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Neurobiology > Green Neuro Upload > Flashcards

Green Neuro Upload Flashcards

1
Q

what types of glutamate receptors are there

A

ionotropic (AMPA, NMDA, kainate) and metabotropic (quisqualate)

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2
Q

what types of GABA receptors are there

A

GABA A and GABA B: Gaba A is post synaptic and a Cl- channel; GABA B is pre-synaptic and is a K+ channel

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3
Q

where do benzos and barbiturates act

A

on GABA receptors

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4
Q

what are the cellular mechanisms of starting seizures?

A

too much exciting (inward Na and Ca channels); NT (glutamate, aspartate)

not enough inhibition (ionic 2/2 inward Cl- or outward K) or NT (GABA)

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5
Q

How are hyperexcitable networks formed? (i.e. seizure-generating networks)

A

excitatory neurons can have axonal sprouting (growth); there can be loss of inhibitory neurons; there can be loss of excitatory neurons that activate inhibitory neurons (loss of regulation)

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6
Q

how do we define epilepsy (ILAE definition)

A
  1. at least two unprovoked seizures > 24 hours apart OR one unprovoked seizure and 60% probability of seizure occuring in next 10 years OR diagnosis of epilepsy syndrome
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7
Q

what kinds of seizures may not need treatment long term?

A

generally, those with provoking cause- i.e. single seizure, febrile seizure, benign syndrome of childhood, simple partial seizure, impact seizure (TBI), provoked seizure

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8
Q

most common etiology of epilepsy?

A

unknown

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9
Q

common causes of seizures in children

A

febrile, congenitla, metabolic

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10
Q

common causes seizures in young adults

A

trauma and tumor

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11
Q

common causes seizure in elderly

A

stroke and degenerative changes

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12
Q

Absence (Generalized non motor) seizure features

A

school aged children; abrupt onset activity arrest and staring; brief, lasting 3-20 seconds bu return to normal abruptly. can happen many times throughout day.

EEG shows 3Hz spike wave, MRI usually will be normal. These tend to resolve by adolescence

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13
Q

General Motor (Myoclonic) Seizures

A

brief, shock like jerks of group of muscles;

these tend to be bilaterally synchronous, lasting < 1 second (hard to assess consciousness as a result).

repeat seizures can have impaired consciousness.

  • these seizures are seen with other epilepsy syndromes and with progressive myoclonic epilepsy.

EEG will show 4-6 Hz polyspike wave

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14
Q

Generalized Motor- Tonic seizures

A

symmetric tonic muscle contraction- extremities and flexion of waist and neck; these will last 2-20 seconds (last longer than myoclonic).

on EEG- shows sudden attenuation with generalized, low voltage fast activity i.e. drop in EEG activity (common) or generalized polyspike wave.

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15
Q

Atonic (generalized onset) motor seizures

A

sudden loss postural tone–> two forms (severe- falls; mild head nods, jaw drops)

consciousness is typically impaired during this, lasting only seconds

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16
Q

general drug warnings with anti seizure meds

A

all anti seizure drugs have potential teratogenicity; all have high risk of suicide. Other genral risks incude eed to monitor blood and liver function (i.e. looking for anemia, increased LFTs). Can have cost issues and compliance issues

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17
Q

“old” anti seizure drugs include

A

phenobarbital, phenytoin, carbamazapine, and valproate; these generaly have higher side effects and require frug level monitoring

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18
Q

adverse effects seen with “old” anti seizure meds (phenobarbital, phenytoin, carbamazapine, and valproate) includes

A

bone marrow suppression, hepatic inflammation, rash, and SJS

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19
Q

what tests should be run to monitor if on an old anti seizure med

A

CBC (monitor for bone marrow suppression) , LFTs (Hepatic inflammation)

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20
Q

phenytoin MOA

A

blocks VG Na channels; aka Dilantin.

hepatic enzyme inducer

elderly individuals can’t tolerate as high a dose as adults

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21
Q

phenytoin side effects

A

gingical hyperplasia, hirsuitism, “coarsening” of featueres;

can have toxicity including cerebellar sx (ataxia, falls), cardiac arrythmia

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22
Q

Carbamazepine MOA

A

blocks VG Na channels; aka Tegretol

hepatic inducer

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23
Q

side effects of carbamazepine

A

can cause SIADH and hypOnatremia

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24
Q

toxic side effects of carbamazepine include

A

double vision and mental clouding

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25
Q

phenobarbital MOA

A

GABA-A inhibition; prolongs the duration of open Cl- channel (more inhibition)

good b/c inexpensive

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26
Q

phenobarbital side effects

A

sedation, respiratory depression, withdrawal sx

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27
Q

valproate MOA

A

multiple- Na channels, T type calcium channels, GABA

hepatic inducer

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28
Q

valproate SE

A

pancreatitis, thrombocytopenia, hyperammonemia

nausea, weight gain, hair loss;

toxic effects include tremor

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29
Q

valproate is CI in those with

A

urea cycle c/o, liver disease, mitochondrial disorders

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30
Q

which old anti seizure med is associated with NTD

A

valproate

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31
Q

anti seizure med used for absence seizure

A

ethosuximide

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32
Q

ethosuximide MOA

A

blocks T type calcium channels

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33
Q

which anti seizure med has risk of weight loss, kidney stones, and glaucoma (acute angle)

A

topiramate

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34
Q

which new anti seizure drug carries risk of rash and SJS

A

lamotrigine (lamictal); Na channel blocker that is hepatically metabolized

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35
Q

Keppra MOA

A

binds to SV2A (synaptic vesicle protein); decreases NT release

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36
Q

parampanel MOA

A

new anti seizure med - AMPA antagonist

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37
Q

Vigabatrin MOA

A

irreversible GABA transaminase inhibitor

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38
Q

Tiagabine MOA

A

new anti seizure med; binds to GABA; prevents re-uptake

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39
Q

drugs that can be used for focal onset seizures

A

anything except ethosuximide (this is only used for absence seizures)

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40
Q

drugs for generalized onset convulsive seizures

A

VLLTZ

valproate, lamotrigine, topriamate, levetriacetam, zonisamide

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41
Q

drugs for absence (generalized non motor) seizures

A

ethosuximide and valproate;

can use other drugs but less effective

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42
Q

when should anti seizure drugs be started?

