DISEASES OF THE CENTRAL

NERVOUS SYSTEM
 CEREBRAL EDEMA

may be vasogenic or, less commonly, cytotoxicThis

edemaVasogenic :

Results from increased pressure or permeability of capillaries and

venules

: It is usually seen around

Brain abscesses .1
Brain tumors .2
Brain contusions .3
Brain infarcts .4

The accumulated fluid is intercellular

:Cytotoxic edema

Results from cell injury with metabolic

derangements

The accumulated fluid is intracellular

:Effects of cerebral edema

Increased intracranial pressure which may be

followed by brain herniation
 HYDROCEPHALUS

:Definitions

Hydrocephalus: Increased volume of CSF within the cranial cavity

Internal hydrocephalus (or simply “hydrocephalus”): Increased CSF

within the ventricular system which thus becomes enlarged

External hydrocephalus: Increased CSF within the sub-arachnoid space

Communicating hydrocephalus: Hydrocephalus in which CSF flows freely

from ventricular system into sub-arachnoid space

Non-communicating hydrocephalus: Hydrocephalus in which CSF does not

flow freely from ventricular system into sub-arachnoid space

Compensatory hydrocephalus: Increased volume of CSF consequent to

loss of brain tissue
: Causes
.1Increased production of CSF seen in association with papillary tumors of
the choroid plexus

.2Decreased absorption of CSF occurring in association with sub-

arachnoid hemorrhage (due to obliteration of the arachnoid villi by
( hemosiderin-containing macrophages

.3Cerebral atrophy . The hydrocephalus here is not accompanied by

increased intracranial pressure

.4Obstruction to CSF flow . This is the commonest cause of hydrocephalus.

: It may result from
a) Congenital stenosis of ventricular foramina or aqueduct
b) Pressure on the ventricular foramina or aqueduct by an intracranial
tumor, brain abscess or tuberculoma
c) Meningitis due to obliteration of 4th ventricle foramina or
sub-arachnoid space following organization of the inflammatory
exudate in the base of the brain
:Pathology

Obstruction of one of the foramina of Monroe leads to .1

enlargement of one lateral ventricle

Obstruction of the third ventricle or aqueduct leads to .2

enlargement of both lateral ventricles

Obstruction of the foramina of the 4th ventricle leads to .3

enlargement of the entire ventricular system

.4Obstruction at the sub-arachnoid space also leads to

enlargement of the entire ventricular system but, in
contrast to the above three conditions,the hydrocephalus
is of the communicating type
:Effects

Enlargement of the head if the .1

hydrocephalus develops before
closure of the cranial sutures
((children

Enlargement of the .2
ventricles and atrophy of
brain tissue

Increased intracranial pressure .3

INCREASED INTRACRANIAL PRESSURE

An increase of CSF pressure above 200mm. water

with the patient recumbent

Causes: Increase of the intracranial volume, mainly

:due to

Brain edema .1
Hydrocephalus .2
Intracranial hemorrhage .3
Brain abscess .4
Intracranial tumors .5
:Clinical manifestations

Headache .1

Vomiting .2

Papilledema (edema of the optic disc) caused by .3

compression of the retinal vein as it traverses the
subarachnoid space in the optic nerve sheath

Increased systolic BP .4

Slow pulse .5

Decreased consciousness & eventually coma .6

 HERNIATION OF THE BRAIN

Expansion of the brain particularly

when localized may lead to
displacement of the brain against
the falx cerebri or the
tentorium cerebelli. If the
,expansion is severe enough
herneation of the brain will occur
i.e. protrusion of a part of the
brain most commonly under the
falx or through the tentorial
appreture or the foramen
.magnum
:Possible effects

Compression of anterior or posterior cerebral .1

arteries

Compression of third cranial nerve .2

Compression of the brain stem with distortion of the .3

respiratory centers in the medulla

Obstruction of CSF flow through the fourth ventricle .4

leading to further increase of intra-cranial pressure

Hemorrhage into the pons & midbrain .5

 CEREBRO-VASCULAR DISEASES

Abnormalities of the brain resulting from

.pathological processes of blood vessels

The term “stroke” is the clinical designation applied

to these diseases particularly when symptoms
.develop acutely

The major cerebro-vascular diseases are

thrombosis ,embolism andhemorrhage. The
first two often lead tocerebral infarction
:CEREBRAL INFARCTION

