APPROACH TO THE PATIENT WITH

NEUROLOGIC DISEASE
THE NEUROLOGIC METHOD
LOCATE THE LESION(S)
The first priority is to identify the region of the nervoussystem that is likely to be responsible
for the symptoms.Can the disorder be mapped to one specific location,is it multifocal, or is a
diffuse process present? Are thesymptoms restricted to the nervous system, or do theyarise
in the context of a systemic illness? Is the problem in the central nervous system (CNS), the
peripheralnervous system (PNS), or both? The first clues to defining the anatomic area
ofinvolvement appear in the history, and the examinationis then directed to confirm or rule
out these impressionsand to clarify uncertainties.
DEFINE THE PATHOPHYSIOLOGY
Clues to the pathophysiology of the disease process may also be present in the history.
Primary neuronal (gray matter) disorders may present as early cognitive disturbances,
movement disorders, or seizures, whereas white matter involvement produces
predominantly ―long tract‖ disorders of motor, sensory, visual, and cerebellar pathways.
THE NEUROLOGIC HISTORY
Attention to the description of the symptoms experienced by the patient and substantiated by
family members and others often permits an accurate localization and determination of the
probable cause of the complaints, even before the neurologic examination is performed,
pertinent features of the history include the following:
1. Temporal course of the illness 6. Drug use and abuse and toxin
exposure.
2. Patients’ descriptions of the complaint. 7. Formulating an impression of the
patient
3. Corroboration of the history by others.
4. Family history.
5. Medical illnesses.
THE NEUROLOGIC EXAMINATION
There is no single, universally accepted sequence of the examination that must be followed,
but most clinicians begin with assessment of mental status followed by the cranial nerves,
motor system, sensory system, coordination, and gait.
MENTAL STATUS EXAMINATION
Individual elements of the mental status examination can be subdivided into level of
consciousness,
orientation, speech and language, memory, fund of information, insight and judgment,
abstract thought,
and calculations.
1. Level of consciousness is the patient‘s relative state of awareness of the self and the
environment, and ranges from fully awake to comatose. Can be assessed using
Glasgow coma scale.
2. Orientation is tested by asking the person to state his or her name, location, and time
(day of the week and date); time is usually the first to be affected in a variety of
conditions.
 3. Speech is assessed by observing articulation, rate, rhythm, and prosody (i.e., the
changes in pitch and accentuation of syllable and words).
4. Language is assessed by observing the content of the patient‘s verbal and written
output, response to spoken commands, and ability to read. A typical testing sequence
is to ask the patient to name successively more detailed components of clothing, a
watch, or a pen; repeat the phrase ―No ifs, ands, or buts‖; follow a three-step, verbal
command; write a sentence; and read and respond to a written command.
5. Memory should be analysed according to three main time scales:
a. immediate memory is assessed by saying a list of three items and having the
patient repeat the list immediately,
b. short-term memory is tested by asking the patient to recall the same three
items 5 and
15 min later, and
c. Long-term memory is evaluated by determining how well the patient is able to
provide
a coherent chronologic history of his or her illness or personal events.
6. Fund of information is assessed by asking questions about major historic or current
events, with special attention to educational level and life experiences.
7. Calculation ability is assessed by having the patient carry out a computation that is
appropriate to the patient‘s age and education (e.g., serial subtraction of 7 from 100 or
3 from 20; or word problems involving simple arithmetic).
CRANIAL NERVE EXAMINATION
The bare minimum: Check the fundi, visual fields, pupil size and reactivity, extraocular movements,
and facial movements.
1. CN I (olfactory) with eyes closed, ask the patient to sniff a mild stimulus such as
toothpaste or coffee and identify the odorant.
2. CN II (optic)
a. Check visual acuity (with eyeglasses or contact lens correction) using a Snellen
chart or similar tool.
b. Test the visual fields by confrontation, i.e., by comparing the patient‘s visual
fields to your own.
c. Optic fundi should be examined with an ophthalmoscope, degree of swelling or
elevation of the optic disc noted, The retinal vessels should be checked for
size, regularity, arterial-venous nicking at crossing points, haemorrhage,
exudates, etc.
3. CN III, IV, VI (oculomotor, trochlear, abducens)
a. Describe the size and shape of pupils and reaction to light and accommodation
(i.e., as the eyes converge while following your finger as it moves toward the
bridge of the nose).
b. To check extraocular movements, ask the patient to keep his or her head still
while tracking the movement of the tip of your finger.
4. CN V (trigeminal)
a. Examine sensation within the three territories of the branches of the trigeminal
nerve (ophthalmic, maxillary, and mandibular) on each side of the face
b. Testing of other modalities, the corneal reflex, and the motor component of CN
V (jaw
clench—masseter muscle)
 5. CN VII (facial)
a. Look for facial asymmetry at rest and with spontaneous movements. Test
eyebrow elevation, forehead wrinkling, eye closure, smiling, and cheek puff.
b. Look in particular for differences in the lower versus upper facial muscles;
weakness of the lower two-thirds of the face with preservation of the upper third
suggests an upper motor neuron lesion, whereas weakness of an entire side
suggests a lower motor neuron lesion.
6. CN VIII (vestibulocochlear)
a. Check the patient‘s ability to hear a finger rub or whispered voice with each ear.
b. Assessing vestibular nerve function in the setting of dizziness, coma
7. CN IX, X (glossopharyngeal, vagus)
a. Observe the position and symmetry of the palate and uvula at rest and with
phonation (―aah‖).
b. The pharyngeal (―gag‖) reflex is evaluated by stimulating the posterior
pharyngeal wall on each side with a sterile, blunt object (e.g., tongue blade),
but the reflex is often absent in normal individuals
8. CN XI (spinal accessory)
a. Check shoulder shrug (trapezius muscle) and (sternocleidomastoid) against
resistance.
9. CN XII (hypoglossal)
a. Inspect the tongue for atrophy or fasciculation, position with protrusion, and
strength when extended against the inner surface of the cheeks on each side.
MOTOR EXAMINATION
The bare minimum: Look for muscle atrophy and check extremity tone. Assess upper extremity
strength by checking for pronator drift and strength of wrist or finger extensors. Tap the biceps,
patellar, and Achilles reflexes. Test for lower extremity strength by having the patient walk normally
and on heels and toes.
The motor examination includes observations of muscle appearance, tone,strength, and
reflexes. Although gait is in part a test of motor function, it is usually evaluated separately at
the end of the examination
 Muscle strength is traditionally graded using the following scale:

