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NEUROLOCALIZATION

Moderator: Dr. Workie (Pediatrician)

Presenter : Dr. Kindsew Abi(R2)

Bahir Dar University Collage of Medicine &Health


Sciences School of Medicine Pediatrics & Child
Health Department
Presentation outlines

Objectives
Introductions
Over view of Neuroanatomy
Approach to patients with neurologic disease
Localization of a lesion in neurologic disease ( where,
what)
Investigation modalities in neurologic disease
References
INTRODUCTION

• Localization derives from the Latin term locus or site


• Localization is the diagnostic exercise of determining
from the signs (most often) or symptoms of the patient
what site of the nervous system has been affected by a
disease process.
• Localization- topographic diagnosis
Cont…

Clinical diagnosis in neurology requires:


• Recognition of impaired function
• depends on a good history and neurologic
examination
• Identification of what site of the nervous system has
been affected, that is, localization
• Definition of the most likely etiology, often resulting in a
differential diagnostic list
• Use of ancillary procedures to determine which of the
different possible etiologies is present in the given
patient
Cont…

• Hypothesize the most likely anatomical site(s) of the


neurological problem,
• whether the lesion involves the central nervous system
(CNS), peripheral nervous system, both, or neither:
Where is the lesion?
• List the most likely cause/causes of the neurological
problem, whether vascular, neoplastic, infectious, and
so on: What is the lesion?
• Establish the onset of illness and the temporal profile
as acute, subacute, chronic, progressive, or
paroxysmal
Approach to patients with neurologic disease

The history provides important etiologic clues, but the


neurological examination and neuroimaging studies
confirm the anatomical location
Acute
•Vascular/infarct – focal
•Hypoxic – diffuse
•Trauma – focal or diffuse
Subacute
•Inflammatory/infectious – focal or diffuse
•Immune – focal or multifocal
•Toxic/metabolic – diffuse
Cont….

Chronic
• Congenital – focal or diffuse
• Degenerative – diffuse or system related
• Neoplastic – focal
Paroxysmal
• Seizure – focal or diffuse
• Vascular/syncope – diffuse
• Pain/headache – focal or diffuse
Cont…

Previous medical history


• Perinatal and postnatal hx
• Developmental milestones
• Immunizations
• Trauma
• Present or previously used medications
• Any bleeding disorder
• Previous hx of any neurological problem
• Previous infection especially involving CNS
Cont…

Family hx
Socioeconomic status
Hx of contact with TB, measles
Neurological examination

Higher cortical functions


Systemic review
Cranial nerve examination
Sensory system
Pyramidal system
Extrapyramidal system
Cerebellum
Evaluation of speech and language
Neuroanatomy

• Neuroanatomy provides the road map for localization


• Localization in neurology requires knowledge of
neuroanatomy, but for the most part, detailed
neuroanatomy is not necessary
• The nervous system consists of the central nervous
system (CNS) and the peripheral nervous system
(PNS)
• The CNS is made up of the brain and the spinal cord
Cont…

• The brain is made up of :


• The cerebrum(telencephalon): is composed of two hemispheres that are
covered by a layer of gray matter, the cerebral cortex.
• Diencephalon: made up of the thalamus, metathalamus, epithalamus,
subthalamus, and hypothalamus
• brainstem and cerebellum
• Has four lobes and four ventricles
Cerebral cortex
• outermost part of cerebrum
• “grey matter”
• a critical role in memory, attention, perceptual
awareness, thought, language, and consciousness
Cont…

• classified on the basis of gross topographical


conventions into four lobes and four ventricles
Cont…

Frontal lobe
• Primary motor cortex
• Frontal eye field
• Supplementary motor cortex
• Prefrontal cortex predominantly Contains Areas 44
and 45 ,they constitute the motor speech area
(Broca's area).
Parietal lobe
• Somatosensory cortex
• Superior and inferior parietal lobules
Cont…

Temporal lobe
• primary auditory area(area 41,42)
• Auditory association A(area 22)
OCCIPITAL LOBE;
• Primary visual area (area 17),
• visual association area(area 18,19)
Subcortical Structures

