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Neurology Flashcards

1
Q

Delirium

A

an acute state of confusion, which may be characterized by

a reduced level of consciousness,
cognitive abnormalities,
perceptual disturbances,
emotional disturbances.

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2
Q

Coma

A

sleeplike state in which the eyes are closed and the patient is unarousable even when vigorously stimulated

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3
Q

Vegetative state

A

complete unawareness of self and surroundings but preserved sleep-wake cycles and at least partial preservation of hypothalamic and brainstem autonomic functions

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4
Q

Causes of Coma

A

50% - cerebrovascular disease

20% - hypoxic injury

30% - toxic, metabolic, infectious

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5
Q

3 important questions to evaluate coma

A

Does the patient have meningitis?

Are signs of a mass lesion present?

Is this a diffuse syndrome of exogenous or endogenous metabolic cause?

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6
Q

Glasgow Coma Scale - interpretation

A

Brain injury is classified as:

Severe: < 9
Moderate: 9-12
Minor >= 13

Always state the individual score for each aspect of the scale

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7
Q

Decorticate posturing

A

Flex elbows and wrists
Addudction of shoulders
Extension of legs

Less severe neuro injury

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8
Q

Decerebrate posturing

A

Extension of elbows, wrists, legs

Adduction + internal rotation of shoulder

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9
Q

What strongly suggests metabolic cause of coma?

A

Myoclonic jerks

Tremor and asterixis in awake pt

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10
Q

Most impt brainstem reflexes

A

Pupilary light
Corneal
Conjugate eye mvmts

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11
Q

Loss of pupilary light reflex

A

Brainstem herniation

If only 1 pupil size blown, suggests temporal lobe herniation and impingement on 3rd cranial nerves

Symmetric small = OD w/ opiates

Symmetric large = Cocaine, TCAs

Eyes deviate to one side = large cerebral lesion (look away from paralyzed side)

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12
Q

Coma after cardiac arrest

A

No pupilary and corneal reflexes @24 hrs
No motor response @ 72 hrs

Little chance of meaningful recovery

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13
Q

Coma w/ focal signs suggests…

A

Structural lesion

Stroke
Hemorrhage
Tumor
Abscess

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14
Q

When do LP in unexplained coma?

A

Meningitis

SAH (but neuroimaging normal)

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15
Q

When do to LP in headache?

A

concern for meningitis (fever and neck stiffness)

or encephalitis (focal neurologic signs, confusion, altered mental status)

if subarachnoid hemorrhage is suspected but imaging studies are normal

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16
Q

Criteria for migraine diagnosis

A

POUND

If you have >=3 of the following:

Pulsatile quality (headache described as pounding or throbbing)

One-day’s duration (episode may last 4-72 hours if untreated)

Unilateral in location

N/V

Disabling intensity (altered usual daily activities during headache episode)

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17
Q

Route of admin of drugs for severe N/V in migraine?

A

Intranasal

Parenteral

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18
Q

Dementia

A

persistent impairment of intellectual function with compromise in at least three of the following spheres of mental activity:

  • language,
  • memory,
  • visuospatial skills,
  • emotion or personality,
  • cognition (abstraction, calculation, judgment, executive function)

ONLY dx if tehre is functional impairment

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19
Q

Dystonia

A

involuntary, sustained contraction of agonist/antagonist muscles, which often can lead to uncomfortable or even painful twisting, bizarre-looking postures

D blocker drugs can cause this

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20
Q

What kind of movement disorder is asterixis?

A

Negative myoclonus

Sudden interruption of sustained muscle constrictions leading to loss of tone

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21
Q

Parkinson diagnosis

A

based on three cardinal clinical features:

  • bradykinesia,
  • resting tremor,
  • postural instability
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22
Q

Dystonic reactions from D2 receptor blocker drugs most often affect…

A 
Ocular muscles
Face
Jaw
Tongue
Neck 
Trunk

Limbs rarely affected

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23
Q

Torticollis

A

Cervical dystonia

Abnormal postures of head, neck and shoulders

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24
Q

1 cause encephalitis in US

A

HSV

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25
Q

1 cause of bacterial meningitis

A

S. pneumo

N. meningitidis is #2

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26
Q

Rash of meningitis

A

Petechial
Maculopapular
Purpuric in appearance

Usually spares soles and palms

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27
Q

What doesn’t the N meningitidis vaccine protect against?

A

Serogroup B

Is 1/3 of cases of bacterial meningitis in US

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28
Q

How do you dx aseptic meningitis?