0 = no movement
1 = flicker or trace of contraction but no associated
movement at a joint
2 = movement with gravity eliminated
3 = movement against gravity but not against resistance
4- = movement against a mild degree of resistance
4 = movement against moderate resistance
4+ = movement against strong resistance
5 = full power
 Reflexes
 Muscle stretch reflexes
 0 = absent 3 = exaggerated
1 = present but diminished 4 = clonus
2 = normoactive
 Cutaneous reflexes
 Primitive reflexes
SENSORY EXAMINATION
The bare minimum: Ask whether the patient can feel light touch and the temperature of a cool object
in each distal extremity. Check double simultaneous stimulation using light touch on the hands.
The five primary sensory modalities— are tested in each limb.
1. Light touch is assessed by stimulating the skin with single, very gentle touches of the
examiner‘s finger or a wisp of cotton.
2. Pain is tested using a new pin, and
3. Temperature is assessed using a metal object (e.g., tuning fork) that has been
immersed in cold and warm water.
4. Vibration is tested using a 128-Hz tuning fork
5. joint position
COORDINATION EXAMINATION
The bare minimum: Test rapid alternating movements of the hands and the finger-to-nose and heel-
knee-shin maneuvers
GAIT EXAMINATION
The bare minimum: Observe the patient while walking normally, on the heels and toes, and along a
straight line

HEADACHE
• Headache: is among the most common reasons patients seek medical attention.
• Primary headache: are those in which headache and its associated features occur in
the absence of any exogenous cause. The most common types are: migraine,
tension -type headache and cluster headache.
• It is often results in considerable disability and decrease in the patient quality of life.
• Secondary headache: are those caused by exogenous disorders
PHYSIOLOGY OF HEADACHE
Pain usually occurs in two pathways and Headache may originate from either orboth
mechanisms.
1. When peripheral nociceptors arestimulated in response to tissue injury, visceral
distension, or other factor. In such situations, painperception is a normal physiologic
response mediatedby a healthy nervous system. Relatively few cranial structures are
pain-producing (scalp, middle meningeal artery, Dural sinuses, falx cerebri, and
proximal segments of the large pial arteries).The key structures involved in primary
headache:
a) The large intracranial vessels and dura mater and theperipheral terminals of
the trigeminal nerve thatinnervate these structures
b) The trigeminocervical complex
c) Rostral pain-processing regions, such as the ventroposteromedial thalamus
and the cortex
d) The pain-modulatory systems in the brain
 2. Pain can also result whenpain-producing pathways of the peripheral or centralnervous
system (CNS) are damaged or activated inappropriately.
COMMON CAUSES OF HEADACHE

PRIMARY HEADACHE SECONDARY HEADACHE

 Tension-type headache systemic infection
 Migraine headache head injury
 Cluster headache vascular disorders
Subarachnoid hemorrhage
Brain tumor

MIGRAINE HEADACHE
• Migraine is a periodic disorder characterized by unilateral episodic headache
associated with sensitivity to light, sound, and movements, vomiting and visual
disturbance.
• It may develop at any age but more common in teens and twenties, women are more
often
• Headache can be triggered by:
Bright light, sound,hunger, excess stress, physical exertion, stormy weather,
hormonal fluctuations during menses, lack of or excess sleep, and alcohol.
Migraine aura
Consisting of visual disturbance with flashing lights or zigzag lines moving across the
visual field or other neurologic symptoms.