A

after second unprovoked seizure, most would start; if only one seizure, consider risk for future seizures and can use MRI/ EEG

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43
Q

what can increase your risk of recurrent seizures

A

previous neuro insult, abnormal EEG, prior seizures, post-ictal paralysis, partial onset paralysis

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44
Q

first line anti seizure med for generalized onset motor seizure

A

valproic acid, lamotrigine, and topiramate

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45
Q

first line med for focal onset seizures

A

carbamazepine, oxycarbamazepine, phenytoin, lamotrigine, valproic

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46
Q

first line treatment for generalized nonmotor (absence) seizures

A

valproic acid, ethosuximide

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47
Q

treatment for status epilepticus

A

Ativan (lorazepam) IV; among other treatments (ABCs, thiamine, dextrose, IV anti seizure drug)

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48
Q

what is general mechanism for AMS

A

poorly understood, but thought to originate from changes to the reticular activating system or global cortical dysfx

in general, AcH plays a key role

the RF is a network of brainstem nerve fibers that extend to diencephalon and connects to centers of hypothalamus; serves to filter incoming informatio, also serves to arouse cerebral cortex into wakefulness

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49
Q

what kind of patient would have hypoglycemia encephalopathy?

A

MRI will show changes similar to hypoxic/ ischemia (similar mechanism); most commonly will be found down and have been hypoglycemic for a while. DWI MRI is the test of choice

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50
Q

hyperglycemia associated seizure

A

epilepsia partialis continua, which is a form of focal motor seizure .

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51
Q

central pontine myelinolysis symptoms

A

AMS, quadriparesis, dysphagia

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52
Q

extrapontine myelinosis sx

A

behavioral disturbances, movement disorders, seizures

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53
Q

triphasic waves on EEG seen with

A

hepatic encephalopathy (seen with metabolic enceph but especially hepatic)

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54
Q

most common causes of hypercalcemia mediated AMS

A

primary hyperparathyroidism and cancer

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55
Q

who is at risk for B12 deficiency

A

pernicious anemia (IF binds B12); malabsorption; vegans

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56
Q

who is at risk for B1 (thiamine) deficiency

A

EtOH use d/o, bariatric surgery, anorexia

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57
Q

stages of B1 deficiency

A

Acute (Wernicke): encephalopathy presents with ataxia, oculomotor dysfunction (nystagmus, lateral rectus palsy, opthalmoplegia)

Chronic (Korsakoff): memory loss and confabulation

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58
Q

mammilary body dysfunction a/w what deficiency

A

B1 (thiamine)

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59
Q

vitamin E deficiency symptoms

A

leads to a slow progessing spinocerebellar syndrome; cerebellar symptoms include ataxia of trunk and limb and myelopathic features include upgoing toes, loss of vibration and proprioception

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60
Q

what kind of CNS damage do hydrocarbons and inhalants cause

A

white matter damage (toxic leukencephalopathy)

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61
Q

what region of brain classically injured in carbon monoxide poisoning

A

bilateral globus pallidus

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62
Q

toxic leukencephalopathy

A

white matter damage that is classically seen with toxin mediated damage to brain (i.e. hydrocarbons)

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63
Q

acute vs. chronic carbon monoxide poisnong

A

acute- HA, nausea, dizziness, malaise

chronic: cognitive changes, personality changes

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64
Q

hallmarks of CNS inflammation that occurs in MS (4 factors)

A

peripheral immune cells infiltrate, CNS immune cells are activated, BBB disrupted, IgG synthesis (intrathecal)

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65
Q

what kinds of peripheral immune cells infiltrate CNS in MS (perivascular, parenchymal, and meningeal)

A

perivascular = T and B cells, monocytes, macrophages

parenchymal: CD8 > CD4 T cells
meningeal: B cells, plasma, follicular cells

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66
Q

where do peripheral immune cells enter the CNS?

A

post capillary venules

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67
Q

what kinds of CNS immune cells are activated in MS

A

microglia are activated; can be shown in PET scan

astrocytes also become reactive

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68
Q

what does gadolinium infiltration on MRI indicate

A

BBB dysruption; seen in patients with MS over areas of ACTIVE lesions

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69
Q

what are the effects of BBB dysruption in MS

A

fibrin deposition, Ig deposition, complement activation

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70
Q

what role do fibrinogen/ fibrin entry into brain play in MS?

A

BBB dysruption leads to entry of these substances –> this can activate microlgia (CNS immune cell activation)

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71
Q

what is meant by dissemination in MS

A

pattern of active (gadolinium entry) and inactive lesions on MRI; indicates inflammatory activity of different acuity. characteristic of MS

dissemination is the basis of relapsing clinical course of MS

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72
Q

oligocloncal bands

A

IgG bands that indicate clonal proliferation of IgG by B cells in MS
(intrathecal synthesis of IgG)

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73
Q

adaptive immune system in MS

A

B cells produce oligoclonal IgG (intrathecal production) in a type of adaptive immunity that can then be used diagnostically

this is part of the CNS mediated immune response in MS (in addition to the peripheral immune cell response that occurs)

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74
Q

where do demyelinating lesions tend to occur in MS

A

periventricular lesions

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75
Q

what eye condition is closely associated with MS

A

optic neuritic (demyelinating lesions of CN II)

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76
Q

how do myelin and axonal damage in MS differ

A

myelin can be reformed (albeit incomplete) whereas axon damage is irreversible

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77
Q

optic neuritis and MS

A

typical clinical presentation = painful blurred vision

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78
Q

myelitis and MS

A

bilateral weakness and/ or numbness with bladder/bowel dysfunction

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79
Q

cerebellitis and MS

A

ataxia

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80
Q

brainstem syndrome

A

seen in MS; presents with double vision, vertigo, dysphagia, dysarthria, incoordination, numbness or weakness

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81
Q

hemispheric syndrome

A

hemiparesis, hemianesthesia, visual field cuts, word finding difficulty; clinically isolated syndrome seen with MS

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82
Q

periventricular lesions hint at

A

MS (prefers to localize to post capillary venules) where BBB is dysrupted

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83
Q

Natalizumab use and MOA

A

used to treat CNS indlammation in MS; MOA= blocks immune cell entry into CNS by binding to and blocking alpha4beta1 integrin, which mediates entry

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84
Q

Fingolimod

A

sphingosine 1 phosphate receptor modulator; used to treat MS

blocks leukocyte exit from lymph nodes

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85
Q

Ocrelizumab

A

CD20 Antibody used to treat MS; targets and depletes B cells and reduces CNS inflammation

B cells are part of peripheral inflammatory cells that enter CNS from perivascular and meningeal area

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86
Q

NMOSD

A

neuromyelitis optica spectrum disorder; an autoimmune demyelinating d/o that likes to target spinal cord and optic nerve, but generally spares brain. Results from anti aquaporin 4 aka anti NMO IgG

