) Suprapontine and Pontine Causes
• Stroke
• Tumors
• Dementia (AD, FTD)
b) spinal causes
• Spinal cord injury
• Compressive disc disease
• Metastatic lesions
• Multiple sclerosis
• Post infectious
• Idiopathic
c) sacral and subsacral causes
causes of chronic cauda equine compression
• Extrinsic tumors
• Primary tumors arising from the cauda
• Spinal stenosis
• Chronic central disc heriniation
Polyneuropathies causing bladder, bowel and sexual dysfunction
• Diabetes mellitus
• Amyloidois
• Porphyria
• Gullian – barre syndrome
• Other chronic neuropathies
Investigations
Noninvasive bladder investigations-
Post void residual volume –
• In out catheterization,
• Ultrasound ( N is <100ml)
Uroflowmetry-
• Voided volume ( >100ml)
• Maximal flow, maximal and average flow rate (M > 20ml/sec and
F > 15ml/sec)
Cystometry-
• Measure detrusor pressure (Intravesical presure – Rectal
pressure)
• Bladder infused till 400 to 600ml – Pressure should not rise to
>15cm water (Stable bladder)
• Neurogenic detrusor overactivity – Involutary detrusor
contraction during filling phase
• Voiding phase – Detrusor pressure M < 50cm
• water F < 30cm water
Sphincter EMG –
• Reinnervation with prolonged duration of MUAPs
Neuroimaging –
• Cauda equina & conus lesions, spinal, supra pontine and pontine
lesions
Treatment - Detrusor overactivity
• Anticholinergics
- Oxybutynin, tolterodine
- M3 blockers- darifenacin
• Tricyclic antidepressants
- Imipramine
• Desmopressin intranasally – once in 24 hrs
• Botulinum toxin A
• Intravesical capsaicin – instilled with a balloon catheter
Treatment
Only Urinary Retention
• (If residual volume > 100ml)
• Clean intermittent self catheterisation (CISC)
• Permanent indwellingcatheter
Detrusor overactivity & Retention
• Anticholinergic drugs
• CISC
Treatment
• External device – condom catheter
• Sacral nerve stimulators – for DI
• Nerve root stimulators – S 2,3,4 for voiding assisting defecation
• Surgery – Augmentation cystoplasty, artificial sphincter, urinary
diversion with
• stoma collection bag
 Miscellaneous
EPILEPSY
INTRODUCTION:
Epilepsy is a group of syndrome characterized by
recurring seizures. Epilepsy syndrome are classified by specific pattern
of clinical features family and history an seizures type. Epilepsy is a
syndrome of another underlying condition such as brain tumor
DEFINITION:
Epilepsy can be defined as a chronic seizure disorder or
group of disorders characterized by seizure that usually recur
unpredictably in the absence of a consistent provoking factor
ETIOLOGY:
Agent factors:
• Exposure to toxins
• Fever
Host factor:
• Age: before the age 20
High during first year of life
Onset of puberty
• Genetic factor
• CVA
• Birth hypoxia
 • Brain tumor and abscess
• Congenital abnormalities
• Trauma
Environmental factors:
• Air pollution
• Unskilled handling at the time of birth
• Intrauterine infection
• Emotional disturbances
• Environmental stressors
• Drugs and alcohol intoxication
CLASSIFICATION:
1. Partial seizures:
a. Simple seizures:
• Without impairment of consciousness
• With motor symptom
• With special sensory or somato sensory symptom
• With psychic symptom
b. Complex seizures
• with impairment of consciousness
• simple partial onset followed by impairment of
consciousness
• impaired consciousness at onset
c. secondary generalized:
• partial onset evolving to generalized tonic – clonic
seizures
2. generalized seizures:
 a. absence:
• alteration of consciousness lasting 10 – 30 sec
• staring and loss in postural tone
• 100 or more daily
• Onset occurs from 3 – 16 yrs disappear in 40 yrs
b. Myoclonic:
• Sudden involuntary jerking of fascial, limb or trunk
muscles in rhythemic manner
c. Clonic:
• Sustained muscle contraction alternating with
relaxation
d. Tonic:
• Sustained muscle stiffing
e. Tonic clonic:
• Sudden loss of consciousness
f. Atonic:
• Sudden loss of postural tone patient fall to the ground
• Occur primarily in children
3. Unclassified seizures:
4. Status epilepsies:
RISK FACTORS:
• High fever especially in infants
• Drug use, alcohol withdrawal
• Near drowing or lack of oxygen from another cause
• Metabolic disturbance
• Head trauma
• Brain tumor
 • Complication of diabetes
• Stroke
• Brain infection
• Past head injury
• Genetic factors
PATHOPHYSIOLOGY:
Paroxysmal discharge in cortical neurons
A seizure originates from gray matter of any cortical or
sub cortical area
↓
Abnormal firing of neurons
↓