DIAGNOSTIC CRITERIA OF MIGRAINE HEADACHE

At least 2 of the following features plus at least 1 of the following features
 Unilateral pain Nausea/ vomiting
 throbbing pain photophobia
 aggravating by movement phono phobia
Andmoderate to severe intensity
MANAGEMENT
 Non pharmacologic:avoidance of triggers, regulated life style-healthful diet, regular
exercise, regular sleep, avoiding excess stress and alcohol.
 Pharmacologic: Combination of NSIDS and antiemetic‘s: aspirin, metoclopramide,
sumatriptan5-HT1 agonist: ergotamine ad dihydroergotamine
TENSION -TYPE HEADACHE
 The term- tension-type headache (TTH) is commonly used to describe a chronic head-
pain syndrome characterized by bilateral tight, band-like discomfort.
 The pain typically builds slowly, fluctuates in severity, and may persist more or less
continuously for many days.
 Clinical features, such as nausea, vomiting, photophobia, phono phobia, osmophobia,
throbbing, and aggravation with movement. Such an approach neatly separates
migraine, which has one or more of these features and is the main differential diagnosis
MANAGEMENT: The pain of TTH can generally be managed with simpleanalgesics such
as acetaminophen, aspirin, or NSAIDs.For chronic TTH, amitriptyline is the only proven
treatment
CLUSTER HEADACHE
 Cluster headache is a rare form of primary headache with a population frequency of
approximately 0.1%.
 The typical cluster headache patient has daily bouts of one to two attacks of
relatively short-duration unilateral pain for 8 to 10 weeks ; this is usually followed by
a pain-free interval that averages a little less than 1 year
 Onset is nocturnal in about 50% of patients, and menareaffected three times more
often than women.
 Cluster headache is associated with ipsilateral symptoms of cranial parasympathetic
autonomic activation: conjunctival injection or lacrimation, rhinorrhoea or nasal
congestion, or cranial sympathetic dysfunction such as ptosis.
MANAGEMENT
In acute Attack: oxygen inhalation, Sumatriptanand prednisone for preventive
treatment.

MENINGITIS
Definition: Is clinical syndrome characterized by inflammation of the meninges.
Etiology: viruses (commonest e.g. enteroviruses), bacteria e.g. pneumococci, fungi, T.B.
Clinical Picture: fever, headache, neck stiffness, photophobia, nausea and
vomiting(projectile), positive Kerning‘s sign, positive Brudzink‘s sign.
Investigation: Lumber Puncture ‗CSF examination‘ (definitive)
 Appearance:
a) Turbid ‗cloudy‘ indicates bacterial cause
b) Clear indicates virus, fungi or T.B.
 Cytology:
a) High lymphocytes, increased protein content with normal sugar
level indicates viral or T.B.
b) High neutrophils ‗polymorph nuclear cells‘, increased protein
content with reduced sugar level indicates bacterial causes.
 Culture
Other investigations: CT scan or MRI to exclude other lesions.
Site of Lumber Puncture: Adults (L3 and L4), infants (L2 and L3)
N.B: According to cytological testes, meningitis is divided into two categories:
 Lymphocytary: viral meningitis or T.B meningitis
 Non-lymphocytary: non-T.B bacterial meningitis

COMPLICATIONS OF MENINGITIS:

 Brain abscess
 Hydrocephalus
 Encephalitis
 Paralysis
 Seizures
 Death

MANAGEMENT:
a) Isolation: to get complete rest and accurate follow-up
b) Antiviral therapy e.g. acyclovir if it is viral origin
c) Antibiotics e.g. penicillin G and ceftriaxone in bacterial causes
d) Anti-T.B e.g. isoniazid, rifampicin, pyrazinamide, and ethambutol in mycobacterial
causes with 9 months duration
e) High dose of dexamethasone in all cases to decrease inflammation. It is the best
choice of all corticosteroids due to its capability to cross the BBB.

ENCEPHALITIS
Definition: inflammation of the brain parenchyma.
Aetiology: viruses e.g. herpes simplex virus type 1 and 2 (commonest cause), bacteria,
parasite.
CLINICAL PICTURE:The onset is usually acute with influenza like symptoms including
 fever,
 headache,
 drowsiness,
 mood change over hours or days with neurological deficit:
 cerebral manifestations (hemiparesis, involuntary movements, convulsions and
coma)
 cerebellar manifestations (ataxia),
 brain stem manifestations (cranial nerve lesions, pyramidal or sensory tract lesions)

INVESTIGATIONS:
a) CT and MRI show diffuse areas of edema e.g. in temporal lobe in viral cases
b) CSF showing raised cell count
c) viral serology
MANAGEMENT:
a) proper nursing
b) acyclovir therapy for viral cases
c) antibiotics for bacterial infections
d) Steroid therapy specially dexamethasone.

BRAIN ABSCESS
Definition: is a collection of pus that develops in response to an infection or trauma.
CLINICAL PICTURE:
 fever,
 headache,
 Focal manifestations e.g. hemiparesis, aphasia, epilepsy, high ICP.