• include the basal ganglia, thalamus, subthalamic


nucleus, hypothalamus, red nucleus, substantia nigra,
cerebellum, and brain stem, and their nerve pathways.
• anatomically and functionally interconnected with the
cerebral cortex
Thalamus
• The major primary sensory relay center from all
sensory modalities except olifactory sense
• Has diffuse and bidirectional connection with the
cortex
• Has function in consciousness as its connection with
RAS
Cont…

Internal capsule
• Pathway for motor and sensory systems
• Fibers coming to and proceeding from the cortex
make up the fan-shaped corona radiate which
converge into a broad band
Basal ganglia
• It consists of caudate nucleus, lenti form nucleus,
subthalamus and substanitia nigra
• Motor plans of movement stored in basal ganglia and
cerebellem
Brain stem

 Brain stem consists of:


1.The midbrain
• CN III and IV
• Cerebral peduncles
• RAS
• Integration of vertical eye movement, sensory and
motor function
Cont….
2.The pons
• Most of the cranial nerves from CN V to CN VIII RAS
• Vital function (e.g.., breathing, consciousness, cardiac
function), motor and sensory functions, lateral eye
movement
3.Medulla oblongata
• CN IX to CN XII
• Major autonomic center
• Swallowing, cardiac function, balance, lingual
movements, motor and sensory function
Cerebellum

• Major center for coordination and control of fine


movement and gait
• Has a vermis and two lateral hemispheres
• Several connection with the brain stem
• is the portion of the brain through which cerebral
motor cortex achieves synthesis and coordination of
individual muscle contractions required for normal
voluntary movements
Spinal cord

• Extends from cervicomedulary junction to conus


medularis
• Divided by a ventral median fissure and dorsal
median sulcus into two hemicords
• Each hemicord has a ventral, lateral and dorsal
column
• At the center is the central canal in which CSF
baths the spinal cord
• Has sensory and motor function of the arms and
legs
Cont…

Covering of the spinal cord


• Connective tissue membranes
• Dura mater: outermost layer: continuous with epineurium of the spinal
nerves
• Arachnoid mater: thin and wispy
• Pia mater: bound tightly to surface
• Based on the spinal vertebral that enclose the cord it divided into 4
parts ( cervical=8; thoracic=12;lumbar=5;sacral=5;cocc=1 ) caudally
Cont…

White matter
• Bundle of axons in the CNS
• Consists of ascending tracts, descending tracts,
association fibers and commissural fibers
Ascending tracts has dorsal lemniscus system for fine
touch and proprioception and anteriolateral (spinal)
lemniscus for touch, pain and temperature
Dorsal lemniscus system crosses at the level of lower
medulla therefore, they lateralize neurologic signs and
symptoms
Cont…

Descending tracts (corticospinal tracts) which controls


voluntary, discrete, highly skilled movements of the
distal portion of the limbs, arises from the primary MC,
lateral premotor cortex (PMC), and supplementary
motor area (SMA)
UMN: Cell body in motor cortex descends ipsilaterally
through internal capsule until decussate at pyramid and
descends contra laterally to Cell body of anterior horn
of the spinal cord
Blood supply of CNS

• There are three major arteries supplying the CNS


 Anterior Cerebral Artery arises from internal carotid
artery and supplies the medial surface of the cerebrum
and the upper border of the frontal and parietal lobes
Middle cerebral artery (MCA), the largest branch of the
ICA, and supplies most of the lateral surface of the
cerebral hemisphere and the deep structures of the
frontal and parietal lobes
Posterior Cerebral Artery arises from the basilar artery
and supplies the occipital lobes and the inferomedial
portions of the temporal lobes
Neurological localization of a lesion

First step in neurological localization is to determine if it


is a central(UMN) or peripheral(LMN) lesion.
Central(UMN) lesion should be localized to
• Cortical brain
• Subcortical brain structures (corona radiata, internal
capsule, basal ganglia or thalamus)
• Brain stem
• Cerebellum
• Spinal cord
Cont…