Breakdown of normal membrane conductance and inability synaptic

current
↓
↓ ↓
Locally widely
↓ ↓
Focal seizure generalized seizure
 ↓ ↓
Abnormally of potassium deficiency of inhibitory
Conductance defect in voltage neurotransmitters increase in
Sensitive ion channel deficiency in excitatory neurotransmitters
Membrane ATPase
↓ ↓
Normal membrane instability abnormal neural activity
↓ ↓
Seizure seizure
DIAGNOSIS:
• Electroencephalogram
• MRI
• CT
• Functional MRI
• Position emission tomography
• Single photon emission CT
MANAGEMENT:
Non medical management:
• Ketogenic diet
• Avoiding of precipitating factors
1. Stress
 2. Sleep deviation
3. Ingestion of excessive amount
• Promotion of emotional wellbeing
• Reduction of psychiatric co – morbidity
Medical management:
• Na+ channel blockers
• Ca+ channel blockers
• CABG activity is increased
• Decreased synaptic activity
Surgical management:
• Lobe resection
• Lesionectomy
• Corous callostomy
• Functional hemiphyrectomy
• Multiple subpial transaction
• Vagus nerve stimulation
• Responsive neuro stimulation device

MYASTHENIA GRAVIS
DEFINITION:
Myasthenia gravis is a autoimmune disorder affecting the
myoneural junction is characterized by varing degrees of weakness of
the voluntary muscles
ETIOLOGY:
• Autoimmune disease
• Thyroid gland abnormality
• In myasthenia gravis, the receptors at the muscle surface are
destroyed or deformed by antibiotics that prevent a normal
reaction from occurring
• The causative factor is unknown, but the disorder may have a
genetic link
• The most commonly affected muscles are those of eyes, swallowing
• It can result in double vision, drooping eyelids, trouble talking,
and trouble walking.
• Those affected often have a large thymus gland or develop a
thymoma.
INCIDENCE:
• 2 – 7 in 10,000 is having a prevelance
• Common in about 40 years
PATHOPHYSIOLOGY:
Due to etiological factors
↓
Lymphocyte produce acetylcholine receptor antibiotics that
attack the post synaptic muscle membrane
↓
Depletion of acetylcholine receptor of neuromuscular junction
 ↓
Defect in the transmission of impulse from nerve to muscle cell
↓
Myasthenia gravis
CLINICAL MANIFESTATION:
• Weakness of eye muscles
• Fatigue
• Flaccid paralysis
• Change in voice
• Drooping position of head
• Difficulty in talking
• Difficulty to climb stairs, lift weight
• Difficulty to chew and swallow
• Breathing difficulty
• Illnesss
• Stress
• Extreme heat
• Drooping of one or both eyelids
• Double vision
• Blurred vision
CLASSIFICATION:
Class 1: patient with ocular involvement alone
Class 2: mild muscular weakness, not incapacity
Class 3: moderate muscle weakness not incapacitating including
oropharangeal and respiratory muscle weakness
Class 4:incapacitating weakness of any muscle system , including
oropharangeal and respiratory muscle weakness
Class 5: life threatening respiratory insufficiency requiring ventilator
assistance
DIAGNOSIS:
• History of the patient
• Blood test
• Clinical and neurological examination
• Imagery test
• Tensilin test
• Endrophonium test
• Neurological test (electromyography)
MANAGEMENT:
Medical management:
• Cholinesterase inhibitors.
• Cortico steroids
• Immunosuppressant
• Plasmapheresis
• Intravenous immunoglobulin
Surgical management:
Thymectomy - surgical removal of the thymus gland. The role of the
thymus gland in MG is not fully understood, and the thymectomy may
or may not improve a child symptom
PREVENTION:
Because the cause of myasthenia gravis is unknown, there is no way to
prevent it. However, once the disease has developed, there may be
ways to prevent episodes of worsening symptoms or flare-ups:
• Give yourself plenty of rest.
• Avoid strenuous, exhausting activities.
• Avoid excessive heat and cold.
• Avoid emotional stress.

