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Immune Disorders and Infections of the Nervous System

Onset after age 10 and before 60; multiple separate areas of nervous system involvement, affecting primarily white matter;, often in episodes separated in time with improvement over weeks or months but with gradually increasing disability with subsequent attacks. Cause presently unknown, but clearly linked to immune mechanism in multiple sclerosis, lupus of the brain, central nervous system lupus, myasthenia gravis, myesthenia gravis, Guillain Barre Syndrome, multiple sclorosis.


Disease of temperate climates, rare in tropical areas.

Risk of disease depends on geographic locale of second decade of life; immigrants carry that risk with them

Link to animal viruses suggested; Farces Islands, household pets

Onset rare before age 10; extremely rare after age 60; peak incidence in 20s and 30s

Prognosis better if: 

--younger age of onset 

--remitting course

--complete or almost complete recovery between 

Common Neurologic Manifestations

Retrobulbar or optic neuritis: decreased visual acuity; poor color vision centrally; central or paracentral scotoma; afferent pupillary defect multiple sclerosis, lupus of the brain, central nervous system lupus, myasthenia gravis, myesthenia gravis, Guillain Barre Syndrome, multiple sclorosis

Spinal cord: girdle pains; paresis (often asymptomatic); paresthesias-typical picture is an asymmetric combination of corticospinal, cerebellar and posterior column deficits (position, vibration, sense) which improve and then reappear; signs often unknown to patient.

Eye movement disorders: internuclear ophthalmoplegia (unilateral or bilateral); paresis of IIIrd of Vlth cranial nerve with diplopia; nystagmus, including vertical nystagmus (dissociated nystagmus particularly suggestive-different degrees of nystagmus in the two eyes); ptosis (rare); ocular dysmetria (cerebellar sign).

Visual problems: loss of color vision, scotoma, blindness, afferent pupillary defect. Ataxia--of gait, limbs, or speech (scanning).

Coarse postural and intention tremor

Facial pain--often resembling trigeminal neuralgia; rarely facial twitching.

Vestibular disorders: dizziness (may resemble acute labyrinthitis), positional vertigo; tinnitus.

Urinary retention and urgency: 2% present with retention.

Intellectual and personality changes.


Steroids-80 mg. prednisone for 10-15 days with

ACTH--IM or IV, 30-40 Units bid, for 7 days, then taper

Both steroids and ACTH appear to shorten the duration of relapses but do not alter the long-term outcome of the disease.

Cytoxan IV-appears to arrest progress of chronic progressive form for a period of 1-2 years in a majority of patients treated; may require repeat administration or booster doses at

Systemic Lupus Erythematosus

Neuropsychiatric Disorders Associated with SLE

A. Neurologic complications


1. Cardiac disease in SLE

2. Thrombotic tendency

3. Cerebral vasculitis


1. Etiologic factors (micro infants/infections/metabolic, antineuronal antibodies)

Cranial and Peripheral Neuropathy



B. Neuropsychiatric Aspects

1. Cognitive Deficits

2. Psychosis, hallucinosis, delusional disorder, personality disorder

3. Depression and adjustment disorder

4. Complications of therapy


A. Neurologic manifestations

1. Cranial neuropathy (optic neuritis, optic atrophy, Vth nerve, sensory loss and tic, unilateral-bilateral Bell's palsy, VIIIth nerve).

2. Aseptic meningitis-meningeal mass

3. Hydrocephalus

4. Encephalopathy-vasculopathy

5. Seizures

6. Neuroendocrine disorder

7. Spinal mass

8. Peripheral neuropathy (polyneuropathy, mononeuritis multiplex, GBS).

9. Myopathy

Bacterial Meningitis and Cerebral Abscess

A. Bacterial meningitis age related etiology: (E. coil, H-flu, meningococcal, pneumococcal.

B. Tuberculous meningitis and tuberculomas.

C. Cerebral abscesses

D. Subdural empyema

Fungal and Parasitic Infection

A. Cryptococcal meningitis

B. Hydaid disease

C. Cysticercosis

D. Toxoplasmosis

Neurosyphilis and Lyme Disease

A. Syphilis

1. Syphilitic meningitis

2. Meningovascular syphilis (CVA)

3. Tabes dorsalis (spinal)

4. General paresis (parenchymal)

5. Ocular and otic syphilis

6. Congenital syphilis

B. Lyme Disease

Focal, disseminated, erythema migrans

1. Neurological disorders with early dissemination Aseptic meningitis Bell's palsy Radicular neuropathy

2. Neurological manifestations with late persistent infection

Chronic encephalopathy



Viral Infections and Neurology of AIDS

A. Viral meningitis

B. Encephalitis


Herpes viruses/CMV 



C. Retroviruses and Neurology of AIDS

1. HIV 1

AIDS meningitis

AIDS dementia complex


Opportunistic infections

2. HTLV I (tropical spastic paraparesis)

D. Slow Virus infections:

1. PML (progressive multifocal leukoencephalopathy)

2. Subacute sclerosing pan encephalitis

3. Prion disease (Jakob-Creutzfeldt disease)