Peripheral(LMN) lesions should be localized to


• Anterior horn cells
• Nerve roots
• Plexus
• Peripheral nerve
• Neuromuscular junction
• Muscle
Cont…

Comparison of UMN and LMN lesion


Cortical lesions

• Characteristic of unilateral hemispheric


pathology is a “hemi” deficit:
• hemi sensory loss, hemiparesis,
• hemianopsia, hemi seizures
• hyperreflexia and pathologic reflexes
• may have aphasia, apraxia, astereognosis, impaired two-point
discrimination, memory loss, cognitive defects,
• Altered mental status : if bilateral lesion occurs
• Weakness of face/arm>leg (or vice versa) if motor homunculus is
damaged
• Pain is not a feature unless the thalamus is involved, and there is no
difficulty with sphincter control unless both hemispheres are involved
Cont…

Neurologic examination findings when cortical area of


the Brian is affected:
• Higher Cortical Function: aphasia, apraxia, agnosia
• Cranial Nerves: normal
• Cerebellar Function: normal
• Motor: weakness if the motor homunculus is affected
• Sensory: sensory abnormalities if the sensory
homunculus is affected
• Deep Tendon Reflexes: hemi-hyper-reflexia
• Pathologic Reflexes: possibly Babinski’s reflex if
corticospinal tracts are damaged
Subcortical lesions

• Hemi distribution of dysfunction but lacks those


elements that are typically cortical (e.g., language
disturbance, apraxia, seizures, dementia)
• Dense hemiplegia motor weakness and sensory
abnormality (leg=arm=face)
• A lesion in the dominant hemisphere that does not
affect language function is more likely to be sub
cortical
• Parkinsonism, tremor, chorea if basal ganglia is
affected
Cont…

Neurologic examination findings when subcortical area


of the Brian is damaged:
• Higher Cortical Function: normal
• Cranial Nerves: visual field cuts
• Cerebellar Function: usually normal
• Motor: weakness in face=arm=leg, UMN
• Sensory: sensory abnormalities in face=arm=leg
• Deep Tendon Reflexes: hemi-hyper-reflexia
• Pathologic Reflexes: Babinski’s reflex and possibly
frontal release signs
Cerebellum

• The cardinal features of cerebellar dysfunction involve


disturbances in
• Motor control,
• Muscle tone regulation and
• Coordination of skilled movements
• Ataxia consists of varying degrees of dyssynergia,
dysmetria, lack of agonist-antagonist coordination,
and tremor
• Inability to make rapid repetitive or rapid alternating
movements (RAMs)
• Hypotonia, asthenia or slowness of movement, and
deviation or drift of the outstretched limbs
Cont.…

• Dysarthria characterized by abnormalities in articulation


and prosody
• Described as scanning, slurred, hesitant, slow altered
accent, and garbled speech
• Nystagmus, which is a rhythmical oscillation of the eyes,
frequently observed in association with cerebellar
disorders
• Common diseases that affecting cerebellar function
• Acute alcohol poisoning
• Congenital agenesis or hypoplasia
• Trauma, infections, tumors, vascular disorders and
poisoning with heavy metals
Brainstem

• Is a compact structure, with cranial nerve nuclei, nerve


fascicles, and long ascending and descending tracts all
closely juxtaposed.
• Structures and centers in the reticular formation control
many vital functions.
• Brainstem diseases are serious, and often life
threatening.
• Brainstem syndromes typically involve dysfunction of
one or more cranial nerves
Cont…

• Involvement of the cranial nerves and the presence


of crossed-findings:
• Ipsilateral cranial nerve lesion
• Contralateral hemiparesis
• Gaze palsy
• Nystagmus
• Dysarthria
• Alteration of mental status & coma at the onset of
an acute severe brainstem lesion
• Brian stem lesion cause symptoms of spinal cord
disease and long Tract signs: (bilateral and crossed)
Cont…