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87
Q

what are the core clinical syndromes a/w NMOSD

A

optic neuritis (similar to MS); myelitis (like MS); area postrema syndrome (NOT like MS), acute brainstem syndrome (like MS); acute diencephalic syndrome, symptomatic cerebral syndrome

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88
Q

what is area postrema syndrome

A

clinical syndrome that is strongly associated with NMOSD;

presents with intractable nausea, vomiting, and hiccups

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89
Q

how is NMOSD characteristically sen on imaging

A

MRI showing longitudinally extensive transverse myelitis (contiguous spinal cord lesion that extends > 3 vertebral segments)

MRI may also show lesions to the area postrema in the medulla; if there are lesions in hypothalamus may have autonomic dysfunction as well

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90
Q

how is NMOSD diagnosed

A

if anti AQP4 (NMO) IgG positive,must have also 1 or more core clinical syndrome (i.e. myelitis, area postrema syndrome, optic neuritis, etc) and other dx excluded

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91
Q

where are the aquaporins that area affected in NMOSD

A

on astrocyte end feet (part of BBB)

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92
Q

treatment options for NMOSD

A

Eculizumab (Ab vs. C5 antibody)- blocks complement activation in NMOSD; prevents clinical relapses

Inebilizumab (Ab vs. CD19)

Satralizumaba (Ab vs. IL-6)

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93
Q

What is the ASIA Impairment Scale (AIS)

A

used to relay degree of sensory or motor function based on spinal cord injury

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94
Q

AIS A

A

highest level of impairment; leads to no motor/ sensory function below the level of injury

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95
Q

AIS B

A

no motor function below level of injury (sensation intact)

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96
Q

AIS C

A

> 50% of motor function below neuro level of ( less than 3/5 strength)

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97
Q

AIS D

A

more than 50% motor function below neuro level of injury (> 3/5 strength)

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98
Q

AIS E

A

full neuro recovery

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99
Q

What is the ambulation potential of people with AIS B

A

50%; better prognosis with sharp/ dull sensation

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100
Q

C3-C5 spinal cord injury

A

innervates diaphrag; impacts breathing without ventilatory support

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101
Q

C5 level spinal cord injury

A

C5- innervates elbow flexors/ biceps

self feeding with adaptive equipment/ setup
driving with chair independent

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102
Q

C7 level SCI abilities

A

elbow extensors (triceps);

this is a key muscle for independent activity- overhead reach, transfer ability, driving with basic hand controls

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103
Q

which UE muscle use/ spinal cord level is very important towards independent activity

A

C7- triceps (extensor);

this allows for more mobility and improves the ability of living independently with appropriate adaptive equipment

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104
Q

C8 level SCI

A

finger flexors- allows for gripping objects and turning knobs.

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105
Q

T1 level SCI

A

finger extensors- allows for full hand function

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106
Q

what role does muscular innervation level play in thoracic level SCI

A

plays a role in sitting balance and in cough (abdominal innervation required for)

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107
Q

T6 level SCI

A

an injury above this level increases risk of autonomic dysreflexia

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108
Q

what is autonomic dysreflexia- what can cause it

A

most commonly it is caused by bladder distension/ constipation/ etc in the context of someone with a SCI above the level of T6.

  • signs include increased BP, bradycardia, flushing, sweating and goosebumps ABOVE LEVEL OF INJURY
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109
Q

how do you trea autonomic dysreflexia

A

control stimuli –> i.e. fix bladder distension/ constipation; can use medication temporarily as needed

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110
Q

what are some of the common complications to SCI?

A

bladder issues; such as detrusor/ sphincter dysynergia (i.e. bladder and the outlet sphincter don’t work in unison); risk for UTI, hydro, autonomic dysreflexia, UTI, stones

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111
Q

what kinds of force are responsible for TBI?

A

rapid acceleration/ deceleration 2/2 shear stress (SLOW COMPRESSION NOT AS IMPLICATED)

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112
Q

types of TBIs include

A

focal and generalized

focal is usually more related to acceleration/ deceleraton type injuries that lead to direct contact of brain and skull. i.e. SDH, epidural hematoma

generalized more related to shear/ tensile strength from acceleration/ deceleration injuries. includes diffuse axonal injury

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113
Q

GCS 13-15 =

A

mild TBI (includes concussion)

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114
Q

GCS 9-12=

A

moderate TBI

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115
Q

GCS of 8 or less =

A

severe TBI (threshold for intubation)

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116
Q

what regions of brain are most disposed to cortical contusions in TBI

A

anatioer inferior frontal lobes and temporal lobes

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117
Q

what are some biomarkers that can be used to demonstrate TBI

A

IL-1 beta, TNF alpha, Il-6, GFAP, UCHL-1

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118
Q

epidural hematoma

A

bleeding between dura mater skull

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119
Q

subdural hematoma

A

bleeding between arachnoid mater and dura mater

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120
Q

neuroexcitation in acute and chronic post TBI

A
  • increased calcium entry
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121
Q

how does glycolysis change after TBI

A

there is early acute hyperglycolysis and later on develop hypoglycolysis

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122
Q

what is diffuse axonal injury

A

typically has little to no gross pathologic finaing but will see scattered damage to cerebral/ brainstem white matter –> especially corpus callosum

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123
Q

what imaging modality is most useful for detecting diffuse axonal injury

A

diffusion tensor imaging (DTI)

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124
Q

what factors influence the adverse complications of repeat TBI

A

how severe the concussion/ TBI is; how long between repeat events; how old you are when injury happens; what premorbid conditons you have

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125
Q

Intraparenchymal vs. extraparenchymal manifestations of neurocysticercosis

A

intraparenchymal symptoms = seizures, but can also be incidental finding. is MCC seizures in some endemic countries

extraparenchymal symptoms.= hydrocephalus; increased ICP with HA, AMS, n/v, death

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126
Q

what are the systemic symptoms of Lyme disease

A

affects skin (rash), joints (artharalgias), and nervous system changes

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127
Q

neurologic sx of Lyme disease

A

classic finding is bilateral Bell’s palsy (CN VII palsy);

can also cause radiculitis (mimics radiculopathy without any mechanically compressive symptoms)

meningtis- similar to viral meningitis (CSF and clinical profile)

“chronic” lyme disease canlead to pain, fatigue, and minor cognitive issues

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128
Q

what are the risk factors for PML

A

immunocompromise, treatment with Natalizumab (alpha4beta1 integrase blocker), infection with JC virus, hematologic malignancy,