INVESTIGATIONS:
 CT or MRI

MANAGEMENT:
(a) high dose of antibiotics
(b) drainage of the abscess

HYDROCEPHALUS
 Definition: a disturbance of cerebrospinal fluid (CSF) formation, flow, or absorption,
leading to an increase in volume occupied by this fluid in the central nervous system
(CNS).
 Normal pressure hydrocephalus (NPH): Enlarged ventricles and normal CSF
pressure at lumbar puncture (LP) in the absence of papilledema led to the term
NPH. The classic Hakim triad of symptoms includes gait apraxia, incontinence,
and dementia.
 Communicating hydrocephalus: occurs when full communication occurs
between the ventricles and subarachnoid space. It is caused by defective
absorption of CSF (most often, includes conditions such as intracranial
hemorrhage or meningitis resulting in damage to the arachnoid granulations,
where CSF is reabsorbed).
 Non-communicating hydrocephalus: occurs when CSF flow is obstructed within
the ventricular system. Caused by intraventricular or extra ventricular mass-
occupying lesions that disrupt the ventricular anatomy.
CAUSES:
 Congenital malformations:
1. Aqueduct stenosis
2. Chiari malformations
3. Dandy-walker syndrome
4. Benign intracranial cysts
5. Congenital CNS infections
 Acquired causes:
 1. Mass lesions (especially those in the posterior fossa): tumors, ventricle
abscess, Haematoma
2. Absorption blockages due to Inflammation (e.g. meningitis, sarcoidosis,
intracranial haemorrhage)

PATHOPHYSIOLOGY:
 Normal route of CSF from production to clearance is the following: From the
choroid plexus, the CSF flows to the lateral ventricle, then to the
interventricular foramen of Monro, the third ventricle, the cerebral aqueduct of
Sylvius, the fourth ventricle, the two lateral foramina of Luschka and one
medial foramen of Magendie, the subarachnoid space, the arachnoid
granulations, the dural sinus, and finally into the venous drainage.
 ICP rises if production of CSF exceeds absorption. This occurs if CSF is
overproduced, resistance to CSF flow is increased, CSF resorption is
decreased, or venous sinus pressure is increased.
SIGNS AND SYMPTOMS:
 Clinical features of hydrocephalus are influenced by the Patient's age, cause,
location of obstruction, duration, rapidity of onset.
 Symptoms in infants include the following: Poor feeding, Irritability,
Reduced activity and Vomiting
 Symptoms in children include the following:
1. Slowing of mental capacity
2. Headaches initially in the morning
3. Neck pain suggesting tonsillar herniation
4. Vomiting, more significant in the morning
5. Blurred vision: This is a consequence of papilledema and later of optic
atrophy
6. Double vision: This is related to unilateral or bilateral sixth nerve palsy
7. Drowsiness
 Symptoms in adults include the following:
1. Cognitive deterioration
2. Neck pain: If present, neck pain may indicate protrusion of cerebellar
tonsils into the foramen magnum.
3. Difficulty in walking

 DIAGNOSIS:is through imaging studies – MRI/CT.

 MANAGEMENT: Surgical treatment is the preferred therapeutic option.
 Diversion of the CSF by means of a shunt:
1. A ventriculoperitoneal (VP) shunt is used most commonly, it is placed
between the ventricular system and the peritoneal cavity
2. A ventriculoatrial (VA) shunt also is called a "vascular shunt." It is
placed between the ventricular system and the right atrium.
 CRANIAL NERVE INJURY
1. TRIGEMINAL NEURALGIA “TIC DOULOREUX‖
It is fifth cranial nerve supplies sensation to the skin of the face and anterior half of the head.
Its motor innervates the masseter and pterygoid masticatory muscles.
CAUSES
 Trauma
 Viral infection
 Compression
 Demyelinating disorders like multiple sclerosis

CLINICAL MANIFESTATIONS
Trigeminal neuralgia is characterized by pain in the lips, gums, check or chin ―facial pain‖
which lasts few seconds or minute.
DIFFERENTIAL DIAGNOSIS
 Tooth ache
 sinusitis
 mastoiditis
 head ache

MANAGEMENT
 Anti-inflammatory
 Corticosteroids
 Vitamin b complex

FACIAL NERVE PARALYSIS

Anatomically facial nerve supplies all the muscles concerned facial expression. The sensory
component is small it conveys taste sensation of anterior ⅔ of the tongue and probably
anterior wall of external auditory canal. The nerves typically travels from the pons through
the facial canal in the temporal bone and exits the skull at the stylomastoid foramen. Then
passes through the parotid gland and subdivides to supply the facial muscle.

CLINICAL FEATURES
Complete interruption of facial nerve at stylomastoid foramen results paralysis all muscles of
facial expression.
 The eyelid will not closed, upon attempted closure of lids, the eye on paralyzed
side rolls up ward ―bells phenomenon‖ that means one eye will be bigger than
other eye.
 The corner of mouth droops
 The lower lid sags and away from conjunctiva permitting tear to split over cheek
 Food collects between teeth and lip
 Saliva drips from corner of mouth
  Later on deviation of mouth occurs
 Patient complains numbness and heaviness of face

BELL’S PALSY: Most common form of facial paralysis

Etiology
 Infection like herpes simplex virus
 Immune cause
 Reactivation of varicella zoster virus

CLINICAL PRESENTATION˗
 Presence of incomplete paralysis in first weak is the most favorable prognostic sign,
 Pain under ear,
 Loss of taste sensation unilaterally and
 Hyperacusis―sensitivity to loud sound‖ may be present.