Mid brain
• It possesses two important cranial nerve nuclei
(oculomotor and trochlear) the red nucleus and
substantia nigra, which greatly influence motor function
• It serves as a conduit for many important ascending
and descending tracts
• Patients with mid brain lesion may present with
diplopia, weakness and abnormal movements (tremors
or ataxia) and Impaired upward gaze, CN III or IV palsy
and contralateral hemiparesis or ataxia on physical
exam

Cont…
Pons
•It possesses several important cranial nerve nuclei (CN V,
CN VI, CN VII, and CN VIII)
•Serves as a conduit for important ascending and
descending tracts
•Patients may present with:
• speech & swallowing difficulties,
•weakness, sensory changes
•diplopia and dysarthria,
•ipsilateral facial(CN VII) weakness
•CN VI palsy
•Horner's syndrome
•Contralateral hemiparesis or sensory loss
•Impaired horizontal gaze
•Nystagmus, ataxia
Cont.…
Medulla
• It possesses cranial nerve nuclei of CN IX,X,XII
Wallenberg’s or lateral medullary syndrome
• due to occlusion of the vertebral or posterior inferior
cerebellar artery. Is characterized by:
• Ipsilateral decreased pain and temperature of the
face(nucleus and spinal tract of the trigeminal nerve)
• Dysarthria, dysphagia due to paralysis of the ipsilateral
palatal and laryngeal muscles
• Ipsilateral Horner’s syndrome (descending sympathetic
fibers)
• Ipsilateral gait and limb ataxia (cerebellum or inferior
cerebellar peduncle)
• Hiccups, vertigo, nausea, vomiting, nystagmus
(vestibular nuclei)
Cont…

Medial Medullary Syndrome


• Thrombosis of the medullary branch of vertebral artery
produces the following signs and symptoms:-
• Contralateral hemiparesis
• Contralateral impaired sensations of position and
movement and tactile discrimination (medial
lemniscus)
• Ipsilateral paralysis of tongue muscles with deviation
to the paralyzed side when the tongue is protruded
(hypoglossal nerve)
Localization of the spinal cord lesions

• The presence of a horizontally defined level below


which sensory, motor, and autonomic function is
impaired is a hallmark of spinal cord disease
• Spinal cord diseases are frequently devastating
• They produce quadriplegia, paraplegia, and sensory
deficits far beyond the damage
• Many spinal cord diseases are reversible if recognized
and treated at an early stage
• are among the most critical of neurologic emergencies
Cont…
Causes
Compressive
•Neoplasm
•Epidural abscess, Epidural hemorrhage   
•Herniated disk
•Posttraumatic compression by fractured or displaced
vertebra or hemorrhage
Non compressive
•Infectious
• Viral: VZV, HSV-1 and -2, CMV, HIV, HTLV-I
•Bacterial and mycobacterial: Borrelia, Listeria, syphilis,
Mycoplasma pneumoniae
•Parasitic: schistosomiasis, toxoplasmosis(HIV +Ve)
Cont…

• Developmental
• Syringomyelia ,Meningomyelocele
• Metabolic
• Vitamin B12 deficiency
• Copper deficiency
• Inflammatory
•  Multiple sclerosis
• Transverse myelitis, Sarcoidosis, Systemic lupus
erythematosus
Cont…

Spinal cord syndromes


1. Complete cord transection
2. Brown-Sequard syndrome
3. Anterior spinal artery syndrome
4. Posterolateral column syndrome
5. Central lesion(Syringomyella)
6. Posterior column syndrome(Tabes dorsalis)
7. Anterior horn cell syndrome
Cont…

Complete cord transection


Cervical cord lesions
Upper cervical cord lesions produce quadriplegia and
weakness of the diaphragm(C1-C4)
Lesions at C4-C5 produce quadriplegia.
 C5-C6, there is loss of power and reflexes in the
biceps &brachioradialis
 C7 and C8 weakness finger and wrist extension and
flexion are impaired
 Horner's syndrome (miosis, ptosis, and facial
hypohidrosis) may accompany a cervical cord lesion
at any level
Cont…

Thoracic cord lesions


• Useful markers for localization are the nipples (T4) and
umbilicus (T10)
• Leg weakness and disturbances of bladder and bowel
function accompany the paralysis
• Lesions at T9-T10 paralyze the lower—but not the
upper—abdomen
Cont…