MOTOR NEURON DISEASE

INTRODUCTION:
• Motor neuron diseases are a group of neurodegenerative
disorders that affects the nerves in the spine and brain to
progressively lose its function.
• They are a rare but serious and incurable form of progressive
neurodegeneration.
• It is a condition that selectively affects the motor system, the cells
which control voluntary muscles of the body.
• The renowned English physicist Stephen Hawking lived with ALS
for many decades until his death in March 2018. Guitar virtuoso
 Jason Becker is another example of someone who has been living
with ALS for several years.

DEFINITION:
It is defined as a progressive disease that involves degeneration
of the motor neurons and wasting of the muscles.

INCIDENCE:
• Motor neuron disease (MND) occur in adults and children.
• It can appear at any age, but most patients are over 40 years old
at diagnosis.
• It affects men more than women.

TYPES OF MOTOR NEURON DISORDER:

• MNDs are classified according to whether they are inherited or
sporadic(i.e-anyone can get it), and to whether degeneration
affects upper motor neurons, lower motor neurons, or both. In
adults, the most common MND is Amyotrophic Lateral Sclerosis
(ALS).
• ALS, or Lou Gehrig's disease, is the most common type, affects
both the UMN and LMN. It can affect the muscles of the arms,
legs, mouth, and respiratory system. Mean survival time is 3 to 5
years, but some people live 10 years or more beyond diagnosis
with supportive care.
• Progressive bulbar palsy (PBP) involves the brain stem(LMN).
People with ALS often have PBP too. The condition causes
frequent choking spells, difficulty speaking, eating, and
swallowing.
• Pseudobulbar Palsy: This is similar to progressive bulbar palsy.
It affects motor neurons that control the ability to talk, chew, and
swallow.
 Pseudobulbar palsy causes people to laugh or cry with no
control.
• Progressive muscular atrophy (PMA): It only affects the LMN in
the spinal cord. Affects slowly but progressively causes muscle
wasting, especially in the arms, legs, and mouth. It may be a
variation of ALS.
• Primary lateral sclerosis (PLS): It is a disease of UMN. It is the
rare form of MND that advances more slowly than ALS. It is not
fatal, but it can affect the quality of life. In children, it is known as
juvenile primary lateral sclerosis.
• Spinal muscular atrophy (SMA) is an inherited MND that affects
children. There are three types, all caused by an abnormal gene
known as SMA1(This gene makes a protein that protects the
motor neurons. Without it, they die.). It tends to affect the trunk,
legs, and arms. Long-term outlook varies according to type.
• The different types of MND share similar symptoms, but they
progress at different speeds and vary in severity .
CAUSES:
The exact causes are unclear.
Some MNDs are inherited, but the causes of most MNDs are not
known.
About 5% of people with motor neurone disease have a close
family relative with the condition or a related condition known as
fronto temporal dementia.
This is called familial motor neurone disease which can be
hereditary or linked to a problem with genes that can cause
problems at a younger age.
• In sporadic or non inherited MNDs,
• environmental,
 • toxic,
• viral, or
• genetic factors likely play a role.

