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Flashcards in Pathology - neuro Deck (41):
1

Senile amyloid plaques, amyloid angiopathy
Intracellular neurofibrillary tangles
decr. ACh
ApoE4

Alzheimer's

incr. risk with Down syndrome, APP

Look for B-amyloid (extracellular) and tau proteins (intracellular), increased intracellular NF tangles --> incr. degree of dementia

decr. ACh in basal nucleus of Meynert

first radiological sign: hippocampal atrophy

2

Pick bodies (spherical tau protein aggregates)
Change in personality

Frontotemporal dementia

Look for parietal lobe sparing

Some parkinsonian aspects as well

3

Dementia + visual hallucinations + parkinsonism

Lewy body dementia

alpha-synuclein defect

4

Protein structure in CJD

Prions form beta-pleated sheet resistant to proteases

5

MS findings

incr IgG in CSF (oligoclonal bands)

MRI is gold standard - periventricular plaques (loss of oligos and reactive gliosis) with destruction of axons

Multiple white matter lesions separated in space and time

6

Autoimmune condition that destroys Schwann cells --> ascending weakness

Guillain-Barre syndrome

Inflammation and demyelination of peripheral nerves and motor fibers (molecular mimicry s/p infection) --> symmetric, ascending weakness

Look for incr. CSF protein w/ normal cells (albuminocytologic dissociation) that may be causing papilledema

7

Autosomal dominant, assoc. with scoliosis and foot deformities

Charcot-Marie-Tooth

defective production of proteins involved in the structure and function of peripheral nerves or the myelin heath

8

Excess buildup of very long chain fatty acids in the nervous system, adrenal gland, testes

Adrenoleukodystrophy

X-linked, seen in males, disruption of metabolism

Progressive, leads to coma/death or adrenal crisis

9

Origin of partial seizures

Medial temporal lobe

preceded by aura, can secondarily generalize

simple = consciousness intact, complex = loss of consciousness

10

headaches, ***bilateral***, steady pain, no aura

Tension

Tx: analgesics, NSAIDs

11

Mechanism of migraine headaches

Irritation of CN V, meninges of blood vessels
release of substance P, calcitonin gene-related peptide, vasoactive peptides

POUND: pulsatile, one-day, unilateral, nausea, disabling

12

Peripheral vs. central vertigo

Peripheral: more common, inner ear etiology, delayed horizontal nystagmus

Central: brain stem/cerebellar lesion, diplopia/dysmetria/skew deviation, immediate nystagmus in any direction, assoc. w/ focal neuro findings

13

Port-wine stain on face
ipsilateral leptomeningal angioma
intellectual disability
early onset glaucoma

Sturge-Weber syndrome (affect small blood vessels/capillaries)

GNAQ gene activation - developmental anomaly of neural crest derivatives

STURGE: stain, tram track calcifications, unilateral, retardation, glaucoma, GNAQ gene, epilepsy

14

Neuro complications of tuberous sclerosis

Mental retardation, seizures, incr. incidence of subependymal astrocytomas and ungual fibromas

15

Hemangioblastomas in retina, brain stem, cerebellum, spine
Cavernous hemangiomas everywhere

von Hippel Landau disease

bilateral renal cell carcinomas + pheochromocytomas

16

Brain tumor that...
... crosses corpus callosum (butterfly glioma)
... stains for GFAP
... pseudopalisading pleomorphic tumor cells

GBM (adults)

1 yr survival

17

Brain tumor that...
... extra-axial, found near surface of brain
... arise from arachnoid villi
... spindle cells in whorl pattern + psmammoma bodies

Meningioma (adults)

Often asymptomatic, may present with seizures or focal signs

18

Brain tumor that...
... cerebellar, assoc. w/ vHL syndrome
... can produce EPO, causing polycythemia
... closely arranged capillaries

Hemangioblastoma (adults)

19

Brain tumor that...
... stains for S-100
... cerebellopontine angle

Schwannoma (adults)

bilateral in NF2

20

Brain tumor that...
... rare, slow growing
... chicken-wire pattern, fried egg cells

Oligodendroglioma (adults)

21

Brain tumor that...
... well circumscribed in posterior fossa
... grossly, cystic and solid components
... stains for GFAP

Pilocytic astrocytoma (children)

benign, good prognosis

22

Brain tumor that...
... highly malignant cerebellar tumor, commonly in the vermis
... neuroectodermal
... can compress 4th ventricle

Medulloblastoma (children)

23

Brain tumor that...
... found in 4th ventricle
... perivascular rosettes
... poor prognosis

Ependymoma (children)

perivascular rosettes

24

Cingulate herniation under falx cerebri

Compression of anterior cerebral artery

Sensory and motor loss in the lower extremities

25

Downward transtentorial herniation

Caudal displacement of brain stem, rupture of paramedian basilar artery branches, Duret hemorrhages

usually fatal

26

Uncal herniation

Medial temporal lobe

Compresses ipsilateral CNIII (blown pupil), ipsilateral PCA (contralateral vision loss), ipsilateral paresis

27

Cerebellar tonsil herniation

Coma and death if there is compression of brain stem

28

Lacunar infarcts

due to hypertensive changes in the arterioles

Look for arteriolar sclerosis

ischemic changes

29

Charcot-Bouchard pseudoaneurysms

less than 1mm in diameter

long-standing hypertension

hemorrhagic changes

30

Lesions found in Wernicke encephalopathy

Foci of hemorrhage and necrosis in mamillary bodies

31

Complications of subarachnoid hemorrhage

secondary arterial vasospasm --> cerebral ischemia

due to impaired brain autoregulation

32

Autosomal recessive, trinucleotide repeat, impairment in mitochondrial functioning, degeneration of spinal cord tracts

Friedrich ataxia

gait ataxia, scoliosis, foot abnormalities, hypertrophic cardiomyopathy

33

impaired upward gaze + hormone secretion (bhCG) + obstructive hydrocephalus

Pineal gland tumor

Most commonly, germinoma

34

Sensory ataxia + lancinating pains + urinary incontinence + Argyll Robertson pupil

Tabes dorsalis caused by syphillis

35

Dystrophies genetics

Duchenne - loss of protein that normally helps anchor muscle fibers (connects actin cytoskeleton to dystroglycan, which connect to ECM)

Myotonic - abnormal expression of myotonin protein kinase, myotonia, cataracts, testicular atrophy,

36

Liquefactive necrosis

Cystic cavity surrounded by gliosis

Release of lysosomal enzymes from ischemic neurons results in degradation of local tissue

phagocytes clean up, then area is surrounded by astrocytes to form a glial scar

37

Alcohol withdrawal

Tremor then autonomic dysfunction then seizures then delirium tremens (hallucinations, confusion, severe symp discharge)

38

Spinal cord syndromes
Tabes dorsalis vs. B12 deficiency vs. ASA syndrome

Tabes dorsalis - dorsal columns, dorsal roots (loss of position/vibration)

B12 deficiency - dorsal columns, lateral corticospinal tracts, axonal degeneration of peripheral nerves (loss of position/vibration, UMN signs, numbness/parasthesia)

ASA - complete loss of strength below the level of injury with loss of pain and temp sensation

39

Global cerebral ischemia

Affects hippocampus first!

Also Purkinje cells of cerebellum

40

Complications of meningitis

Communicating hydrocephalus - decr. function of the arachnoid granulations

41

Association with cerebral amyloid angiopathy

Recurrent hemorrhagic strokes