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Neuro II > Puthoff Lecture 3 - Demyelin Disease > Flashcards

Puthoff Lecture 3 - Demyelin Disease Flashcards

1
Q

What is marked by loss of sensory neurons from dorsal root ganglia, w/lymphocytic inflammation and may be found with limbic encephalitis?

A

Subacute sensory neuropathy

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2
Q

What are the symptoms of NMO?

More common in whom?

A

Synchronous bilateral optic neuritis and spinal cord demyelination

Women (a lot more)

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3
Q

What kind of tumor arises from plexiform neurofibromas?

What subtype?

A

MPNST

Triton tumor (looks like teratoma)

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4
Q

What is more common in ALS: familial or sporadic?

What kind of disease?

A

Sporadic

Motor neuron disease

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5
Q

Describe the AD morphology

A

Diffuse, bilateral cerebral cortical atrophy

Hirano bodies

Reactive gliosis

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6
Q

Cognitive function is generally spared in what disease?

A

ALS

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7
Q

MELAS disease is characterized by what?

What mutation is common?

A

Lactic acidosis
Stroke-like episodes

MTTL1

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8
Q

What are the neuroanatomic systems involved in MSA and what do they each result in?

A

Striatonigral -> parkinsomism
Olivopontocerebellar -> ataxia
ANS -> autonomic dysfunction, orthostatic HTN

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9
Q

Intravascular lymphoma is described how?

A

Infiltrating, B-Cell

Widespread microscopic infarcts due to occlusion of vessels

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10
Q

Schwannomas have loss of what protein?
What does the protein normally restrict?

What important morphology?

A

Merlin
EGFR

Carney complex

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11
Q

When is hepatic encephalopathy found?

What may be found in cerebral cortex/basal ganglia?

A

Impaired liver function

Alzheimer type II cells

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12
Q

Which glial lesions primarily affect the young?

A

Pilocytic astrocytoma
Pleomorphic astrocytoma
Brainstem glioma

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13
Q

What is the clinical pattern associated with FTLD?

A

EARLY personality changes, behavior changes
Aphasia
Dementia over time

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14
Q

What are the aggregates of Multiple System Atrophy (MSA)?

In what?

A

Cytoplasmic inclusions of alpha-synuclein

In oligodendrocytes

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15
Q

What neuronal tumor causes seizures, is common in children, and is located in the superficial temporal lobe?

Prognosis?

A

Dysembryoplastic neuroepithelial tumor

Good

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16
Q

Acute necrotizing hemorrhagic encephalomyelitis occurs in whom?

When and results in what?

A

Young adults in children

after a viral uri infection of unknown cause, mostly fatal

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17
Q

Where are medulloblastomas and ependymomas normally located?

A

Around the 4th ventricle

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18
Q

What is characterized by subacute dementia and marked by perivascular inflammatory cuffs, microglial nodules most evident in the anterior and medial portions of the temporal lobe?

A

Limbic encephalitis

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19
Q

Which neurofibromatosis is more common? Inheritance?

Morphology?

A

NF1
Autosomal dominant

Lisch nodules, cafe au lait spots

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20
Q

What disease is characteristic of the following?

Cysts, hemangioblastomas
renal cell carcinoma, Pheochromocytoma
Secondary polycythemia

A

VHL disease

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21
Q

Definite the inheritance of the following genes:

DJ-1
PINK1
Parkin
LRRK2
alpha-synuclein
A 
AR
AR
AR
AD
AD
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22
Q

What familial syndrome is characterized by medullablastoma or glioblastoma, mutation in APC?

A

Turcot syndrome

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23
Q

What grade do most MPNST tumors have?

Arise from what?

A

High-grade

NF1

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24
Q

Where do germ cell tumors most often occur?

Affect who?

Called what in the brain?