RISK FACTORS:
• HEREDITARY.
• AGE.
• SEX.
• Some experts have linked military experience to a higher chance
of developing the disease.
• Studies have found that professional footballers are more likely to
die from ALS, Alzheimer's disease, and other neurodegenerative
diseases, compared with other people. This implies a possible link
with recurrent head trauma and neurological disease.

CLINICAL MANIFESTATINS:
• MND can be divided into three stages; early, middle, and
advanced.
Early stage signs and symptoms:
• Symptoms develop slowly and can be confused with symptoms of
some other unrelated neurological conditions.
• Early symptoms depend upon which body system is affected first.
Typical symptoms begin in one of three areas: the arms and legs,
the mouth (bulbar), or the respiratory system.
• They include:
• a weakening grip, making it hard to pick up and hold things
• Fatigue, difficulty swallowing
• muscle pains, cramps, and twitches
• slurred and sometimes garbled speech
• weakness in the arms and legs
• increased clumsiness and stumbling
• trouble breathing or shortness of breath
Middle stage signs and symptoms:
• As the condition progresses, symptoms become more severe.
• Muscle pain and weakness increase, and spasms and twinges
worsen.
• Limbs become progressively weaker.
• Limb muscles start to shrink.
• Movement in affected limbs becomes more difficult.
• Limb muscles may become abnormally stiff.
• Joint pain grows.
• Eating, drinking, and swallowing become harder.
• Drooling occurs, due to problems controlling saliva.
• Yawning occurs, sometimes in uncontrollable bouts
• Jaw pain may result from excessive yawning.
• Speech problems worsen, as muscles in the throat and mouth
become weaker
• The person may show changes in personality and emotional state,
with bouts of uncontrollable crying or laughing.
• Secondary symptoms include insomnia, anxiety, and depression.