:Risk factors

Atheroma - Hypertension - Abnormal serum lipids -

Coronary artery disease - Cardiac failure -
Smoking - Atrial fibrillation - Diet - Obesity -
Alcohol
:Causes

Thrombosis.1: on top of atherosclerosis of

a) Anterior, posterior & middle cerebral arteries

b) Basilar artery

c) Internal carotid arteries

d) Vertebral arteries

The commonestintra-cranial site of thrombotic vascular

occlusion is themiddle cerebral artery

The commonestextra-cranial site of thrombotic vascular

occlusion is the origin of aninternal carotid artery
Embolism:from thrombi in .2

a) The left side of the heart:

vegetations of subacute bacterial
endocarditis or mural thrombi in
atrial fibrillation or myocardial infarction

b) The aorta

c) Neck arteries
:Miscellaneous causes .3

a) Systemic circulatory insufficiency in the presence of

severe atherosclerotic narrowing of cerebral or neck
arteries

b) Arteritis , either infective or due to collagen diseases

c) Blood diseases e.g polycythemia, sickle cell disease

d) Pregnancy and perpeurium

e) Oral contraceptive pills

f) Drug addiction
:Pathology

Thecommonest site for infarction is the

distribution of the middle cerebral artery
i.e.basal ganglia and internal capsule

Grossly, the infarct may be hemorrhagic or

pale & eventually undergoes liquefaction &
absorption to be replaced or surrounded
.by glial tissue
1
:Effects

Depend on the site of the infarct, most

commonlyhemiplegia due to
destruction of the pyramidal tract in
the internal capsule
SPONTANEOUS INTRACEREBAL
:(INTRAPARENCHYMAL) HEMORRHAGE

:Risk factors

Hypertension - Vascular malformations - Arteritis -

Bleeding diathesis
:Causes

Hypertension due to rupture of one of the .1

numerous micro-aneurysms found in the brain,
usually following a sudden rise of blood pressure.
.This is the commonest cause

Rupture of Vascular malformations .2

Hemorrhage into a brain tumor .3

Blood diseases e.g acute leukemia .4

:Pathology

Thecommonest site is the region of

the basal ganglia & internal capsule

The blood in the brain may dissect its

way until it reaches the ventricles or
the sub-arachnoid space
:Effects

Sudden severe rise of intracranial pressure .1

Herniation of the brain .2

Signs of meningeal irritation if the blood reaches .3

the subarachnoid space

Hemiplegia due to destruction of the pyramidal .4

tract in the internal capsule
:SUB-ARACHNOID HEMORRHAGE

s:Cause

(Rupture of a berry aneurysm (65% .1

(Rupture of a vascular malformation (5% .2

Extension of intracerebral or intraventricular .3

hemorrhage into the sub-arachnoid space

Blood dyscrasias .4

(Unknown cause (25% .5

:Berry (sacular, congenital) aneurysm

Occurs in arteries of the circle of Willis at the

base of the brain in1 - 2% of adults and is
multiple in 10 - 15%. of cases

It results from a congenital defect in the

.media of the artery at a bifurcation
:Other types of aneurysms

Atherosclerotic aneurysm: fusiform, .1

mostly in the basilar artery

Mycotic aneurysm: resulting from .2

destruction of the vessel wall by mild
inflammation e.g following impaction of
emboli derived from vegetations of
subacute bacterial endocarditis
:Effects of aneurysms

Rupture leading to sub-arachnoid .1

hemorrhage : Berry aneurysm usually
ruptures between 40 and 50 years of
age and atherosclerotic aneurysm in
the 6th decade or later

Thrombosis in the aneurysm which .2

may lead to brain infarction

Hydrocephalus .3
:Vascular Malformations

Include:cavernous angiomas ,capillary

telangiectasias (hemangiomas) andarteriovenous
malformation. (AVM) which is the most important