A

CSF PCR

or

IgM antibody capture ELISA

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29
Q

Viral encephalitis EEG findings

A

EEG findings include focal delta activity over the temporal lobes, typically occurring between 2 and 14 days after symptom onset; periodic lateralizing epileptiform discharges (PLEDs) also may be noted.

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30
Q

When do you brain bx for encephalitis?

A

Pts who don’t respond to acyclovir

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31
Q

When do you tx HTN in acute stroke?

A

When greater than 220/120

OR

There is another acute indication for lowering BP such as ACS, CHF, aortic dissection, hypertensive encephalopathy or AKI
- Lower Bp by 15% over 1st 24 hrs

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32
Q

In stroke, If the patient is otherwise a candidate for thrombolytic therapy, what should BP be?

When give it?

Contraindications?

A

blood pressure must be stabilized and lowered to 130 mm Hg, systolic blood pressure >185 mm Hg, diastolic blood pressure >110 mm Hg, or intracerebral hemorrhage

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33
Q

urgent anticoagulation with heparin for stroke?

A

is not recommended for patients with ischemic stroke unless cerebral venous thrombosis, basilar occlusion/stenosis, or extracranial arterial dissection is suspected.

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34
Q

When do you suspect vasculitis as cause of mononeuropathy?

A

When acute involvement of individual nerves is accompanied by pain

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35
Q

Difference in presentation of axonal vs demyelinating polyneuropathy?

A

Axonal - symmetric distal sensory loss +/- burning, tingling (eg diabetes)

Demyelinating - p/w motor sx (eg Guillain Barre)

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36
Q

Most sensitive for diagnosis of meningitis

A

Jolt accentuation of headache from horizontal mvmt of head

More sensitive than Kernig or Brudzinski sign

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37
Q

What HSV is more common causing encephalitis in

  • adults
  • children?
A

Adults - HSV 1

neonates - HSV 2

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38
Q

How does HSV encephalitis usually happen in adults?

A

Reactivate latent virus in trigeminal ganglion —> inflammatory necrotic lesions in temporal cortex and limbic system

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39
Q

Intention or kinetic tremors are most characteristic of damage to

A

Cerebellum

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40
Q

What happens to Parkinson tremor when a person sleeps?

A

Resting tremor STOPS when relaxation progresses to sleep

BUT USUALLY HAVE PILL ROLLING TREMOR AT REST

Most tremors caused by basal ganglia pathology stop during sleep

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41
Q

What does hyperacusis tell you about where injury to CN 7 is?

A

Close to origin from brainstem b/c nerve to stapedius is one of 1st branches of facial nerve

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42
Q

Fasiculations occur because

A

Indicate denervation

Occur through hypersensitivity to Ach acting at denervated NMJ

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43
Q

Most common location for lumbar disk herniation

A

b/n L5 and S1

b/n L4 and L5

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44
Q

Common objective sign of S1 radiculopathy

A

Loss of ankle jerk or achilles tendon reflex

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45
Q

Hollenhorst plaques

A

Cholesterol and calcific deposits in retinal arterioles

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46
Q

West syndrome

A

Generalized seizure d/o of infants characterized by

  • recurrent spasms
  • EEG pattern of hypsarrhythmia
  • retardation

Tx: ACTH

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47
Q

Women are more susceptible to symptomatic aneurysms than men in their 40s adn 50s. Where are the aneurysms?

A

Most especially true of those that are on internal carotid on segment of artery that lies in cavernous sinus

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48
Q

Difference between classic vs. common migraine

A

Classic = has preceeding aura of neuro dysfunction (usually visual)

DOES NOT HAPPEN IN COMMON

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49
Q

Difference between ischemic vs. compression injury of CN 3

A

Ischemic

  • usually due to diabetes
  • usually spare superficial pupilloconstricor fibers b/c of different vascular supply from rest of nerve

Compression
- usually get pupilloconstrictor fibers first b/c superficial

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50
Q

What artery lesions compress CN3?