DIAGNOSIS:
• In the early stage, MND can be hard to diagnose, because the
signs and symptoms are common to other conditions, such as
multiple sclerosis (MS), an inflamed nerve, or Parkinson's disease.
• Blood test.
• Urine test.
• Mri.
• Emg(electromyography).
• Nerve conduction study.
• Lumbar puncture or spinal tap.
• Muscle biopsy.
• Transcranial magnetic stimulation.
MANAGEMENT:
• There is no cure for MND, so treatments focus on relieving the
symptoms, slowing the progression and maximizing patient
independence and comfort.
• This can include the use of breathing, feeding, mobility and
communication appliances and devices.
• Rehabilitation therapy may include physical, occupational and
speech therapy.
Medical management:
• Two drugs are currently approved by the U.S. Food and Drug
Administration (FDA) for ALS.
• Riluzole or Rilutek lowers the amount of glutamate in the body. It
appears most effective in the early stages of ALS and in older
individuals. It has been given to improve survival.
• In early 2017, the drug Radicava (Endaravone) was approved by
the FDA for the treatment of ALS.
• How it works is not well understood, but it may delay disease
progression by working against tissue damage.
• Scientists are currently exploring a possible role for stem cells in
the treatment of ALS.
• Muscle cramps and stiffness can be treated with physical therapy
and medications, such botulinum toxin (BTA) injections. BTA
blocks the signals from the brain to the stiff muscles for about 3
months.
• Scopolamine, a drug for motion sickness, may help control
symptoms of drooling. It is worn as a patch behind the ear.
• Antidepressants, called serotonin reuptake inhibitors (SSRIs), may
help with episodes of uncontrollable laughter or crying, known as
emotional ability.
• Advice from a speech and language therapist.
• Advice from a dietitian about diet and eating.
PROGNOSIS:
• Prognosis varies depending on the type of MND and the age of
onset.
• Some MNDs, such as PLS are not fatal and progress slowly.
• People with SMA may appear to be stable for long periods, but
improvement should not be expected.
• Some MNDs, such as ALS and some forms of SMA, are fatal.
ALZHEIMERS DIEASE
INTRODUCTION:
Alzheimer’s disease is an neurological brain disorder. It is the
most common cause of dementia, a group of disorders that impairs
mental functioning. It is progressive and irreversible.
Memory loss is one of the earliest symptoms, along with a
gradual decline of other intellectual and thinking abilities called
cognitive function and changes in the personality or the behavior.
Many scientists believe that Alzheimer’s disease results from
an increase in the production or accumulation of a specific protein in
the brain that leads to nerve cell death.
DEFINITION:
It is a slowly progressive disease of the brain that is
characterized by impairment of memory and eventually by disturbances
in reasoning, planning, language and perception.
EPIDEMIOLOGY:
• 3.5 million world wide have Alzheimer’s disease
 • Every 50 sec one gets affected due to Alzheimer’s in America
• Alzheimer’s is the 5th leading cause of death in 65 and older
• 1 in 6 women over 55
• 1 in 10 men over 55
ETIOLOGY:
• Idiopathic
• Cholinergic hypothesis
Caused by reduced synthesis of acetylcholine
Destruction of these neurons causes disruption in distant
neuronal networks
• Amyloid hypothesis
Abnormal breakdown, buildup of Amyloid beta deposits
Damaged Amyloid proteins build up to toxic levels, causing
call damage and death
• Genetics
• Environmental influence
• Trauma
• Dietary habits
• Lifestyle
• Blood pressure {high}
• Plagues – deposits of the brain beta-amyloid that accumulate in
the spaces between the nerve cells
• Tangles – deposits of the protein that accumulate inside of the
nerve cells
• Atrophy of cerebral cortex
• Enlargement of ventricles
• Family history
• Mutation in 4genes
• Decreased neurotransmitters
• increasing in the age
• down syndrome
• insulin resistance
• obesity
• high cholesterol
SYMPTOMS:
• difficulty in performing familiar tasks
• memory loss affects job skills
• problems using language
• disorientation of time and place
• loss of good judgment
• problems with abstract thinking
• misplacing things
• rapid mood swings
• personality changes
• loss of intiative, sleeping longer than usual, and loss of interest
CLINICAL MANIFESTATIONS ACCORDING TO THE TYPES:
1. MILD:
• Memory disturbance
• Poor judgement
• Irritability
• Agitation
• Suspicious
• Apathy
• Cognitive impairment
2. MODERATE:
• Language disturbance
• Impaired word finding
• Spontaneous speech
• Paraphasis
• Motor disturbance
 • Hyper orality
• Swallowing difficulty
• Depression
• Delusion
3. SEVERE:
• Communication disturbance
• Urinary, faecal incontinence
• Pneumonia
STAGES OF ALZHEIMER’S:
Stage 1: normal
Mentally healthy person
Stage 2: normal aged forgetfulness
Persons over the age of experience subjective complaints of
cognitive or functional difficulties
Stage 3: mild cognitive impairment
The capacity to perform executive functions also becomes
compromised. Commonly, for persons who are still woring, job
performance may decline
Stage 4: mild Alzheimer’s disease
The most common functioning deficit in these patients is a
decreased ability to manage instrumental activities of daily life.
Stage 5: moderate alzheimer’s disease
 This is manifest in a decrement in the ability to choose proper
clothing to wear for the weather condition and for the daily
circumstances
Stage 6: moderate severe alzheimer’s disease
At this stage, the ability to perform basic activities of daily life
becomes comprmised
Stage 7: severe alzheimer’s disease
At this stage, AD patients require continuous assistance with
basic activities of daily life for survival
DIAGNOSIS:
• Medical history
• Physical examination
• Neuropsychological testing
• MRI
• CT
• Mental state examination
• PET
TREATMENT:
MEDICAL MANAGEMENT:
• Memantine
• Galantamine
• Rivastigmine
• Donepezil
• Tacrine
 • Sodium valproate