MANAGEMENT
 Corticosteroids
 Rehabilitation of muscle

GLOSSOPHARYNGEAL NEURALGIA
The pain is intense and paroxysmal which originates from one side of throat approximately
in the tonsillar fossa.in some cases pain localizes in ear or may radiate from throat to ear
because of involvement of tympanic branch of glossopharyngeal nerve. Spasms of pain may
be initiated by swallowing or coughing. Dysphagia and dysphonia, loss of gag reflex may be
present
SCIATIC NEUROPATHY
Sciatic neuropathies commonly complicate hip arthroplasty, pelvic procedures in which
patients are placed in a prolonged lithotomy position, trauma, hematomas, tumor infiltration,
and vasculitis, ankylosing spondylitis. In addition, many sciatic neuropathies are idiopathic.
CLINICAL FEATURES
 Weakness may involve all motions of the ankles and toes.
 Lower back pain.
 Pain in the rear or leg that is worse when sitting.
 Hip pain.
 Burning or tingling down the leg.
 Weakness, numbness, or difficulty moving the leg or foot.
 A constant pain on one side of the rear.
 A shooting pain that makes it difficult to stand up.
MYASTHENIA GRAVIS
 DEFINITION: Myasthenia gravis (MG) is a relatively rare acquired, autoimmune
disorder caused by an antibody-mediated blockade of neuromuscular transmission
resulting in skeletal muscle weakness.
PATHOPHYSIOLOGY:
 Myasthenia gravis is an autoimmune disease, most commonly caused by
autoantibodies to acetylcholine receptors in the post-junctional membrane of
the neuromascular junction, which are found in around 80% of affected
patients.
 The resultant blockage of neuromascular transmission and complement-
mediated inflammatory response reduces the number of acetylcholine
receptors and damages the end plate.
CLINICAL FEATURES:
1. Fatigable muscle weakness; movement is initially strong but rapidly weakens
as muscle use continues.
2. Worsening of symptoms towards the end of the day or following exercise is
characteristic.
3. Intermittent ptosis or diplopia, but weakness of chewing, swallowing, speaking,
or limb movement (most commonly those of the shoulder girdle) also occurs.
4. Respiratory muscles may be involved, myasthenia crisis, and respiratory
failure may cause sudden death.

INVESTIGATIONS:
 Edrophonium test: Injection of the chemical edrophonium chloride that
results in a sudden, temporary improvement in muscle strength might indicate
that you have myasthenia gravis. Edrophonium chloride blocks
acetylcholinesterase, the enzyme that breaks down acetylcholine.
 Repetitive nerve stimulation: by electrodes.
MANAGEMENT:
 Cholinesterase inhibitors: pyridostigmine and neostigmine enhance
communication between nerves and muscles.
 Corticosteroids: Corticosteroids such as prednisone inhibit the immune
system, limiting antibody production.
 Immunosuppressants: Such as azathioprine, mycophenolate, or
cyclosporine

SEIZURES AND EPILEPSY:

A seizure (from the Latin sacire, "to take possession of") is a paroxysmal event due to
abnormal excessive or synchronous neuronal activity in the brain. 5–10% of the population
will have at least one seizure, with the highest incidence occurring in early childhood and
late adulthood.
The meaning of the term seizure needs to be carefully distinguished from that of epilepsy.
Epilepsy describes a condition in which a person has recurrent seizures due to a chronic,
underlying process. This definition implies that a person with a single seizure, or recurrent
seizures due to correctable or avoidable circumstances, does not necessarily have epilepsy.
Epilepsy refers to a clinical phenomenon rather than a single disease entity, since there are
many forms and causes of epilepsy. The incidence of epilepsy is 0.3–0.5% in different
populations
Throughout the world, and the prevalence of epilepsy has been estimated at 5–10 persons
per 1000.
CLASSIFICATION OF SEIZURES
Determining the type of seizure that has occurred is essential for focusing the diagnostic approach
particular etiologies, selecting the appropriate therapy, and providing potentially vital information
regarding prognosis.
1. Partial seizures: simple or complex can secondarily generalize.
A. simple (preserved LOC)
 motor: postural, phonatory, forceful turning of eyes and/or head, focal muscle
rigidity/ jerking ± Jacksonian march (spreading to adjacent muscle groups)
 sensory: unusual sensations affecting vision, hearing, smell, taste, or touch
 autonomic: epigastric discomfort, pallor, sweating, flushing, piloerection, papillary
dilatation
 psychiatric: symptoms rarely occur without impairment of consciousness and are
more commonly complex partial
B. complex (altered LOC)
 patient may appear to be awake but with impairment of awareness
 classic complex seizure is characterized by automatisms such as chewing,
swallowing, lip-smacking, scratching, fumbling, running, disrobing, and other
stereotypic movements
 other forms: dysphasic, dysmnesic (déjà vu), cognitive (disorientation of time
sense), affective (fear, anger), illusions, structured hallucinations (music,
scenes, taste, smells), epigastric fullness
2. generalized seizures (decreased LOC)
 absence (petit mal): usually only seen in children, unresponsive for 5-10s with arrest
of activity, staring, blinking or eye-rolling, no post-ictal confusion; 3 Hz spike and slow
wave activity on EEG
 clonic: repetitive rhythmic jerking movements
 tonic: muscle rigidity in flexion or extension
 tonic-clonic (grand mal, generalized tonic-clonic [GTC])
 prodrome of unease or irritability hours to days before the episode
 tonic ictal phase: muscle rigidity
 clonic ictal phase: repetitive violent jerking of face and limbs, tongue biting,
cyanosis, frothing, incontinence
 post-ictal phase: flaccid limbs, extensor plantar reflexes, headache, confusion,
aching muscles, sore tongue, amnesia, elevated serum CK lasting hours
 myoclonic: sporadic contractions localized to muscle groups of one or more
extremities
 atonic: loss of muscle tone leading to drop attack
 Temporal lobe epilepsy is suggested by an aura of fear, olfactory or gustatory
hallucinations, and visceral or déjà vu sensations
 Frontoparietal cortex seizures are suggested by contralateral focal sensory or
motor phenomena
INVESTIGATIONS