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129
Q

radiographic findings a/w PML

A

unilateral or bilateral demyelinating foci (discrete foci); these do not cause mass effect and do not enhance contrast

tend to be located at the grey/ white junction in parieto-occipital and frontal lobes

vs. MS- small multifocal lesions that are ovoid and perpendicular to corpus callosum

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130
Q

toxoplasmosis infection routes

A

contaminated water/ soil from environment; can also get from eating meat that has been contaminated. latent infection from intracellular parasite toxoplasma gondii

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131
Q

toxoplasmosis MRI findings

A

toxo is the most common CNS infection in untreated AIDS –> leads to ringed lesions on MRI

clinically, presents with HA, confsuion, fever when reactivated infection. can also have focal neuro sx depending on location of lesion

132
Q

what are unique features of cryptococcal meningitis

A

increased ICP (can be as high as 40, whereas normal = 10-20); can also see CN VI palsy (a/w increased ICP, not specific to crytpo)

133
Q

who is at risk for cryptococcal meningitis

A

those with AIDS and CD4 < 100; if do not have HIV, may have chronic immunosuppressant use or hematologic malignancy

134
Q

what is the mech of prion disease

A

exact MOA unknown, but originates from protein malformation info beta sheets (unlike typical alpha helices)

altered proteins convert normal prion proteins –> abnormal forms –> aggregate and can’t be degraded.

135
Q

describe the life cycle of tenia solium

A

parasitic infection that is endemic in areas including Mexico; lives in pork. pork eaten –> tapeworm in people; tapeworm makes eggs –> people poop out eggs –> eggs get eaten by others. larvae hatch and enter blood stream –> larvae enter other systems including brain (intra/extraparenchymal), eyes, and subcutaneous

136
Q

how long does it take for infection –> symptom onset in cysticervosis life cycle (tenia solilum)

A

3.5 years = average incubation time

137
Q

diagnosis and treatment for PML (progressive multifocal leukoencephalopathy)

A

dx= LP with JC virus serology

treatment: start ARD (if AIDS +), stop immunosuppression, stop natalizumab

poor prognosis- death within months

138
Q

what condition can give you chronic meningitis?

A

crypto- fever, malaise and headache over several weeks/ months, associated with increased ICP (unique to crypto)

may also have vomiting and CN VI palsy (diplopia) 2/2 this

139
Q

how do you evaluate creutzfeld jacob disease

A

presents as a rapidly progressive dementia; death within 1 year

140
Q

symptoms of creutzfeld jacob disease

A

rapidly progressive dementia

90% have myoclonus (especially with startle)

can also present with cerebellar ataxia, UMN signs, Parkinsonism

141
Q

How do you diagnose CJD

A

MRI is the best, most important test for this

can also use EEG (periodic sharp wave complexes) and CSF biomarkers (14-3-3 protein)

142
Q

what does CJD + MRI show

A

hyperintense signal of cortex, caudate, and putamen on DWI sequence

(recall that hyperintensity also seen in stroke pathology)

143
Q

causes of CJD

A

most cases are sporadic (85-95%); other cases can be caused by genetic mutations, mad cow disease, or iatrogenic (transplant, cadaveric grafts, HGH from cadavers, cadaver corneal transplant)

144
Q

what structures sit medial to the putamen?

A

globus pallidus (internal and external segments);

145
Q

structures involved in basal ganglia

A

corpus striatum, which includes the putamen and caudate nucleus; globus pallidus, and thalamus with subthalamic nuclei

146
Q

what afferent input is sent to the caudate?

A

input from head and eye movement

147
Q

what afferent input sent to the putamen?

A

limbs/ trunk movement

148
Q

where is dopamine produced?

A

substantia nigra pars compacta (not the pars reticulata)

149
Q

where is the superior colliculus and what function does it play?

A

in the midbrain, plays role in gaze

150
Q

what are medium spiny neurons? where are they found,

A

these are found in corpus striatum; these are inhibitory neurons that are quiet at baseline. They receive input (glutamate) and send out inhibitory signals (GABA)

151
Q

what is the function of the indirect pathway in basal ganglia? what structures are involved?

A

goal is to have multiple targets in motor/ pre motor cortex that are nearby direct pathway targets. Indirect pathway seeks to reinforce inhibition of neighboring cortex to create smooth movement. This is done by targeting MSN nearby. this will disinhibit the subthalamic nuclei, which then release glutamate to activate neighboring internal MSN –> MORE inhibitory tone blocking motor activation

152
Q

D1 receptors

A

MSNs that target the GP internal express more of these. when there is more dopamine released, these D1 receptors activate more easily –> fire faster (faster motion)

153
Q

D2 receptors

A

MSNs targeting the GP external express more of these; these are inhibitory in nature, when there’s more dopamine released there will be less activation of MSNs –> these

154
Q

what causes Huntington’s disease

A

degeneration of the striatal neurons (MSNs expressing D2) to the globus pallidus external (indirect pathway loss); can result genetically from mutation in the HTT gene, with CAG repeats

155
Q

vestibulocerebellum role

A

uses vestibular and visual input to regulate posture and balance as well we vestibulo-ocular reflex (i.e. maintaining fixed gaze)

156
Q

Spinocerebellum function

A

receives spinal input and evaluates this input as well as desired motion. Main function is to predict and refine movement;

  • this provides fast and precise feed forward execution of movement
157
Q

Cerebrocerebellum function

A

input from cortical cognitive and motor centers;

refines complex behavioral responses via feedback. In particular, integrades complex spatial and temporal sequencing info. This is expanded in humans (compared to other vertebrates)

158
Q

superior peduncle contents

A

white matter pathway that mostly contains efferent

159
Q

middle peduncle contents

A

mostly afferent (from cortex)

160
Q

inferior peduncle function

A

mostly afferent fibers

161
Q

in addition to peduncle white matter tracts entering cerebellum, what other tracts enter?

A

mossy fibers (pontine) and climbing fibers from inferior olivary nucleus

162
Q

where do mossy fibers enter the cerebellum

A

pontine enter via middle peduncle

proprioceptive information from spinocerebellar tract enter via inferior peduncle (along with climbing fibers)

163
Q

where do climbing fibers enter the cerebellum

A

via inferior cerebellar peduncle

164
Q

what kinds of cells are critical for calculating function in the cerebellum?

A

purkingje fibers; these receive input from both climbing fibers (inferior olivary nucleus, via inferior peduncle) and from mossy fibers (pontine enters via middle peduncle and spinocerebellum via inferior peduncle);

165
Q

what information do mossy fibers carry?

A

generally, carry information about proprioception.

166
Q

what information do climbing fibers carry?