DYSAUTONOMIA
INTRODUCTION:
• Much of the action of the body in maintaining, cardiovascular,
gastrointestinal and thermal homeostasis occurs through the
autonomic nervous system (ANS).
 • The ANS is our primary defense against challenges, to maintain
homeostasis. It provides involuntary control and organization of
both maintenance and stress responses.
DEFINITION:
• It is network of nerves & ganglia that controls involuntary
physiologic parameters & maintains internal homeostasis &
stress responses.
• It is primarily peripheral efferent system.
• Autonomic - self governing
FUNCTIONAL ANATOMY:

DIFFERENCE BETWEEN SOMATIC AND AUTOMATIC:

SOMATIC AUTONOMIC
Organ supplied Skeletal muscles All other organs

Distal most synapse Within CNS Outside the CNS

Nerve fibers Myelinated Preganglionic -
myelinated
Postganglionic- non-
 myelinated

Peripheral nerve Absent Present

formation
Efferent transmitter ACH ACH, nor adrenaline
Effect on nerve section Paralysis and atrophy Activity maintained,
in which organ is no atrophy
supplied

CENTRAL AUTONOMIC ORGANISATION:

Cerebral cortex is the highest level of ANS integration.
• The principal ANS organization is the Hypothalamus.
• SNS functions are controlled by nuclei in the postero-lateral
hypothalamus.
• PNS functions are governed by nuclei in the midline and some
anterior nuclei of the hypothalamus.
• The anterior hypothalamus is involved in regulation of
Temperature.
• The supra-optic hypothalamic nuclei regulates water metabolism.
DIFFERENCE BETWEEN SYMPATHETIC AND
PARASYMPATHETIC
SYMPATHETIC PARASYMPATHETIC
Origin Dorso lumbar Craniosacral (3,7,9,10,
s2-s4)
Distribution Wide Limited to head, neck
and trunk
Ganglia Away from the organs On / close to the organ
Post-ganglionic fibre Long Short
Post-ganglionic fibre 1:20-1:100 1:1 to 1:2 except in
Ratio entric plexus
Transmitter Nor adrenaline ACH
Acytylcholine
Stability of stable, differ for Ach rapidly
transmitter wider activity destroyed locally
Imp. Function Tackling, stress, and Assimilation of
emergency food,
conservation of energy

