Pathology - neuro

Question 1

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

Answer 1

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

Question 2

Pick bodies (spherical tau protein aggregates)
Change in personality

Answer 2

Frontotemporal dementia

Look for parietal lobe sparing

Some parkinsonian aspects as well

Question 3

Dementia + visual hallucinations + parkinsonism

Answer 3

Lewy body dementia

alpha-synuclein defect

Question 4

Protein structure in CJD

Answer 4

Prions form beta-pleated sheet resistant to proteases

Question 5

MS findings

Answer 5

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

Question 6

Autoimmune condition that destroys Schwann cells –> ascending weakness

Answer 6

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

Question 7

Autosomal dominant, assoc. with scoliosis and foot deformities

Answer 7

Charcot-Marie-Tooth

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

Question 8

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

Answer 8

Adrenoleukodystrophy

X-linked, seen in males, disruption of metabolism

Progressive, leads to coma/death or adrenal crisis

Question 9

Origin of partial seizures

Answer 9

Medial temporal lobe

preceded by aura, can secondarily generalize

simple = consciousness intact, complex = loss of consciousness

Question 10

headaches, bilateral, steady pain, no aura

Answer 10

Tension

Tx: analgesics, NSAIDs

Question 11

Mechanism of migraine headaches

Answer 11

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

Question 12

Peripheral vs. central vertigo

Answer 12

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

Question 13

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

Answer 13

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

Question 14

Neuro complications of tuberous sclerosis

Answer 14

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

Question 15

Hemangioblastomas in retina, brain stem, cerebellum, spine

Cavernous hemangiomas everywhere

Answer 15

von Hippel Landau disease

bilateral renal cell carcinomas + pheochromocytomas

Question 16

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

Answer 16

GBM (adults)

1 yr survival

Question 17

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

Answer 17

Meningioma (adults)

Often asymptomatic, may present with seizures or focal signs

Question 18

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

Answer 18

Hemangioblastoma (adults)

Question 19

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

Answer 19

Schwannoma (adults)

bilateral in NF2

Question 20

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

Answer 20

Oligodendroglioma (adults)

Question 21

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

Answer 21

Pilocytic astrocytoma (children)

benign, good prognosis

Question 22

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

Answer 22

Medulloblastoma (children)

Question 23

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

Answer 23

Ependymoma (children)

perivascular rosettes

Question 24

Cingulate herniation under falx cerebri

Answer 24

Compression of anterior cerebral artery

Sensory and motor loss in the lower extremities

Question 25

Downward transtentorial herniation

Answer 25

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

Question 26

Uncal herniation

Answer 26

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

Question 27

Cerebellar tonsil herniation

Answer 27

Coma and death if there is compression of brain stem

Question 28

Lacunar infarcts

Answer 28

due to hypertensive changes in the arterioles Look for arteriolar sclerosis ischemic changes

Question 29

Charcot-Bouchard pseudoaneurysms

Answer 29

less than 1mm in diameter long-standing hypertension hemorrhagic changes

Question 30

Lesions found in Wernicke encephalopathy

Answer 30

Foci of hemorrhage and necrosis in mamillary bodies

Question 31

Complications of subarachnoid hemorrhage

Answer 31

secondary arterial vasospasm --> cerebral ischemia due to impaired brain autoregulation

Question 32

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

Answer 32

Friedrich ataxia gait ataxia, scoliosis, foot abnormalities, hypertrophic cardiomyopathy

Question 33

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

Answer 33

Pineal gland tumor Most commonly, germinoma

Question 34

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

Answer 34

Tabes dorsalis caused by syphillis

Question 35

Dystrophies genetics

Answer 35

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,

Question 36

Liquefactive necrosis

Answer 36

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

Question 37

Alcohol withdrawal

Answer 37

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

Question 38

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

Answer 38

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

Question 39

Global cerebral ischemia

Answer 39

Affects hippocampus first! Also Purkinje cells of cerebellum

Question 40

Complications of meningitis

Answer 40

Communicating hydrocephalus - decr. function of the arachnoid granulations

Question 41

Association with cerebral amyloid angiopathy

Answer 41

Recurrent hemorrhagic strokes