CBC, electrolytes, fasting blood glucose, Ca2+, Mg2+, ESR, Cr, liver enzymes, CK,
prolactin also consider toxicology screen, EtOH level, AED level (if applicable)
 CT/MRI (if new seizure without identified cause or known seizure history with new
neurologic signs/symptoms)
 LP (if fever or meningismus)
 EEG: EEG findings suggestive of epilepsy: abnormal spikes, polyspike discharges,
spike-wave complexes 20-59% of first EEG are positive in epilepsy; 59-92% of
epilepsy is picked up with repeated EEGs; normal interictal EEGs do not rule out
epilepsy.
 Evoked EEG is used sometimes to induce epilepsy.
TREATMENT
Avoid precipitating factors
indications for medical therapy (anticonvulsants): 2 or more unprovoked seizures,
known organic brain disease, EEG with epileptiform activity, first episode of status
epilepticus, abnormal neurologic examination or findings on neuroimaging
 psychosocial issues: stigma of seizures, education of patient and family, status of
driver‘s license, pregnancy issues
 Safety issues: driving, operating heavy machinery, bathing, swimming alone consider
surgical treatment if focal and refractory.
STATUS EPILEPTICUS
 Definition: unremitting seizure or successive seizures without return to a baseline state
of greater than 5 min. it‘s a Medical Emergency:
 Status epilepticus can cause irreversible brain damage without treatment
 Complications: anoxia, cerebral ischemia and cerebral edema, MI, arrhythmias, cardiac arrest,
rhabdomyolysis and renal failure, aspiration pneumonia/pneumonitis, death (20%).
 The most common causes of status epilepticus are failure to take AEDs and first
presentation of epilepsy Status epilepticus as a result of EtOH withdrawal is rare, despite
it being a very common cause of seizures.
 Treatment: benzodiazepines
ANTIEPILEPTIC DRUGS
 Generalized-onset and partial-onset seizures: lamotrigine, topiramate, valproic
acid
 Partial seizures (simple partial, complex partial, and secondarily generalized
seizures): carbamazepine, gabapentin, oxcarbazepine phenobarbital, phenytoin,
pregabalin (note: these drugs may exacerbate generalized seizures)
 Absence seizures: ethosuximide (Zarontin®)

CNS MALFORMATIONS
SYRINGOMYELIA
Syringomyelia is a developmental cavity of the cervical cord that is prone to enlarge and
produce progressive myelopathy. Symptoms begin insidiously in adolescence or early
adulthood, progress irregularly, and may undergo spontaneous arrest for several years.
Many young patients acquire a cervical-thoracic scoliosis. More than half of all cases are
associated with Chiari type 1 malformations in which the cerebellar tonsils protrude through
the foramen magnum and into the cervical spinal canal. The pathophysiology of syrinx
expansion is controversial, but some interference with the normal flow of CSF seems likely,
perhaps by the Chiari malformation.
CLINICAL PRESENTATIONS
 Central cord syndrome consisting of dissociated sensory loss (loss of pain and
temperature sensation with sparing of touch and vibration) and
 Areflexic weakness in the upper limbs.
 The sensory deficit has a distribution that is ―suspended‖ over the nape of the neck,
shoulders, and upper arms (carpel tunnel)
 Muscle wasting in the lower neck, shoulders, arms, and hands with asymmetric or
absent
 As the cavity enlarges and further compresses the long tracts, spasticity and
weakness of the legs, bladder and bowel dysfunction, and a Horner‘s syndrome
appear. Some patients develop facial numbness and sensory loss from damage to
the descending tract of the trigeminal nerve (C2 level or above).

