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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.
Epidemiology
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.
Treatment
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
Stroke
1. Cardiac disease in SLE
2. Thrombotic tendency
3. Cerebral vasculitis
Seizures
1. Etiologic factors (micro infants/infections/metabolic, antineuronal antibodies)
Cranial and Peripheral Neuropathy
Chorea
Meningitis
B. Neuropsychiatric Aspects
1. Cognitive Deficits
2. Psychosis, hallucinosis, delusional disorder, personality disorder
3. Depression and adjustment disorder
4. Complications of therapy
Neurosarcoidosis
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
Neuropathy
Encephalomyelitis
Viral Infections and Neurology of AIDS
A. Viral meningitis
B. Encephalitis
Rabies
Herpes viruses/CMV
Enteroviruses
Arboviruses
C. Retroviruses and Neurology of AIDS
1. HIV 1
AIDS meningitis
AIDS dementia complex
Myelopathy
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)