A

carry information that determines if the body needs to make a correction (in position, etc); this is integrated with signal from the mossy fibers, which relay proprioceptive information

167
Q

cerebellar lesions involving lower leg could result from damage to which vessels?

A

the superior cerebellar or anterior inferior cerebellar artery

168
Q

posterior inferior cerebellar lesion can affect what part of body

A

head

169
Q

superior cerebellum fed mainly by which vessel

A

SCA (superior cerebellar)

170
Q

where does AICA feed on cerebellum

A

inferior middle portion

171
Q

where does PICA feed

A

inferior portion of cerebellum, from midline to lateral

172
Q

how could PICA stroke effect cerebellar function? which parts would be involved?

A

could lead to damage to cerebrocerebellu, thereby affecting this tracts’ ability to aid in learning and fine tuning movements. This would affect contralateral side if affected

173
Q

how do lesions to spinocerebellum and cerebrocerebellum differentially affect body function?

A

spinocerebellar lesions have ipsilateral effects to lesion

cerebrocerebellum has contralateral (crosses midline between the cerebellar cortex to VL complex in thalamus)

174
Q

what cerebellar structures are involved in the learning process that improves coordination?

A

cerebellar cortex, deep cerebellar nuclei (dentate/ interposed nuclei), red nuclei, inferior olive, climbing tracts

175
Q

fastigial nucleus of cerebellum involved in which portion of cerebellar function (movement)

A

spinocerebellar functionn- i.e. feed forward, predicting and refining movement.

176
Q

where is the dentate nucleus located

A

lateral most nucleus on the cerebellum (would not be likely affected by central lesion)

177
Q

lesion to vestibulocerebellum would cause what?

A

balance issues, postural issues, issues with vestibulooclear reflex

178
Q

where do fibers from spinocerebellar deep cerebellar nuclei (fastigial nuclei) project to? what structures do they pass through?

A

pass through the inferior cerebellar nuclei to the superior and inferior colliculi

179
Q

what causes intention tremors? what do they look like?

A

kinetic tremors that happen with goal oriented movemnet (i.e. pointing). These tremors are linked to spinocerebellar ysfunction. dyscuntion is related to spinocerebellar dysfunction (the cerebellum does not properly predict the extremity’s position in relation to goal)–> dysmetria

180
Q

which part of cerebellum, if lesioned, could lead to dysarthria?

A

lateral cerebrocerebellum; the cerebellum plays a role in action sequencing, including speech. Speech would become chippy and deconstructed to syllabus, not fluent.

181
Q

movement deficits associated with cerebellar lesion

A

ataxia (truncal, nystagmus, limb), dysmetria, intention tremor, dysarthria

182
Q

vermis of spinocerebellum function role in movement and lesion effect

A

coordination of trunk movement; lesion would cause truncal ataxia

183
Q

paravermis of spinocerebellum role in movement and lesion effect

A

limb movement coordination; limb ataxia

184
Q

which part of cerebellum is involved in planning and sequencing of movement and speech?

A

cerebrocerebellum

185
Q

which part of cerebellum involved in motor learning?

A

cerebrocerebellum

186
Q

gait ataxia and balance dysfunction can result from lesions to

A

spinocerebellum and vestibulocerebellum

187
Q

left limb dysmetric and tremor would result from lesions to which cerebellar structure?

A

cerebellar left hemisphere

188
Q

loss of MSN in caudate that projct to the external globus pallidus seen in what disease

A

Huntington’s disease (leads to weakening of indirect pathway); loss of diffuse inhibition

189
Q

symptoms seen with Huntington’s (from early to later stages)

A

involuntary mvmts, depression/ mood changes, clumsy, sexual dysfunction, trouble walking, delusions, speech difficulty

190
Q

degernation of dopaminergic neurons in substantia nigra pars compacta

A

Parkinson’s disease

191
Q

treatment for Huntington’s includes

A

dopamine antagonist (haloperidol)

192
Q

what causes bradykinesia in Parkinsons?

A

over inhibited direct pathway, overactive indirect pathway limits movement and –> difficulty initiating/ slow movement. shuffled gait results from this, difficulty turning

193
Q

what causes rigidity in parkinson’s

A

over sensitive indirect pathway –> stiff muscles that are resistant to stretch, contribues to postural deformities

194
Q

what causes resting tremors in Parkinson’s disease?

A

synchrony of low frequency brain activity due to indirect pathway overactivity ; note that resting tremor often unilateral!

195
Q

Hemiballism

A

hyperkinetic disorder resulting from lesion (stroke) to subthalamic nucleus; weakens the indirect pathway and leads to contralateral contractions of proximal limb muscles

196
Q

Hypokinetic disorders

A

Parkinson’s disease, Parkinson’s plus disorders, Secondary Parkinsonism

197
Q

Hyperkinetic disorders

A

tremor, dystonia, tics, chorea

198
Q

what factors should you look at when evaluating tremor?

A

location of tremor, what makes tremor better/ worse, frequency of tremor, amplitude of tremor

199
Q

low/ medium/ high frequency tremor

A 
4 = low
4-7= high
>7 = high
200
Q

what is a resting tremor

A

tremor in a body part that is not being actively used; this must be totally supported against gravity! These tremors will increase with activation (mental i.e. math or motor i.e. moving different body part) and improve with voluntary action

201
Q

what is an action tremor?

A

these are any tremor that occur with voluntary contraction of a muscle; several types including
postural, kinetic (simple vs. intention), task specific, and isometric

202
Q

most common movement disorder

A

essential tremor

203
Q

when do most essential tremors start? genetic linkage?

A

can occur at any age but mean onset betwen 35-45 y/o. In 60% of people, there is +FHx, other 40% have no family history

204
Q

what kinds of tremors are involved in essential tremors?

A

typically ehse are posture and action tremors that progress over time. Sometimes can see resting tremors with this.

205
Q

what locations most commonly affected with essential tremor?

A

bilateral hands> head > voice ; then leg, jaw, trunk and face less commonly affected.

206
Q

first line treatment for essential tremor

A

beta blockers (propranolol is best); Primidone (anti seizure med)

207
Q

propranolol side effects

A

first line treatment for essential tremor; generally well tolerated but can cause ED in men, depression, fatigue

208
Q

primidone side effects

A

first line tx for essential tremor; SE can include dizziness and sedation (typically taken at night)

209
Q

Second line essential tremor treatments (commonly used ones)

A

gabapentin and topiramate (others include alprazolam, atenolo, sotalol)

210
Q

Surgical options for treating essential tremor

A

ablation and deep brain stimulator (typically into nucleus of thalamus)

211
Q

average age onset PD

A

62; higher prevalence in men (estrogen = protective?)