A

Pineal and suprasellar regions, 10% in Japan

Mostly males

Germinoma

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25
Progressive supranuclear palsy is characterized by what inclusion? What clinical pattern?
Tau protein Parkinsonism with abnormal eye movements
26
Degeneration of what structure is associated with PD? What else?
Substantia Nigra pars compacta (pallor) Locus ceruleus
27
What neuronal tumors present with seizures and aphasia, most common in temporal lobe and cystic? What gene mutation?
Ganglioglioma BRAF
28
What is the most severe form of Spinal Muscular Atrophy? What gene product is mutated?
Type I Werdnig-Hoffman disease, marked loss of LMN and progressive weakness, death within 2 years SMN1
29
What does hyperglycemia cause?
Dehydration, confusion, stupor, coma | Followed by cerebral edema
30
What else can be helpful to monitor acute plaque formations in MS?
Myelin basic protein assay
31
Describe the CSF in MS patients:
IgG levels increased Increased protein Oligoclonal IgG bands observed on immunoelectrophoresis
32
what is found in remnant neurons in PD? Composed of what?
Lewy bodies Alpha-synuclein
33
What is the central triad features of PD?
Tremor Rigidity Bradykinesia / Akinesia TRAP - also Postural instability
34
What neuronal tumor is rare and seen within the ventricular system?
Central neurocytoma
35
Pleomorphic Xanthoastrocytoma are usually where? What prognosis?
Temporal lobe in children and young adults (with hx of seizure) Good
36
What kind of plaques are present in MS?
Active Inactive Shadow
37
What tumor is associated with NF2?
Adults with spinal ependymomas
38
What are the major clinical features of PD?
``` Diminished facial expression Stooped posture Rigidity Slow voluntary movement Festinating gait Tremor (pill-rolling) ```
39
What tumors are asymptomatic and incidental findings and most often found in the lateral and 4th ventricles?
Subependymomas
40
What kind of degeneration does Primary lateral sclerosis have?
UMN only
41
What causes Wernicke encephalopathy? Characterized by what?
Thiamine (B1) deficiency Psychotic symptoms and Ophthalmoplegia
42
Toxic doses of what may injure neurons of layers III and V of the cerebral cortex? What else may be injured?
Carbon monoxide CA1 of hippocampus, b/l necrosis of GP
43
Central pontine Myelinolysis is most commonly associated with what? What is another name for it and describe the symptoms
Rapid correction of hyponatremia "Locked-in", pontine lesions leads to quadriplegia
44
Neuromyelitis Optica (NMO) has an antibody to what?
Anti-aquaporin 4
45
What familial syndrome is characterized by medullablastomas, mutation in TP53?
Li-Fraumeni syndrome
46
If Wernicke encephalopathy is not treated, what may it progress to? Characterized by what?
Korsakoff syndrome Short term memory loss and confabulation
47
What are possible tumors if cystic lesions in the posterior fossa are found?
Pilocytic astrocytoma | Cerebellar hemangioblastomas
48
What disease is associated with: Brain tubers, hamartomas Renal angiomyolipomas Pulmonary lymphangioleiomyomatosis Cardiac rhabdomyomas
Tuberous sclerosis complex (Autosomal dominant)
49
What is the most common mutation in oligodendrogliomas?
IDH1 and IDH2 which have a better prognosis than astrocytic tumors Deletions of ch. 1 and 19
50
What is caused by antibodies against VG calcium channels in the NMJ?
Lambert-Easton myasthenic syndrome
51
Describe the clinical features of Huntington's disease
Movement symptoms precede cognitive decline that may progress to a severe dementia
52
Acute disseminated encephalomyelitis (ADEM) occurs when? Symptoms?
1-2 weeks after an infection Headache, lethargy, coma and in 20% death
53
What familial syndrome is characterized by dysplastic gangliogliocytoma of the cerebellum with mutations in PTEN?
Cowden syndrome
54
What are most common sites of metastatic tumors to the brain?
``` Lung Breast Skin Kidney Gi tract ```
55
What tumors are most often found in children and occur in the lateral ventricles? These same tumors appear in adults in the 4th ventricle, can cause hydrocephalus
Choroid plexus papillomas
56
FTLD has what associations of protein build up?
Tau | TDP-43
57
What disease is characterized in infancy and involves seizures/hypotonia and lactic acidosis?
Leigh Syndrome
58
What kind of tumor is found in the Cauda Equina?
Myxopapillary ependymoma
59
What kind of hemangioblastomas are there in VHL?