Lumbar
• lesions at the L2-L4 spinal cord levels paralyze flexion and
adduction of the thigh
• weaken leg extension at the knee, and abolish the patellar
reflex
• lesions at L5-S1 paralyze only movements of the foot and
ankle, flexion at the knee, and extension of the thigh, and
abolish the ankle jerks (S1)
Sacral
• prominent bladder and bowel dysfunction, urinary retention and
incontinence with and impotence.
• characterized by low back and radicular pain, asymmetric leg
weakness and sensory loss
Cont…
• The conus medullaris caudal termination of the spinal cord
• The distinctive conus syndrome consists:
• bilateral saddle anesthesia (S3-S5)
• prominent bladder and bowel dysfunction (urinary
retention and incontinence with lax anal tone)
• Lesions of the cauda equina characterized by:
• low back and radicular pain
• asymmetric leg weakness and sensory loss,
• variable areflexia in the lower extremities
• relative sparing of bowel and bladder function
• But mass lesions in the lower spinal canal often produce
a mixed clinical picture
Cont…

Brown-Sequard syndrome
•Ipsilateral weakness and loss of proprioception
•contralateral loss of pain and temperature sense one or two
levels below the lesion
•Segmental signs, such as radicular pain, muscle atrophy, or
loss of a deep tendon reflex, are unilateral

Central Cord Syndrome
•Mostly occur in cervical cord
•dissociated sensory loss: loss of pain and temperature with
preservation of fine touch sensation and proprioception
• Spinal trauma, Syringomyelia, hydromyelia, haematomyelia
and intrinsic cord tumors are the main causes
Cont…
Anterior spinal artery syndrome
• Vasculitis, aortic dissection, postoperative are the main
causes
• Back of neck pain of sudden onset
• Rapidly progressive flaccid and areflexic paraplegia
• Loss of pain and temperature to a sensory level
• Preservation of proprioception and vibration sensation
• Urinary incontinence
Cont…

Posterior spinal artery syndrome


• Loss of proprioception and vibratory sense
• Preserved pain and temperature sensation except for
involved segment of cord, where global anesthesia is
present
• Isolated Focal Motor or Sensory Deficits in
Extremities, Which May Represent Lacunar
Infarctions
• Slowly progressive paraparesis or quadriparesis
(Hypoxic myelopathy)
Cont…

Posterolateral column syndrome


• The posterior and lateral columns in the upper spinal
cord may be selectively damaged in subacute
combined degeneration of the spinal cord due to vit.
B12 deficiency.
• Posterior and lateral spinal cord involvement is also
seen in cases of vacuolar myelopathy associated
with (AIDS)
• Loss of position, vibration and pressure are affected
• Bilateral corticospinal tract dysfunction results in
spasticity, hyperreflexia, and bilateral Babinski's
signs
Cont…

Posterior column syndrome(Tabes dorsalis)


• The posterior columns are selectively damaged by
tabes dorsalis (tabetic neurosyphilis, progressive
locomotor ataxia).
• Inflammation and degeneration of the dorsal roots
causing destruction of the posterior columns of the
spinal cord
• Position, vibration and pressure are affected
• Romberg's sign +VE
Cont…

Anterior horn cell syndrome


• Diffuse weakness, atrophy, and fasciculation
• Muscle tone is usually reduced and muscle stretch
reflexes may be depressed or absent
• Sensory changes are absent because the sensory
tracts remain unaffected
• Certain disease processes selectively damage the
anterior horn cells, like spinal muscular atrophies,
polio
Localization of lesions in Peripheral Nervous
System

• Disorders affecting peripheral nerves cause various


symptoms and signs corresponding, in anatomic
distribution, to regions supplied by each nerve
• To make a correct topographic diagnosis of peripheral
nerve lesions, the clinician must thoroughly know the
area of the sensory supply of each nerve, the muscles
it innervates, and any muscle stretch reflex sub served
by the nerve
• In general, with peripheral nerve lesions, the area of
light touch sensory loss is greater than the area of
pinprick sensory loss
Cont…