212
Q

early features of Parkinson’s disease include

A

resting tremor, muscle rigidity/ cogwheeling, bradykinesia/ akinesia

213
Q

late features of parkinson’s disease

A

gait disorder, postural change (leaning forward), postural instability, freezing

214
Q

diagnostic criteria for Parkinson’s disease

A

bradykinesia with at least one of the following: rigidity, 4-6 Hz (medium frequency) resting tremor, postural instability

215
Q

alpha synuclein causes of parkinsonism

A

PD and multiple system atrophy

216
Q

tau proteinopathies that can cause parkinsonism

A

progressive supranuclear palsy

corticobasal degeneration

217
Q

alpha synuclein and Lewy body + is seen wtih

A

Parkinson’s disease

218
Q

MSA-P

A

multiple system atrophy-P; this is glial cytoplasm inclusion of alpha synuclein. type of neurodegenerative d/o

rapidly progressive Parkinsonism that doesn’t respond to L-dopa.

219
Q

MSA-P clinical presentation

A

rapidly progressive postural instability and or dysphagia within few years; can present wiht gait/ limb ataxia, autonomic dysfunction (rare in PD)

220
Q

putamen discolaration can be seen in

A

MSA-P

221
Q

glial alpha synuclein seen in

A

MSA-P (multiple system atrophy- Parkinsonism)

222
Q

progressive supranuclear palsy aka

A

Steel Richardson Olszewski syndrome

223
Q

progressive supranuclear palsy clinical presentation

A

Parkinsonism with extrapyramidal sx; can present with supranuclear opthalmoplegia (vertical gaze palsy). can have cognitive problems including dementia, nuchal/ truncal dystonia

224
Q

PSP sex and age average

A

2:1 male to female; avg age 50-70

225
Q

what regions of the brain are affected in PSP?

A

substantia nigra, CN X and XII, globus pallidus, hippocampus, dentate nucleus of cerebellum, locus ceruleus, subthalamic nucleus, CN II, IV nuclei, red nucleis, colliculus, periaqueductal cord grey matter

226
Q

phases of corticobasa degeneration (CBD)

A

phase 1- asymmetric clumsiness
phase 2- Parkinsonism (akinetic, rigid), “alien hand”, sensory cortical dysfunctions,
phase 3: cognitive decline

227
Q

what is difference between motor unit and motor neuron pool

A

motor unit is made of single motor neuron and all of the muscle fibers that it innervates. Size of motor unit proportional to size of motor neuron (large = innervate lots of muscle fibers, generate lots of force. Small motor unit = innervates fewer fibers, does finer movement i.e. eye movement)

motor neuron pool = a group of motor neurons that innervate fibers in the same muscle. These can come from multiple spinal cord levels.

228
Q

what are the three types of motor units, how do they differ

A

three types- slow, fast fatiguable, and fast fatigue resistant

slow = slowly contracting muscle fibers that generate low force but don’t get tired easily

fast fatigue resistant= contract with intermediate speed. generate intermediate force but will lost some of max force with repeat stimulation

fast fatiguable= contract rapidly and generate max force but get tired fastest

229
Q

how is graded muscle force developed

A

partially based on the three different types of muscle fibers but also based on recruitment

overall, force is based on the number of active motor units

size principle= recruitment will occur accoridng to size of motor unit! i.e. smaller recruited first

230
Q

how do intra and extrafusal muscle fibers differ

A

extrafusal muscle fibers are those that general force

intrafusal muscle fibers are included in the muscle spindle apparatus and include nuclear bag and chain fibers; these are in parallel with extrafusal fibers and detect changes in length

231
Q

how does the muscle spindle regulate muscle length

A

muscle spindle contains fibersr that respond to changes in muscle length (this includes Ia and II afferent fibers)

Ia respond to dynamic changes in muscle length (i.e. upon tap with reflex hammer); linear stretch = increased firing, release = stop diring

II respond to the degree of stretch/ static response

232
Q

what is the role of the gamma fiber in muscle spindle function

A

motor fiber that attaches to the ends of the spindle and helps to maintain contracted tone and responsiveness to stretch in the muscle spindle unit

233
Q

Stretch reflex

A

monosynaptic; afferent fibers= Ia, efferent = alpha motor; responds to changes in muscle length (contract synergist, relax antagonist)

234
Q

Golgi reflex

A

disynaptic; afferent = Ib, efferent = alpha motor; responds to changes in muscle tension. leads to relaxation of synergist, contraction of antagonist

235
Q

flexor withdrawal reflexes

A

afferent= II, C (pain), and A delta; multiple interneurons/ polysynaptic; efferent = bilateral alpha motor neurons. Ipsilaterla will lead to flexion (withdraw from pain); contralateral = extension, stabiliize

236
Q

what are UMN- function, location, and signs of damage?

A

UMN- modulate activity of LMN to control movement; send descening projections to LMNs

located in motor cortex (precentral and paracentral lobules) and brainstem (vestibular nuclei, reticular formation, etc)

symptoms include: spastic paresis, hyperreflexia, hypertonia, upgoing Babinski

237
Q

what are LMN- function, location, and signs of damage?

A

LMN- final common pathway for initiating movement; project axons directly to skeletal muscle

located in spinal cord (ventral aka anterior horn) and brainstem (cranial nerve motor nuclei)

LMN symptoms include: paralysis or weakness (paresis), loss of reflexes or decreased reflexes, flaccidity/ loss of tone, muscle atrophy/ wasting, fibrillations/ fasciculations

238
Q

structures involves in corticospinal tract

A

UMN in layer 5 of motor cortex –> posterior internal capsule –> midbrain crus cerebri/ cerebral peduncle –> basilar pons –> medullary pyramids –> decussasion at the pyramidal decussation of medulla –> lateral cortical spinal tract in spinal cord (laterla white matter) –> synapses along ventral/ anterior horn with LMN

239
Q

structures involved in corticobulbar tract

A

ventrolateral precentral gyrus of motor cortex (layer 5 pyramidal neurons) –> internal capsule genu –> crus cerebri (midbrain) –> bilateral termination along CN nuclei (general rule) in brainstem

exception: hypoglossal innervates contralateral and lower facial = only crossed innervation

240
Q

how is posture controlled (feedforward/ feedback mechanisms)

A

feedforward mech via reticulospinal tract; feedback via vestibulospinal tract. There is also some input from rubrospinal tract, which arises from red nucleus and innervates UE extensors

241
Q

what kind of neurons project from LMNs?