AVM appearsgrossly as a mass of tortuous blood

vessels occuring most commonly in relation to
.the middle cerebral artery
It may rupture leading tosub-arachnoid or intra-
cerebral hemorrhage most commonly between
the ages of 10 and 30 yrs
 INFECTIONS OF THE CNS
:PYOGENIC INFECTIONS

Acute suppurative ( pyogenic, bacterial, septic)

:meningitis

Acute suppurative inflammation of the leptomeninges

((pia/arachnoid

:Causative organisms

Niesseria meningitidis (meningococcus): the commonest .1

E.coli: in neonates .2

Other organisms: Strept. pneumonie, H. influenzae .3

:Mode of Infection

Droplet inhalation from nasopharyngeal carriers. .1

The organisms reach the meninges via the
blood stream. This isthe usual mode of
infection particularly in meningococcal
meningitis

Local spread of infection from middle ear or .2

paranasal sinuses

Direct infection through a compound fracture of .3

the skull
:Pathology

:Gross

The sub-arachnoid space is .1

filled with pus, thickest and
most abundant in the
base of the brain and the
sulci

The meningeal vessels are .2

dilated and engorged with
blood
:Microscopic

The sub-arachnoid .1
space is filled with
neutrophils, pus cells and
fibrin

The meningeal vessels .2

dilated & engorged with
blood
:CSF Changes

Increased amount, under pressure and turbid or .1

frankly purulent

Increased protein .2

Increased cells, mainly neutrophils .3

Markedly decreased or absent glucose .4

Causative organism are present & can be .5

detected in Gram- stained films or after culture
:Effects & Complications

:Septicemia which may lead to .1

a)Acute bacterial endocarditis
b)Acute adrenal insufficiency due to hemorrhage in the adrenals
( glands (Waterhouse-Friderichsen syndrome

Hydrocephalus .2

Ptosis, diplopia and squint due to damage of the 3rd, 4th and 6th .3
cranial nerves following organization of the inflammatory
exudate in the base of the brain

Vascular thrombosis which may lead to ischemic lesions of the .4

brain

Encephalitis due to direct spread of infection to the brain .5

Increased intracranial pressure .6

:Brain Abscess

Causative organisms: The commonest are Staph and Strept

:Mode of infection

Local spread of infection from suppurative otitis media, .1

mastoiditis or sinusitis

Blood-borne infection from suppurative diseases of the lung .3

(vertebral system of veins) or, less commonly, acute
(bacterial endocarditis (systemic pyemia

Direct infection through a compound fracture of the skull .2

:Sites

.1Temporal lobe in cases complicatingotitis

media

.2Cerebellum in cases complicatingmastoiditis

.3Frontal lobe in cases complicatingsinusitis

.4Parietal lobe and cerebellum in cases

complicatingsuppurative lung diseases

.5Multiple abscesses allover the brain inpyemia

:Pathology

:Acute abscess
An irregular cavity with a thin wall, filled with fluid
pus and surrounded by a zone of congestion

:Chronic abscess
A regular, spherical or oval cavity with a thick fibro-
glial wall and a smooth lining, filled with thick
.pus
Microscopically, fibrous tissue, hypertrophied
astrocytes and foamy macrophages are seen in
the abscess wall
:Effects & complications

Increased intracranial pressure .1

Focal neurological manifestations depending on .2

the site of abscess

Hydrocephalus .3

Rupture into the sub-arachnoid space or .4

ventricles leading to meningitis
:TUBERCULOSIS

Is alwayssecondary and usually

results fromhematogenous dissemination of

.a tuberculous focus elsewhere in the body

It takes two main forms : tuberculous

meningitis and tuberculoma
:Tuberculous meningitis

:Mode of infection

Blood-borne infection .1

Rarely, local spread of infection from a .2

tuberculous vertebral body
:Pathology

:Gross

A gelatinous exudate is found at the base of the .1

brain

Minute tubercles may be detected in the .2

(meninges or the surface of the brain (hand lens

:Microscopic

A tuberculous reaction with obliterative endarteritis

is seen in the meninges
:CSF Changes

Increased volume, under pressure and clear or .1

opalescent

Increased protein .2

Increased cells, mainly lymphocytes .3

Normal or slightly reduced glucose .4

A fine fibrin web develops on the surface of on .5

standing
:Effects & Complications

Ptosis, diplopia & squint .1

Hydrocephalus .2

Focal neurological manifestation due to small .3

brain infarcts resulting from obliterative
endarteritis

Increased intracranial pressure .4

:Tuberculoma

A localized tuberculous lesion that appears

grossly as a well circumscribed, often
encapsulated mass that may show central
caseous necrosis or dystrophic
.calcification