FUNCTION OF ANS:
Sympathetic
• “Fight or flight”
• “E” division
• Exercise, excitement, emergency, and embarrassment
Parasympathetic
• “Rest and digest”
• “D” division
• Digestion, defecation, and diuresis
AUTONOMIC DYSFUNCTION:
• Dysautonomia is a con dition in which the ANS malfunctions. It is
an umbrella term used to denote many ANS disorders. It is a type
of neuropathy affecting the nerves that carries information from
the brain and spinal cord to various visceral organs. The
diagnosis is achieved through the functional testing of the
ANS,focussing on the affected organ system.
CAUSES:
• Dysautonomia may be due to inherited or degenerative
neurological disorders[primary dusautonomia] or may be due to
injury of ANS due to an acquired disorder[secondary
dysautonomia
• Side effects of some drugs can cause abnormalities in the function
of ANS,producing an iatrogenic form of dysautonomia
• Diabetes mellitus
• Multiple sclerosis
• Parkinson’s disease
• HIV and AIDS
 • Amyloidosis
• Boutilism
• Pure autonomic failure
• Lyme disease amd Tuberculosis
• Lupus,Sjogen’s syndrome,sarcodiasis
• Chronic alcohol misuse
• Spinal cord injury
• Physical trauma or injury
CLINICAL MANIFESTATIONS:
• The primary symptoms present in patients with dysautonomia
include:
• Excessive fatigue
• Excessive thirst (polydipsia)
• Lightheadedness or dizziness, often associated with orthostatic
hypotension (abnormally low blood pressure on standing),
sometimes resulting in syncope (fainting)
• Rapid heart rate or slow heart rate
• Blood pressure fluctuations
• Difficulty with breathing or swallowing
• Shortness of breath with activity or exercise
 • Distension of the abdomen
• Mydriasis (abnormal dilation of the pupils) leading to blurry
vision
• Urinary incontinence or neurogenic bladder dysfunction
• Gastroparesis (delayed gastric emptying) with associated nausea,
acid reflux and vomiting
• Constipation
• Excessive sweating or lack of sweating (ahydrosis)
• Heat intolerance brought on with activity and exercise
• Sexual problems including erectile dysfunction in men and vaginal
dryness and orgasmic difficulties in women
TEST OF AUTONOMIC NERVOUS SYSTEM FUNCTION:
• These tests measure how the various systems in the body,
controlled by autonomic nerves, respond to stimulation. The data
collected during testing will indicate functioning of ANS.
• These tests help to identify patients with autonomic neuropathy
and is predictive of mortality and morbidity.
• Tests are done to monitor BP, blood flow, heart rate, skin
temperature and sweating.
TEST OF PARASYMPATHETIC SYSTEM
HR response with valsalva manoeuver
 • Valsalva manoeuver: valsalva ratio is an index of HR response to
BP changes that occur during valsalva manoeuver resulting from
mechanical and cardiovascular effects.
• Measure baseline HR and BP 3 minutes before this test
• Patient takes a deep inhalation, a complete exhalation, inhales
again and then blows into a mouthpiece for 15 seconds
• Expiratory pressure is maintained at 40 mm Hg. This pressure can
be measured by having the patient to exhale through a mouth
piece attached to a transducer
• BP and HR are measured throughout the manoeuver for 60
seconds after temination of the manoeuver
• An average of 2 trials are taken for analysis
• Caution while performing the test in elderly with pulmonary
disease who may not be able to perform the test satisfactorily
• As intraocular pressure is known to rise, the test must not be
performed in those with recent retinal surgery
• VR values are aggregated. There is a decrease with age.
DISEASES ASSOCIATED WITH PROGRESSIVE
NEUROLOGICAL IMPAIRMENT OF AUTONOMIC NERVOUS
SYSTEM
• They can be primary, familial or due to secondary systemic
disease or idiopathic.
Primary :
 • 1. Idiopathic Orthostatic Hypotension
• 2. Shy-Drager type of Orthostatic Hypotension
Familial :
• 1. Riley-Day Syndrome (Autonomic neuropathy in infants and
children)
• 2. Lesch-Nyhan Syndrome
• 3. Gill Familial dysautonomia
Secondary to systemic disease:
• Aging
• Diabetes Mellitus
• Chronic Alcoholism
• Chronic Renal Failure
• Hypertension
• Rheumatoid Arthritis
• Carcinomatosis
• Chaga's disease
• Tetanus
• Spinal cord injury – Transection
– Acute
 – Chronic
• Neurological diseases
– Tabes Dorsalis
– Syringomyelia
– Amyloidosis
TREATMENT OF ADVERSE AUTONPMIC REFLEXES:
• Atropine commonly used for both prevention and treatment.
• Topical anaesthesia can eliminate the reflex.
• Intravenous lidocaine is more effective than topical anaesthesia.
• Cessation of applied stimulus immediately.
• Vasopressors injected if there is persistent hypotensive response.
• Depth of anaesthesia should be increased. Most of the
intrathoracic and intraabdominal reflexes are observed during
surgery when anaesthesia is to light or relaxation is inadequate.