• Peripheral neuropathy ( lesion ) is the general term


indicating Peripheral nerve disorders of any cause
• Also called as lower motor neuron lesion ( LMNL )
• Possible causes include:
-trauma
-infections ( eg. Poliomyelitis )
-vascular disorders
- degenerative diseases
-neoplasms
Cont…

• Peripheral (LMN ) lesions should be localized to :


- Anterior horn cells
- Nerve root(S)
- Nerve plexus
- Peripheral nerve
- Neuromuscular junction (NMJ )
- Muscle
Cont…

• Patterns
- Mononeuropathy
- Polyneuropathy
- Mononeuropathy multiplex (multifocal neuropathy )
- Radiculopathy
- Polyradiculoneuropathy
- Plexopathy
Cont…

Radiculopathy
• Most radiculopathies are due to disc herniation or
spondylosis When severe, there are both motor and
sensory deficits and depressed DTR in the distribution
of the involved root(s)
• Pain is common and often severe, usually
accompanied by limitation of motion of either the neck
or lower back
• no bowel or bladder dysfunction
Cont…

Peripheral Neuropathy
• Common causes include diabetes mellitus, alcoholism
and GBS
• Patients with generalized polyneuropathy have
symmetric, predominantly distal weakness
• sensory loss, depressed or absent DTRs,
• no bowel or bladder dysfunction
• Peripheral nerve disease can be mononeuropathy,
polyneuropathies and multiple mononeuropathy
(mononeuritis multiplex)
Cont…

 Guillain barre’ syndrome


• Is an acute , frequently severe, and fulminant
Polyradiculoneuropathy that is autoimmune in nature
• Clinical manifestations include :-
• rapidly evolving areflexic motor paralysis with or
without sensory disturbance
• the usual pattern is an ascending paralysis
• weakness typically evolves over hours to few days
• legs are usually more affected than arms
• Bladder dysfunction may occur in severe cases
• In severe cases autonomic involvement is common
Cont…

 Chronic Inflammatory Demyelinating


Polyradiculoneuropathy ( CIDP )
• Distinguished from GBS by its chronic course
• Onset is usually gradual, sometimes subacute in a
few
• Motor and sensory symptoms in most cases
• Weakness of limbs is usually symmetric
• Tremor in approximately 10% of cases
Localization of Neuromuscular Junction
Disorders
• The cardinal manifestation of diseases involving the
NMJ is weakness due to impaired neuromuscular
transmission (NMT)
• The most common conditions encountered clinically
are MG and the Lambert-Eaton myasthenic syndrome
(LEMS)
• Other rare disorders that can cause clinically significant
NMT disorders include botulism, hypermagnesemia and
exposure to some toxins
Cont…
 Myasthenia Gravis
• is a neuromuscular disorder characterized by weakness
and fatigability of skeletal muscles
oClinical Features
• Lids and extraocular muscle impairment
• Diplopia and ptosis
• Facial weakness produces a “ snarling’’ expression
when the patient attempts to smile
• Weakness in chewing and swallowing
• In 85% of patients, the weakness becomes generalized
and often proximal and may be asymmetric
• Despite the muscle weakness , deep tendon reflexes
are preserved
Localization Of Muscular Disorders

• Skeletal muscle disease or myopathies are disorders with


structural changes or functional impairment of muscle.
o Clinical Features:
• Muscle Weakness
- intermittent or persistent
- accompanied by fatigue (pathologic fatigability)
• Muscle pain (Myalgias), Cramps, and Stiffness
- can be localized or generalized
- accompanied by weakness, tenderness to palpation, or
swelling
• Cause of muscular weakness may be LMN, UMN &neuro
muscular lesion
Reference

1. DeJong's The Neurologic Examination, 6th edition


2. Nelson text book of pediatrics twentieth edition
3. Localization in clinical neurology fifth edition
4.child neurology, seventh edition
5. Color Atlas of Neurology
6.Swaiman’s Pediatric Neurology fifth edition
7.up to-date 21.6

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