INVESTIGATIONS
MRI scans accurately identify developmental and acquired syrinx cavities and their
associated spinal cord enlargement
TREATMENT
The Chiari tonsillar herniation is usually decompressed, generally by
suboccipitalcraniectomy, upper cervical laminectomy, and placement of a dural With Chiari
malformations, shunting of hydrocephalus should generally precede any attempt to correct
the syrinx.
CHIARI MALFORMATION
Condition in which brain tissue extends into the spinal canal. Chiari malformation is
uncommon, Doctors categorize Chiari malformation into three types.
CLINICAL PRESENTATIONS
 Many people with Chiari malformation have no signs or symptoms and don't need
treatment.
 Headaches, often severe, are the classic symptom of Chiari malformation. They
generally occur after sudden coughing, sneezing or straining.
 Neck pain, unsteady gait (problems with balance),
 Poor hand coordination (fine motor skills), Numbness and tingling of the hands and
feet, Dizziness
 Difficulty swallowing,
 Less often, people with Chiari malformation may experience, slow heart rhythm,
Curvature of the spine (scoliosis) related to spinal cord impairment.
 Abnormal breathing, such as central sleep apnea, which is when a person stops
breathing during sleep.
 The signs and symptoms can include those related to a form of spina bifida called
myelomeningocele
 Chiari malformation type III, a portion of the lower back part of the brain (cerebellum)
or the brainstem extends through an abnormal opening in the back of the skull.
COMPLICATIONS
 Spina bifida
 Myelomeningocele.
 Syringomyelia
 Tethered cord syndrome
DIAGNOSIS
Magnetic resonance imaging (MRI). An MRI is often used to diagnose Chiari malformation.
Computerized tomography (CT) scan.
TREATMENTS.
 Reducing pressure usually treat symptomatic Chiari malformation
 Surgery for Chiari malformation, called posterior fossa decompression.
 If a syrinx or hydrocephalus, you may need a tube (shunt) to drain the excess fluid.

SUBARACHNOID HEMORRHAGE
Subarachnoid hemorrhage (SAH) renders the brain critically ill from both primary and
secondary brain insults. Excluding head trauma, the most common cause of SAH is rupture
of a saccular aneurysm.
SACCULAR ("BERRY") ANEURYSM
PATHOPHYSIOLOGY
Saccular aneurysms occur at the bifurcations of the large- to medium-sized intracranial
arteries; rupture is into the subarachnoid space in the basal cisterns and often into the
parenchyma of the adjacent brain. Approximately 85% of aneurysms occur in the anterior
circulation, mostly on the circle of Willis. Aneurysm size and site are important in predicting
risk of rupture. Those >7 mm in diameter and those at the top of the basilar artery and at the
origin of the posterior communicating artery are at greater risk of rupture.
Mycotic aneurysms are usually located distal to the first bifurcation of major arteries of the
circle of Willis
CLINICAL MANIFESTATIONS
 Most unruptured intracranial aneurysms are completely asymptomatic. Symptoms are
usually due to rupture and resultant SAH,
 Mass effect on cranial nerves or brain parenchyma.
 ICP suddenly rises. This may account for the sudden transient loss of consciousness
that occurs in nearly half of patients.
 Sudden loss of consciousness
 excruciating headache,
  A third cranial nerve palsy, particularly when associated with pupillary dilation, loss of
ipsilateral (but retained contralateral) light reflex, and focal pain above or behind the
eye
 The initial clinical manifestations of SAH can be graded using the Hunt-Hess or World
Federation of Neurosurgical Societies classification schemes (Table 275-3

TREATMENT: SUBARACHNOID HEMORRHAGE

 Surgical repair involves placing a metal clip across the aneurysm neck, thereby
immediately eliminating the risk of rebleeding.
 The medical management of SAH focuses on protecting the airway, managing blood
pressure before and after aneurysm treatment, preventing rebleeding prior to
treatment, managing vasospasm, treating hydrocephalus, treating hyponatremia, and
preventing pulmonary embolus.
 Anticonvulsants are sometimes given as prophylactic therapy since a seizure could
theoretically promote rebleeding.
 Glucocorticoids may help reduce the head and neck ache caused by the irritative
effect of the subarachnoid blood.