It is oftenmistaken clinically and

radiologically for an.intra-cranial tumor
13
:Effects

Increased intracranial pressure .1

Focal neurological manifestations .2

Hydrocephalus .3

Rupture into subarachnoid space causing .4

tuberculous meningitis
:NEUROSYPHILIS

Although spirochetes may reach the CNS as

early as the second stage of syphilis,
lesions appear only in the third stage
(tertiary or meningo-vascular
syphilis) or as late as 20 years after the
primary infection (quaternary or
parenchymatous syphilis which includes
general paralysis of the insane and
.(tabes dorsalis
:Tertiary neurosyphilis

:Characterised by

A subacute meningitis with gummas .1

Syphilitic obliterative endarteritis .2

which may lead to focal ischemic
lesions in the brain
:(General Paralysis of the Insane (GPI

Characterised by a subacute encephalitis

:which leads to

Progressive dementia .1

Various motor and sensory disturbances .2

:Tabes Dorsalis

Characterized bydegeneration of
posterior spinal nerve roots (most
commonly the lumbo-sacral) leading
loss of sensation with consequent
degenerative changes in the joints
Charcot’s joint ) andataxia)
VIRAL INFECTIONS :OF THE CNS

Virusesreach the CNS by theblood stream or

rarelyalong peripheral nerves.( (rabies virus

They mayenter the body: through

The skin or mucous membranes (Herpes simplex .1
( virus
The alimentary tract (enteroviruses), or by .2
(Arthropod (insect) bites (arboviruses .3

Viral infections of CNS may take the form of : viral

(aseptic) meningitis or viral encephalitis
:Viral aseptic meningitis

A mild, usually self-limited meningitis

Caused most commonly by one of the enteroviruses

Pathology: Infiltration of meninges and subarachnoid space

by lymphocytes, plasma cells and macrophages

:CSF changes

Pressure slightly increased .1

Protein slightly to moderately increased .2
Cells increased, mainly lymphocytes .3
Normal glucose level .4
:Viral encephalitis

A viral infection of the brain

parenchyma, almost always
accompanied by inflammation of
meninges (meningo-encephalitis)
and sometimes with simultaneous
involvement of the spinal cord
((encephalo-myelitis
Common microscopic
:features

Perivascular & .1
parenchymal infiltration
by lymphocytes & plasma
cell

Neuronal degeneration .2
neuronophagia &

(Glial cell reaction (gliosis .3

:Poliomyelitis

An acute viral infection of the anterior horn cells

of the spinal cord, particularly those of the
lumbar and cervical enlargements, leading to
paralysis of limb muscles. Motor nuclei of the
.brain stem are also commonly affected

It usually occurs in infants & young children

and may appear in sporadic or epidemic forms

Cause: Poliovirus, an RNA enterovirus

Mode of infection: Ingestion of food or drink

contaminated by the virus, which
multiplies in the mucosa and lymphoid
tissue of the intestine, then travels to the
(CNS via the blood stream (viremia

Incubation period: 1 - 3 weeks

:Pathology

Perivascular infiltration by lymphocytes and .1

plasma cells

Degeneration of the neurons and neuronophagia .2

Wallerian degeneration and demyelination in the .3

axons of motor neurones

Denervation atrophy of muscles supplied by .4

damaged neurons
:Clinical features

Prodromal stage characterized by fever, .1

headache, maliase, nausea, vomiting and sore
throat. It either subsides (abortive
:poliomyelitis) or progresses to

Preparalytic stage characterized by stiffness of .2

the neck, pain in the back and limbs and signs
of meningeal irritation. It either subsides or
:progresses to