A

alpha motor neurons; project directly via ventral root via spinal nerve to peripheral nerves

242
Q

lateral vs. medial ventral horn difference

A

lateral anterior/ ventral horn contains cell bodies of distal muscles (fine motor fx)

media anterior/ ventral horn cell bodies contain proximal muscle (posture, balance)

243
Q

nucleus ambiguus

A

nucleus of CN IX and X; controls pharynx/ larynx muscles

244
Q

how do golgi fibers and muscle spindles respond differently to stretch and tension/ force

A

muscle spindles are responsive to stretch; golgi fibers are responsive to tension;

when a muscle contracts you see increased afferent Golgi activity whereas you have decreased muslce spindle activity

245
Q

difference between fasciculations and fibrillations

A

both involve spontaneous electrical activity of muslce fibers that are denervated in cases of LMN symptoms.

fibrillations = need EMG to detect

fasciculations= can be seen with naked eye (i.e tongue fasciculations)

246
Q

acute poliomyelitis

A

fecal orally spread viral infection that can cause initial phase of fever, fatigue, nausea, HA, URI/ GI sx, neck stffness and limb myalgia

most severe symptoms present when there is virus attack of LMB in ventral horn (spinal polio); this leads to irreversible flaccid paralysis of limbs. if it involves the brainstem/ bulbar muscles can lead to respiratory failure (bulbar polio)

LMN syndrome and will therefore expect decraeased reflexes but intact sensation

Treatment: NONE; supportive. best = polio vaccine. eradicated most of the world except still endemic in Pakistan and Afghanistan

247
Q

what is the function of the precentral cortex

A

part of primary motor cortex; serves to aid in anticipatory/ feedforward motor action planning (not actually initiation of movement)

248
Q

positive shar waves and fibrillation potential on EMG

A

sing of inflammation and necrosis; seen with spontaneous EMG (i.e. without contractile effort)

249
Q

myotonic discharged on EMG

A

sign of pompe’s disease, toxic myopathy (i.e statins, colchicine), myotonic d/o, periodic paralysis

seen with spontaneous EMG (i.e. without contractile effort)

250
Q

nerve conduction studies typical findings with myopathy

A

typically these are normal, unless there is myopathy affecting distal muscles (may see low CMAP amplitude)

251
Q

angualted atrophic fibers on muscle biopsy

A

indicate neurogenic changes

252
Q

rounded atrophic fibers on muscle biopdy

A

indicate myopathic changes

253
Q

which myopathies are associated with asymmetric muscle findings

A

FSH-D (facioscapulohumorla dystrophy) and inclusion body myositis

254
Q

asymmetric winged scapula

A

can be seen with FSH-D or with inclusion body myositis

255
Q

pectoral creases

A

seen with FSH-D

256
Q

physical exam findings characteristic of FSH-D

A

asymmetric winged scapula and pectoral creases

257
Q

inclusion body myositis

A

knee extensor weakness, hand/ wrist flexor weakness. most common muscle disorder in those > 50 y/o. ASYMMETRIC muscle weakness.

can also present with asymmetric winged scapula (like FSH-D)

258
Q

rimmed vacuole seen on biopsy with

A

inclusion body myositis

259
Q

myotonic dystrophy subtypes and differences

A

both result in fixed weakness/ myotonia of mucles. forms = DM1 and DM2

a. DM1= more common, results from CTG repeat, defect in DMPK on chromosome 19q; presents with distal weakness, myotonia bifacial weakness, temporal wasting, frontal balding, ptosis.It is also often a/w cardiac problems, respiratory failure, OSA, cataracts, and endocrine dysfunction.
b. DM2= results from CCCG repeat in ZFN9 on chromosome 3
1. presents with calf hypertrophy and symptoms similar to DM1, but less CNS sx. Proximal muscle weakness/ atrophy

260
Q

Duchenne Muscular Dystrophy

A

A. caused by deletion of dystrophin gene; presents around age 3 with proximal > distal weakness that is symmetric in arms and legs. Tend to lose ability to ambulate by adolescence (9-13); Can have pseudohypertrophy of calves (fat buildup, not muscle). Commonly see contractures (esp at ankles) and scoliosis, especially after loss of ambulatory ability.

a. diagnostic labs include: high CK and ALT/ AST.
b. other disease risks = cardiomyopathy and MR

261
Q

Dermatomyositis

A

inflammatory disorder that presents with relatively acute onset proximal muscle weakness plus limb and facial rash. in adults, considered possible paraneoplastic syndrome –> cancer screening done. treatment = steroids, followed by steroid sparing agent.

262
Q

McArdle’s Disease

A

dz with glycogen phosphorylase with typical onset < 15. presents with exercise and fatigue and can have second wind phenom. Myoglobinuria common (50%). EMG can can show myopathic changes. Testing includes non-ischemic forearm test –> reduced lactate rpoduction with normal ammonia increase. Can also genetically test. On histo- glycogen filled vacuoles in muscle stores. Will also show deficiency myophosphorylase

263
Q

CPT2 deficiency

A

can lead to exercise intolerance 2/2 defect with FA metabolism. leads to weakness –> tends to occur with longer periods of exercise than in Mcardles

264
Q

A alpha fiber

A

skeletal muscle efferent

265
Q

IB fiber

A

skeletel muscle efferent (muscle spindle)

266
Q

IA fiber

A

skeletal muscle efferent (stretch)

267
Q

A delta fibers

A

skin afferents (temp and fast pain)

268
Q

C fibers

A

skin afferents for slow pain and SNS post gang afferents

269
Q

B fibers

A

SNS pregang visceral afferents

270
Q

A gamma fibers

A

muscle spindle efferent fibers (keep muscle spindle tone appropriate)

271
Q

what kind of neuropathy follows stocking glove distribution pattern

A

polyneuropathy

272
Q

describe the clinical presentation of guillain barre syndrome

A

tends to present ~2 weeks after a GI or respiratory illness with acute onset proximal and distal weakness that is rapidly progressive and ASCENDING (distal to proximal)

areflexia is common, as is respiratory impairment (can require intubation) and autonomic dysfunction

most common agent = campylobacter jejuni –> molecular mimicry

273
Q

what kind of damage does GBS cause?

A

demyelinating polyradiculopathy

274
Q

how is GBS diagnosed

A

mostly a clinical diagnosis based on history and exam!!

CSF and EMG studies may be early normal early on, but eventually can show

CSF: cytoalbuminologic dissociation

electrical studies- acquired demyelinating polyneuropathy

MRI can show hypertrophc, contrast enhancing nerve roots

275
Q

hypertrophc, contrast enhancing nerve roots on MRI can be indicative of what?

A

GBS

276
Q

how is GBS treated?