GUILLAIN-BARRÉ SYNDROME
DEFINATION
Guillain-Barre syndrome (GBS) - is an acute, frequently severe, and fulminant
polyradiculoneuropathy that is autoimmune in nature.
Males are at slightly higher risk for GBS than females, and in Western countries adults are
more frequently affected than children.
CLINICAL MANIFESTATIONS
GBS presents
 Rapidly evolving areflexic motor paralysis with or without sensory disturbance.
 Ascending paralysis.
 facial diparesis
 Weakness and frequently accompanied by tingling dysesthesias in the extremities
(mostly legs than arms).
 Ower cranial nerves damage causing (difficulty handling secretions and maintaining
an airway).
 Pain in the neck, shoulder, back, or diffusely over the spine.
 bladder dysfunction
 Autonomic involvement(loss of vasomotor control with wide fluctuation in blood
pressure, postural hypotension,and cardiac dysrhythmias)
SUBTYPES OF GBS
1. Acute inflammatory demyelinating polyneuropathy (AIDP).
2. Acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy
(AMSAN).
3. Limited or regional GBS (Miller Fisher syndrome (MFS), which presents as rapidly
evolving ataxia and areflexia of limbs without weakness, and ophthalmoplegia and
pupillary paralysis.
NB-70% of cases of GBS occur 1–3 weeks after an acute infectious respiratory or
gastrointestinal.( Campylobacter jejuni, human herpes Virus, CMV or Epstein-Barr virus
Mycoplasma pneumonia)
PATHOPHYSIOLOGY
In the demyelinating forms of GBS, the basis for flaccid paralysis and sensory disturbance is
conduction block.
LABORATORY FEATURE
CSF protein level (1–10 g/L [100–1000 mg/dL]) without accompanying pleocytosis. The CSF
is often normal<48 h; by the end of the first week, the level of protein is usually elevated.
DIAGNOSIS
The diagnosis of AIDP is made by recognizing the pattern of rapidly evolving Paralysis with
areflexia, absence of fever or other systemic symptoms, and characteristic antecedent
events.
DIFFERENTIAL DIAGNOSIS include
acute myelopathies,
diphtheria (early oropharyngeal disturbances); porphyria,
Lyme polyradiculitis and other tick-borne paralyses; myasthenia
gravis
 botulism poisonings with organophosphates,
 Paralytic shellfish poisoning; or severe hypophosphatemia (rare).
TREATMENT
Treatment should be initiated as soon after diagnosis as possible, intravenous immune
globulin (IVIg) or plasmapheresis.
In the worsening phase of GBS, most patients require monitoring in a critical care setting,
with particular attention to vital capacity, heart rhythm, blood pressure, nutrition, deep vein
thrombosis prophylaxis, cardiovascular status, early consideration (after 2 weeks of
intubation) of tracheotomy, and chest physiotherapy. NB Approximately 85% of patients with
GBS achieve a full functional recovery within several months to a year,
 Multiple sclerosis
Demyelinating disorders are immune-mediated conditions characterized by preferential
destruction of central nervous system (CNS) myelin.
The peripheral nervoussystem (PNS) is spared, and most patients have no evidence of an
associated systemic illness.
Multiple sclerosis(MS),the most common disease in this category, is secondary to trauma as
a cause of neurologic disability beginning in early to middle adulthood.
PHYSIOLOGY
Nerve conduction in myelinated axons occurs in a salutatory manner, with the nerve impulse
jumping from one node of Ranvier to the next without depolarizationof the axonal membrane
underlying the myelinsheathbetween nodes.
CAUSES
 Genetic predisposition
 Autoimmune
CLINICAL MANIFESTATIONS
 The onset of MS may be abrupt or insidious. Symptoms may be severe or seem so
trivial that a patient may not seek medical attention for months or years.
 Weakness of the limbs may manifest as loss of strength,speed, or dexterity, as
fatigue, or a disturbance of gait.
 Exercise-induced weakness is a characteristic symptom of MS
 Signs such as spasticity, hyperreflexia, and Babinski‘s signs.
 Optic neuritis (ON) presents as diminished visual acuity,dimness, or decreased color
perception (desaturation)in the central field of vision.
 Bladder dysfunction is present in >90% of MSpatients.
 Constipation
 Cognitive dysfunction
 Depression, Fatigue, Facial weakness
DISEASE COURSE
Four clinical types of MS have been described (Fig. 39-2):
1. Relapsing/remitting MS (RRMS) accounts for 85%of MS cases at onset and is
characterized by discrete attacks that generally evolve over days to weeks (rarely over
hours). There is often complete recovery over the ensuing weeks to months.
2: Secondary progressive MS (SPMS) always begins asRRMS. At some point, however,
theclinical course changes so that the patient experiencesa steady deterioration in function
unassociated withacute attacks (which may continue or cease duringthe progressive phase).
SPMS produces a greateramount of fixed neurologic disability than RRMS.
3. Primary progressive MS (PPMS) accounts for ∼15% ofcases. These patients do not
experience attacks but only a steady functional decline from disease onset. Compared to
RRMS disability develops faster (atleast relative to the onset of the first clinical symptom).
4. Progressive/relapsing MS (PRMS) overlaps PPMS and SPMS and accounts for ∼5% of
MS patients. Likepatients with PPMS, these patients experience a steady deterioration in
their condition from disease onset. However, like SPMS patients, they experience
occasional attacks superimposed upon theirprogressivecourse (Fig.39-2D).

DIAGNOSIS
There is no definitive diagnostic test for MS.
 Diagnostic criteria for clinically definite MS require documentation of two or more
episodes of symptoms and two or more signs that reflect pathology in anatomically
non contiguous white matter tracts of the CNS (The second may be documented by
abnormal preclinical tests such as MRI or evoked potentials (EPs).
 Cerebrospinal fluid
CSF abnormalities found in MS include a mononuclearcell pleocytosis and an
increased level of intrathecally synthesized IgG. The total CSF protein is usually
normal or slightly elevated. .
DIFFERENTIAL DIAGNOSIS
 Acute disseminated encephalomyelitis (ADEM),
 Antiphospholipid antibody syndrome.
 Behçet‘s disease
 Cerebral autosomal dominant arteriopathy,
 subcortical, infarcts, and leukoencephalopathy (CADASIL)
 Congenital leukodystrophies
 Human immunodeficiency virus (HIV) infection.
 Ischemic optic neuropathy (arteritic and nonarteritic),
 lyme disease,
 Sjögren‘s syndrome,
 Stroke and ischemic cerebrovascular disease,
 Syphilis, Systemic lupus erythematosus
 vascular malformations
TREATMENT
 Glucocorticoid treatment is usually administered asintravenous methylprednisolone,
500–1000 mg/d f3–5 days, either without a taper or followed by a courseof oral
prednisone beginning at a dose of 60–80 mg/dand gradually tapered over 2 weeks.
Orally administmethylprednisolone or dexamethasone .
 Interferons