Paralytic stage characterized by flaccid paralysis .3

of limb muscles
:Rabies

Cause: Rhabdovirus, an RNA virus

Mode of infection: Bite of a rabied animal, most

.commonly a dog
The virus is present in the saliva of the animal that
.contaminates the bite wound
.It travels to the CNS along peripheral nerves

IP: 1-3 months depending on the distance between

the bite and the CNS
:Pathology

A severe diffuse viral encephalitis is seen,

.affecting mainly the gray matter

It is distinguished from other viral infections

of the brain by the presence of
characteristic eosinophilic inclusions in the
cytoplasm of the damaged neurons known
asNegri bodies. Rabies virus can be
detected within these bodies by electron
microscopy or immunohistochemistry
:Clinical Features

The earliest symptom is hydrophobia (spasm of .1

(the pharyngeal muscles on attempt to drink
Excitability .2

Flaccid paralysis .3

Death results from respiratory center failure .4

 DEMYELINATING DISEASES

Acquired conditions characterized by

destruction of myelin with relative
.preservation of axons

The most important are multiple

(disseminated) sclerosis and acute
disseminated encephalomyelitis
:MULTIPLE SCLEROSIS

.The commonest demyelinating disease

Characterized by multiple foci of demyelination &

gliosis in the brain & spinal cord

:Pathology

Grossly, discrete foci of demyelination & gliosis

known asplaques are seen, most commonly
around the ventricles and in the brain stem,
spinal cord & optic nerves
,Microscopically

:Acute lesions show

Demyelination with infiltration.1

by lipid containing macrophages
(gitter cells)

Perivascular infiltration by lymphocytes and plasma .2

cells

Old lesions show replacement of the cellular infiltrate

by gliosis
:Clinical Features

The disease is rare in childhood and after the age .1

of 50 years

It is chronic characterized by relapses and .2

remissions over many years

Clinical manifestations include abnormalities of .3

vision, parasthesia, paraplegia and ataxia
depending on the area of the CNS affected
:Etiology
Not established but the following factors may
ed:involvbe

Genetic factors .1

Environmental factors .2

Immunological factors : MS may result from an .3

autoimmune reaction against a neural tissue
component OR an abnormal immune response
to a neurotropic virus
:ACUTE DISSEMINATED ENCEPHALOMYELITIS

Ausually self-limited demyelinating disease

occuring in children and young adults, following
:either

An acute viral disease eg mumps, measles, .1

rubella, chicken pox (post-infectious
(encephalomyelitis

A viral immunization ( .2post-vaccinal

(encephalomyelitis

Itmay be due to an autoimmune reaction against

myeline basic protein or an abnormal immune
response to virus or virus-infected cells
 DEGENERATIVE DISEASES

Diseases of the gray matter

characterized by progressive loss of
neurons in the absence of any
previous or associated neurological
.disease

The most important are Alzheimer’s

disease and motor neurone disease
:ALZHEIMER’S DSEASE

A degenerative disease of the CNS

.characterized by progressive dementia

It usually begins after the age of 50 years

and is the commonest cause of dementia
.in the elderly

It is commoner in females
:Etiology

:Obscure but the following factors may be involved

A genetic predisposition as supported by the following .1

: observations
a) The disease is familial in 5 - 10% of cases
b) Pathological changes identical to those of Alzheimer’s
disease are seen in patients with trisomy 21 (Down’s
syndrome) who survive beyond the age of 45 yrs

Slow viral infection .2

Trauma .3

Intoxication by heavy metals eg lead .4

:Pathology

.1Grossly , the brain is atrophied, weighing 1000g. or less. The

atrophy is most marked in the frontal lobes

.2Microscopically:, there is

a) Generalized loss of neurones and gliosis

b) A large number ofsenile (Alzheimer’s) plaques in the gray

matter, composed of masses of argyrophilic filaments and
granules, often having a central core ofamyloid material

c) A large number ofneurofibrillary tangles in the cytoplasm of

neurones , appearing as bundles of argyrophilic filaments
that surround or displace the nucleus
:MOTOR NEURONE DISEASE