A

IV Ig or plasma exchange

also need to monitor respiratory function and for signs of autonomic dysfunction. send to rehab!

277
Q

common causative agents of GBS

A

campylobacter jejuni = most common cause (30%); 10% caused by CMV

278
Q

what are some common histologic findings a/w GBS?

A

perivenular and endoneurial inflammatory infiltrates such as histiocytes, plasma cells along with segmental demyelination

279
Q

Chronic Inflammatory Demyelinating Polyradiculopathy

A

onion skinning changes on biopsy secondary relapsing and remission pattern of disease (like chronic GBS)

280
Q

Charcot Marie Tooth Disease

A

inherited demyelinating disorder that is most commonly AD form; can also be AR.

treatment is supportive and involves bracing

281
Q

most commonly inherited neurologic disorder

A

charcot marie tooth disease

282
Q

CMT1 genetic cause

A

duplicaion of PMP22 gene (peripheral myelin protein 22); located on chromosome 17

283
Q

classic initial symptom of charcot marie tooth disorder

A

foot drop and foot deformities; can be followd by distal muscle atrophy and sensory loss

284
Q

when do symptoms of charcot marie tooth disease typically start

A

early childhood to early adulthood

285
Q

signs of axonal damage on nerve studies include

A

decreased signal amplitude; CV should be normal to slightly decreased (not less < 75% normal); latency should be normal to slightly prolonged

286
Q

signs of demyelination on nerve study include

A

decrased conduction velocity, prolonged latency, conduction block

287
Q

median mononeuropathy etiology and sympptoms

A

caused by compression of median nerve along passage through carpal tunnel; pain, paresthesias along median nerve course.

288
Q

recurrent thenar motor branch of medina nerve supplies

A

thenar eminnnce- opponens, abductor pollicis brevis, superficial head of flexor pollicis previs

289
Q

motor division of median nerve innervates

A

first and second lumbricals

290
Q

palmar cutaneous sensory branch of median nerve innervates

A

thenar eminence- note that this branches before carpal tunnel, so could be spared in compressive mononeuropathy

291
Q

management of ulnar nerve entrapment

A

generally conservative, splinting and avoiding prolonged flexion of elbow. if sx last > 6 months –> consider surgery

292
Q

where does ulnar nerve originate from (spinal levels)

A

C8-T1; continues along lower trunk to medial cord and continues along medial elbow

293
Q

fibular nerve spinal level derivation

A

L4-S1

294
Q

where does fibular nerve bifurcate

A

popliteal fossa into common fibular nerve and lateral cutaneous branch.

295
Q

lateral cutaneous nerve of knee

A

branches of the common fibular nerve near the popliteal fossa and supplies sensory innervation of lateral knee

296
Q

branches of common fibular nerve

A

deep and superficial branch

297
Q

deep fibular nerve innervation pattern

A

toe extensors (i.e. extensor digitorum longus, hallicus longus, digitorum brevis)

298
Q

superficial fibular nerve innervation includes

A

mid to lower calf sensation; this will also divide into the medial and intermediate dorsal cutaneous nerves, which supply dorsum of foot and dorsal medial 3-4 toes

299
Q

common causes of fibular neuropathy

A

prolonged surgery, leg crossing, squatting, casting

300
Q

fibular neuropathy symptoms include

A

foot drop, weak eversion (weak fibularis longus), and weak toe extension

301
Q

what sensory pattern follows with fibular neuropathy

A

along superficial/ deep fibular nerve distribution –> mid to lower calf, dorsum of foot, dorsal medial 3-4 toes

302
Q

which muscles are typically spared in ALS

A

extraocular muslces, sphincters, sensory nerves

303
Q

painless weakness = hallmark of which disease

A

ALS

304
Q

symptoms seen with ALS

A

UMN = spasticity, hyperreflexia; LMN = atrophy and fasciculations,

pseudobulbar affect (labile), frontotemporal dementia, cognitive changes

305
Q

mutations seen in familial forms of ALS

A

10% of cases are familial –> 40-50% have c90rf mutation, 10% have SOD1 mutation

306
Q

disease modifying drugs in ALS

A

riluzole and edavarone

307
Q

edavarone use and MOA

A

used to modify ALS; MOA not fully known but thought to be antioxidant

308
Q

Riluzone use and MOA

A

used to modify ALS; MOA = blocks glutamatergic NT

309
Q

Spinal muscular atrophy inheritance pattern and mutation

A

autosomal recessive 2/2 mutation in survival motor neuron gene (SMN1)

310
Q

SMN1 mutation

A

seen in SMA

311
Q

most common fatal genetic disease in infants

A

SMA (spinal muscular atrophy)

312
Q

anterior horn degeneration –> weakness and muscle atrophy describes what condition

A

spinal muscular atrophy; AR disease 2/2 mutation in SMN1 (survival motor neuron gene)

313
Q

baby with decreased muscle tone (hypotonia)

A

SMA (spinal muscular atrophy)

314
Q

most common type of spinal muscular atrophy

A

type 1, infant onset (sx onset between 0-6 months); these babies have progressive proximal muscle weakness, hypotonia, loss of reflexes;

on exam can have bell shaped chest (poor chest wall expansion with preserved diaphragm)

315
Q

cognition in SMA1

A

usually normal

316
Q

TDP-43 inclusions seen in which disease

A

implicated in ALS (amyotrophic lateral sclerosis); presents wiht protein rich inclusions of TDP_43(TAR DNA binding protein 43)

also seen in tau and alpha synuclein negative frontotemporal lobar degeneration

317
Q

what segments of spinal cord will show demyelination in ALS

A

lateral and anterior corticospinal tracts

318
Q

Bunina body

A

seen in ALS; cytoplasmic feature seen in motor neurons

319
Q

characteristics of Werndig Hoffman Disease

A

affects nervous system and skeletal muscle; grossly will present with shrunken anterior nerve roots and skeletal muscle atrophy

320
Q

nerve conduction studies are useful for what disease

A

peripheral nerve disease

321
Q

EMG is useful for what types of diseaes

A

anterior horn syndromes

322
Q

diabetic neuropathy

A

generally motor sparing; affects sensation. But can have different forms –> mononeuropathy, autonomic neuropathy, etc.

323
Q

cytoalbuminologic dissociation

A

elevated protein and normal WBC count on CSF analysis = sign of GBS

324
Q

benign peripheral nerve sheath tumors

A

Schwannoma and neurofibroma

325
Q

malignant peripheral nerve sheave tumor

A

malignant peripheral nerve sheath tumor (MPNST)

Neurobiology (2 decks)

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