Cerebellum | Retina
60
How many CAG repeats of Huntington lead to diseases and effect?
36 or greater
61
What do patients with Friedreich Ataxia die of? What other association?
Cardiomyopathy Diabetes
62
What is characteristic of mitochondrial Encephalomyopathies?
CNS and muscle disease Heteroplasmy Elevated tissue lactate levels
63
What markers in the CSF may diagnose a germ cell tumor?
alpha-fetoprotein | Beta-hCG
64
What MEDULLABLASTOMA has an intermediate prognosis?
Shh
65
What familial syndrome is characterized by medulloblastoma, mutation in PTCH?
Gorlin syndrome
66
MS is due to the role of what?
TH1 and TH17 cells and their secretion of IFN-Gamma
67
What is the genetic linkage involved in MS?
``` DR2 MHC (DRB1*1501) allele IL-2, IL-7 receptors ```
68
What does hypoglycemia cause?
Injury to large pyramidal neurons of the cerebral cortex, may affect CA1 section
69
What morphologic finding is common in meningiomas? What extra types are there?
Psammoma bodies Secretory and microcystic
70
Schwannomas have immunoreactivity for what? What kind of special morphology is seen with them?
S-100 Antoni A and B areas Verocay bodies
71
What is the protein associated with Ataxia Telangiectasia? What clinical features?
ATM protein Recurrent sinopulmonary infections Also dysarthria, and development of T-cell leukemias
72
Toxic doses of what cause blindness and preferentially affects the retina?
Methanol
73
What are some progressive clinical manifestations of PD?
Autonomic dysfunction cognitive function impairment Dementia
74
Atypical Teratoid/Rhabdoid tumors are located where? What grade? Affect mostly whom? What gene mutation?
Posterior fossa/supratentorial compartment Grade IV (highly aggressive) Children hSNF5/INI1
75
What kind of degeneration does progressive muscular atrophy have?
LMN degeneration only
76
Primary CNS lymphoma arises where? In what patients? What else about it?
Arose in the brain, in immunocompromised (HIV Pts) Multifocal, EBV
77
What kind of tumor comes down from the superior aspect of the 4th ventricle?
Medulloblastoma
78
How is MERRF transmitted? Characterized by what?
Maternally Myoclonic epilepsy Ragged red fibers
79
What is the protein of Friedreich Ataxia? What is the trinucleotide repeat? Inheritance?
Frataxin GAA Autosomal recessive
80
What kind of tumor is most often seen in young adults and is attached to the roof of the 3rd ventricle where it causes non-communicating hydrocephalus?
Colloid cyst of the 3rd ventricle
81
What diseases are mono phasic? Polyphasic?
ADEM and ANHE M.S.
82
Pineal Parenchymal tumors have what kind of differentiation? What grades are they?
Neuronal High grade in children Low grade in adults
83
What disease has an association with Down Syndrome?
AD (early onset)
84
What are the aggregates of CBD? Clinical features?
Tau protein Parkinsonism with asymmetric movement disorder
85
Acute demyelination due to MS is usually described as what?
Periventricular
86
What kind of degeneration does progressive bulbar palsy have? What important clinical features?
LMN (bulbar region) Abnormalities of deglutition and phonation Atrophy and fasciculations of the tongue and dysphagia
87
What is seen in oligodendrogliomas?
Coarse calcifications, even on plain films
88
What kind of inheritance is Huntington's disease? What is the major characteristic of it morphologically?
Autosomal Dominant Loss of caudate nucleus
89
What is the prognosis of an astrocytoma (glioma) with a mutant form of IDH1 compared to a wild-type form?
Better prognosis
90
what is NF2 also associated with?
Schwannomas, meningiomas, ependymomas
91
What is the critical initiating event for the development of AD?
AmyloidBeta generation
92
What are the major clinical signs with MS?
Optic neuritis Ataxia, nystagmus, INO Motor and sensory impairment, spasticity, bladder control lost
93
What other disorders can cause thiamine deficiency?
Carcinoma Chronic gastritis Persistent vomiting
94
Describe the clinical course of AD
Progressive disorientation, memory loss, loss of learned motor skills, aphasia Cortical dysfunction
95
Which MEDULLABLASTOMA has the best prognosis? Worst?
WNT Group 3 (i17q and MYC amplification)
96
B12 deficiency causes what? Characterized by what?
Subacute combined denervation of spinal cord B/l symmetrical numbness, tingling, slight ataxia and spastic weakness of the lower extremities

Neuro II (5 decks)

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