A degenerative disease of the CNS characterized by

.progressive loss of motor neurons in adults

It is commoner in males and is

. usually fatal in 2-3 yrs

Theetiology unknown but genetic factors ,

infection by polio virus and intoxication by heavy
metals may be involved
Threevariants: are recognized

Progressive muscle atrophy with selective .1

involvement of the cervical spinal cord

Progressive bulbar palsy with involvement of .2

cranial nerve nuclei in the brain stem

Amyotrophic lateral sclerosis with involvement of .3

upper motor neurones
 TUMORS OF THE CNS

:GENERAL CONSIDERATIONS

Distinction between “benign” and “malignant” lesions is less sharp .1

than in other organs

The anatomic location of the neoplasm can have lethal .2

consequences irrespective of its histologic type

Even the most aggressive types rarely metastasize ouside the .3

CNS, however, they may become disseminated by the CSF to
other sites in the CNS.CSF dissemination occurs most
commonly with ependymoma and medulloblastoma

.4Common effects include: Increased intra-cranial pressure, brain

herniation, hydrocephalus, brain edema, hemorrhage, and focal
neurologic manifestations depending on the site of the tumor

Most arise from glial cells, particularly astrocytes i.e. are .5

astrocytomas
:GLIOMAS

:Derived from glial cells and include

Astrocytomas derived from astrocytes .1

Oligodendroglioma derived from oligodendroglia .2

Ependymomas derived from epedymal cells .3

:ASTROCYTOMAS

I. Diffuse fibrillary astrocytoma (WHO grade II

:(astrocytoma

Sites: Most common in cerebrum

Age: (Most common in adults (30 – 50 years

Gross appearance: Poorly defined, firm or soft

grayish mass
:Microscopic

Consists of stellate astrocytes irregularly

distributed in a fibrillary background made
up of astrocytic cell processes

:Prognosis

Moderate with a mean survival of 5 years

II. Anaplastic astrocytoma (malignant
:(astrocytoma, WHO grade III astrocytoma

Differs from diffuse fibrillary astrocytoma as

:follows

More cellular .1

Shows nuclear pleomorphism and mitosis .2

Shows mild endothelial cell proliferation .3

III. Glioblastoma multiforme (GBM, WHO grade IV
:(astrocytoma

:Differs from grade III astrocytoma as follows

Grossly, .1well demarcated and often shows necrosis and

hemorrhage on the cut surface

More cellular .2

Shows more nuclear pleomorphism and more frequent mitosis .3

Shows multinucleate tumor giant cells .4

Shows marked endothelial cell proliferation leading to “glomeuloid .5

“bodies

Shows areas of necrosis surrounded by “pseudopalisades” of .6

neoplastic cells
Prognosis of anaplastic
:astrocytoma and GBM

Very poor particularly for GBM which

has a mean survival of 8 – 10
months
IV. Pilocytic astrocytoma (WHO grade I
:(astrocytoma

Sites: Most common in the cerebellum, the

floor and walls of the third ventricle and
the optic nerves

Age: Children and young adults

Gross: Cystic with a “mural nodule” in the

cyst wall
Microscopic:: Consists of

Cellular areas made up of bipolar cells .1

with long, thin processes

Less cellular areas with microcysts and .2

Rosenthal fibers

Prognosis: Very good

:NEURONAL TUMORS

The most important iscerebral

neuroblastoma which occurs in
children and ismalignant with a poor
prognosis. It resembles peripheral
.neuroblastomas microscopically
:POORLY DIFFERENTIATED (EMBRYONAL) TUMORS

The commonest is Medulloblastoma

:Medulloblastoma

Age: Mostly children

Site: Exclusively, thecerebellum

Gross: Awell defined, soft, gray mass mostly located in the

(midline of the cerebellum (vermis
:Microscopic

A highly cellular tumor composed of

sheets of small, undifferentiated cells
with scanty cytoplasm and
hyperchromatic nuclei, forming
variable numbers ofHomer-Wright
rosettes
:Prognosis

