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36 Cards in this Set

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Lewy bodies - what are they made of and what disease do they go with?
alpha-synuclein - intracellular inclusions

Parkinsons
Symptoms of Parkinsons
T - tremor
R - rigidity - cogwheeling
A - akinesia
P - postural instability
What type of injury does hemiballismus go along with?
Contralateral subthalamic nucleus lesion, which is common with lacunar strokes in a patient with a history of hypertension
What causes neuronal cell death in Huntingtons?
NMDA-R binding/ glutamate toxicity
What nuclei are atrophied with Huntingtons?
Striatum - caudate and putamen - and since these are the big inhibitors of movement, their loss leads to chorea and athetosis
Central pontine myelosis - what causes it and how does the brain's structure change as a result?
Caused by too rapid of a correction of hyponatremia

Abnormal increase in the size of the pons
Symptoms of central pontine myelosis?
Acute paralysis, dysarthria, dysphagia, diplopia, and loss of consciousness
What disease is characterized by acute paralysis, dysarthria, dysphagia, diplopia, and loss of consciousness
Central pontine myelosis
What does damage to the language center in the non-dominant brain cause?
Aprosodia - inability to change inflection, tone, pitch, and rhythm of speach (both their own and other's).
What is global aphasia and what causes it?
Nonfluent aphasia and impaired comprehension.

Damage to both Brocas and Wernickes areas.
What is conduction aphasia and what causes it?
Poor repetition, but fluent speech with intact comprehension.

Damage to the arcus fasciculus, which connects Broca's and Wernicke's areas. Cannot connect speech comprehension to speech production.
Top 2 watershed zones susceptible to injury with hypotension.
-Btw ACA and MCA - Upper arm/upper leg weakness

-Btw MCA and PCA - Defects in higher order visual processing
Berry aneurysms are associated with what other syndromes?
ADPKD, Ehlers-Danlos, and Marfan's
What is a Charcor-Bouchard microaneurysm?
Associated with chronic hypertension. Affects small vessels of the basal ganglia, thalamus, etc. Forms small pseudoaneurysms that are d/t hyaline arteriolosclerosis.
These are the most common cause of intraparenchymal hemorrhage.
Familial forms of alzheimers have what genes (early and late)?
Early onset -
amyloid precursor protein - APP (chromosome 21);
presenilin-1 (chromosome 14)

Late onset -
ApoE4 (chromosome 19)
(ApoE seems to be protective against alzheimers)
What 4 things are found with Alzheimers?
-Widespread cortical atrophy

-Decreased ACh

-Senile plaques - made from extracellular beta-amyloid

-Neurofibrillary tangles - intracellular, abnormally phosphorylated tau proteins that are insoluble cytoskeletal elements - tangles correlate with degree of dementia
Pick's disease
Frontotemporal dementia

Dementia, aphasia, parkinsonian aspects, changes in personality
Findings with Picks disease
-Frontotemporal atrophy

-Pick bodies - intracellular aggregated tau proteins - eosinophilic inclusions found within cortical neurons
Lewy body dementia
Parkinsonism with dementia and hallucinations

Due to an alpha-synuclein defect
Creutzfeld-Jakob disease
Rapidly progressive dementia with myoclonus

Spongiform cortex

Prions form that are resistant to proteases
AIDS dementia
Subacute combined degeneration of corticospinal and dorsal column tracts.

Multinucleated giant cells are diagnostic.
Findings with MS
-Increased protein (IgG) in the CSF
-Oligoclonal bands are diagnostic
-MRI is the gold standard
-Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with preservation of axons
Treatment for MS
Beta-interferon
Immunosuppression
Natalizumab
Charcot's classic triad of MS:
Scanning speech
Intention tremor
Incontinence
Internuclear ophthalmoplegia
Nystagmus
What is Guillain-Barre?
Inflammation and demyelination of peripheral nerves and motor fibers of ventral roots

Sensory effects less severe than motor
How does Guillain-Barre present?
Symmetric ascending motor weakness beginning in distal lower extremities

Facial paralysis in 50% of cases

Autonomic function may be severely affected
What is the treatment and prognosis for Guillain-Barre?
Almost all patients recover

Respiratory support is critical until recovery

Treatment along the way may include plasmapheresis, IVIG
What causes Guillain-Barre?
Associated with infections (Campylobacter jejuni; herpes) > autoimmune attack of the peripheral myelin due to molecular mimicry
Progressive multifocal leukoencephalopathy
Demyelination of CNS due to destruction of oligodendrocytes.

Associated with JC virus and seen in 2-4% of AIDS patients (reactivation)

Rapidly progressive until fatal
Acute disseminated (postinfectious) encephalomyelitis
Multifocal perivenular inflammation and demyelination after infection (chickenpox; measles) or certain vaccinations (rabies; smallpox)
Metachromatic leukodystrophy
AR - lysosomal storage disease - most commonly due to arylsulfatase A deficiency. Buildup of sulfatides leads to impaired production of myelin sheath.
Charcor-Marie-Tooth disease
Hereditary motor and sensory neuropathy (HMSN)

Progressive hereditary nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or the nerve sheath
Sturge-Weber syndrome
Congenital disorder with Port-Wine stains, typically in V1 distribution

Ipsilateral leptomenengeal angiomas

Pheochromocytomas

Can cause glaucoma, seizures, hemiparesis, and mental retardation
Tuberous sclerosis
Hamartomas in the CNS and skin

Adenoma sebaceum - cutaneous angiofibromas usually around nose and mouth

Rhabdomyomas

Ash-leaf lesions

Renal angiomyolipomas

Seizures

Mental retardation
Neurofibromatosis I

VonRecklinghausen's disease
AD - Mutated NF-1 gene - chromosome 17

Cafe au lait spots

Lisch nodules (pigmented iris hamartomas)

Neurofibromas in skin (tumors of schwann cell origin)

Optic gliomas

Pheochromocytomas
Von Hippel-Lindau disease
AD - Mutated tumor suppressor VHL on chromosome 3

Cavernous hemangiomas of skin, mucosa, and organs

Bilateral renal cell carcinoma

Hemangioblastoma in retina, brain stem, and cerebellum

Pheochromocytomas