The tumor ishighly malignant and

commonly disseminates by CSF.
However, it ismarkedly
radiosensitive, so that the 5-year
survival after total excision may be
as high as 75%
:OTHER PARENCHYMAL TUMORS

Primary brain lymphoma .1

Germ cell tumors .2

Pineal body tumors .3

:MENINGEOMAS

Benign tumors that arise from the meningothelial

cells of the arachnoid

Age: Adults

Site: Along the surfaces of the brain, rarely within

the ventricular system

Gross: Well encapsulated, firm grayish mass,

attached to the dura and compressing the brain
Microscopic:: There are several histologic patterns

Syncytial (meningothelial), composed of .1whorls of oval cells with

indistinct cell boundaries. A variable number of calcospherules
(psammoma bodies) are present in the centers of the neoplastic
cell whorls

Fibroblastic, composed of fascicles of spindly cells resembling .2

fibroblasts

Transitional, showing features of syncytial and fibroblastic types .3

Psammomatous containing numerous psammoma bodies .4

Angioblastic which is .5highly vascular and folows anaggressive

behavior. This now considered to bea hemangiopericytoma of the
brain rather than a type of meningioma
:PERIPHERAL NERVE SHEATH TUMORS

:(Schwannoma (neurilemoma .1

A benign tumor derived fromSchwann cells

:Sites

a) Eighth cranial nerve at thecerebellopontine angle

((acoustic neuroma

b) Spinal nerve roots in thespinal canal

:Pathology

a) Asolitary encapsulated mass, attached to the nerve

of origin
b) Microscopically, it shows a
biphasic pattern consisting of
cellular areas made up of
spindly cells showingnuclear
palisading (Antoni type A) and
less cellular areas made up of
stellate cells widely separated
by a myxoid stroma containing
(microcysts (Antoni type B
: Neurofibroma .2

A benign tumor consisting offibroblasts, Schwann

.cells and collagen

.Site: Spinal nerve roots in thespinal canal

Pathology: Anon-encapsulated mass

.“interrupting” the nerve of origin
It may besolitary or multiple (in multiple
neurofibromatosis or von Recklinghausen’s
disease which is an autosomal dominant
.(hereditary disorder
:METASTATIC TUMORS

Represent 50% of intracranial tumors

May occur in the brain or meninges

The commonest primary sites are the

lungs, breast, kidney and GIT
:PARANEOPLASTIC SYNDROMES

Occur most commonly in association with

small cell carcinoma of the lung and
:include

.1Cerebellar degeneration leading to

cerebellar signs and symptoms

.2Limbic encephalitis leading to subacute

dementia

.3Subacute sensory neuropathy

DISEASES OF PERIPHERAL
NERVES
 PERIPHERAL NEUROPATHY

A degenerative disease of peripheral

nerves leading to muscle atrophy
and weakness, sensory disturbances
.and autonomic defects
:CAUSES

.1Immune-mediated (Acute “idiopathic” inflammatory

.( polyneuropathy,Guillain-Barre syndrome

.2Infections: Diphtheria, leprosy, varicella zoster

.3Diabetes mellitus

.4Vitamin defficiencies: Thiamine, B12, B6, E

.5Toxic chemicals: Alcohol, lead, arsenic, hydrocarbons

.6Drugs: Diethyl hydantoin, dapsone

( .7Malignancies (paraneoplastic syndromes

:GUILLAIN-BARRE SYNDROME

. This is thecommonest peripheral neuropathy

It is usuallypreceded by an acute influenza-like

.illness

. It is most probablyimmunologically mediated

Most cases recover in one to several weeks

but rare cases develop chronic disease with
.relapses and remissions
 TUMORS OF PERIPHERAL NERVES

.1SCHWANNOMA (NEURILEMOMA): See tumors

of CNS

.2NEUROFIBROMA: This may change into a

malignant peripheral nerve sheath tumor
particularly in patients with neurofibromatosis
((see tumors of CNS

.3MALIGNANT PERIPHERAL NERVE SHEATH

TUMOR (MALGNANT SCHWANNOMA,
NEUROFIBROSARCOMA): Rare. May arise de
novo or in a neurofibroma, particularly in
patients with neurofibromatosis