A

Posterior communicating

Also but less common:

  • SCA
  • PCA
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51
Q

Trigeminal neuralgia can be associated with…

A

Multiple sclerosis

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52
Q

Lennox-Gasttaut syndrome

A

Mental dysfunciton
Multiple seizure types
- complex partial seizures usually
1-2 Hz generalized spike wave discharges on EEG

Many children have hx of infantile spasms (West syndrome)
20% of Tuberous sclerosis kids will develop this

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53
Q

Hypsarrhythmia

A

EEG pattern of children w/ paroxysmal flexions of body, waist or neck

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54
Q

Landau-Kleffner syndrome

A

Loss of language and function

Abnormal EEG during sleep

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55
Q

Atypical facial pain vs. trigeminal neuralgia

A

Trigeminal neuralgia
- paroxysmal lacrimating pain

Atypical pain

  • constant, deep pain
  • usually bilateral but can be unilateral
  • often sensitive to antidepressant meds
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56
Q

Olfactory aura preceding seizure - where is the lesion?

A

Hippocampus or parahippocampal gyrus

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57
Q

Epilepsia partialis continua

A

Condition of persistent focal motor seziure activity
- a focal motor status epilepticus

Distal hand and foot muscles most often affected

Response to therapy is poor

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58
Q

Causalgia

A

Can result from trauma to nerves in extremities

Is a disturbance in sensory perception characterized by

  • hypesthesia
  • dysesthesia
  • allodynia

Bullets can cause this as their high velocity can cause enough energy transmission through adjacant tissues to produce damage to nerve

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59
Q

Hypesthesia

A

Decrease in accurate perception of stimuli

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60
Q

Dysestheisa

A

persistent discomfort

- usually unremitting burning pain

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61
Q

Allodynia

A

perception of pain w/ application of nonpainful stimuli

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62
Q

Musculocutaneous N innervation

A

Damaged w/ fracture of humerus

Supplies

  • biceps brachii
  • brachialis
  • coracobrachialis muscles

Carries sensory info from lateral cutaneous nerve of forearm

Damage causes:
- impaired flexion at elbow with forearm supinated

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63
Q

For subdural hematoma of a few days, what imaging do you use?

A

Not CT - after few days, blood can be degraded into less dense fluid and difficult to distinguish
- eventually will be hypodense to brain

USE MRI

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64
Q

Which brain bleeds are unilateral? bilateral?

A

bilateral - subdural

unilateral - epidural

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65
Q

Tinel sign

A

sensation of tingling radiating away from percutaneous percussion of a peripheral nerve

Usually happens w/ carpal tunnel syndrome
60% sensitivity
67% specificity

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66
Q

Brudzinski and Kernig signs

A

Indication of meningeal irritation

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67
Q

Monrad-Krohn test

A

Confirm psychogenic upper extremity monoparesis

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68
Q

What is most common sign of neuro disease w/ encephalitis lethargica?

A

Disturbed eye movements

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69
Q

Most common sequelae of encephalitis lethargica

A

Unremitting parkinsonism w/ signs and symptoms similar to in idiopathic Parkinson

UNIQUE: oculogyric crises = episodes where eyes deviate to one side or up

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70
Q

Facial injury most likely to develop w/ sarcoidosis

A

Facial paresis

Weakness on 1 side of face
+
no substantial loss of sensation over paretic side

Maybe decreased sensitivity to touch

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71
Q

Sequelae of Schistosoma mansoni

A

Endemic in tropics

Can cause subacute evolving paraparesis

DOES NOT invade spinal cord
- deposits eggs in valveless veins of Batson which drain intestines and communicate w/ drainage from lumbosacral spinal cord

Develop granulomas around ova that lodge in spinal cord
- granulomatous lesions crush the cord

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72
Q

Echinococcosis clinical findings

A

Usually acquired by ingesting material contaminated w/ fecal matter from sheep or dogs

Children - cerebral lesions more often than adults
= encephalic hydatidosis

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73
Q

CSF findings with CJD?

A

Usually normal

May have elevated protein level

20% of cases may have increase in ratio of IgG : total protein, occasionally w/ oligoclonal bands

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74
Q

Most common etiologies of rim-enhancing brain lesions in AIDS pts

A

Primary CNS lymphoma
- CSF EBV PCR test is highly sensitive and specific for this

Toxoplasma infection

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75
Q

What kind of lesions in brain do you have with HIV and CMV encephalitis?

A

Microglial nodules

HIV = usually nodules around blood vessels throughout brain

CMV = nodules usually subpial and subependymal

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76
Q

Where do discharges in herpes encephalitis on EEG typically occur? What kind of discharges?

A

Over temporal regions

Bilateral, periodic epileptiform discharges

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77
Q

How do abscesses in brain usually form?

A

Hematogenous spread of infection

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78
Q

Where do brain abscesses usually start in the brain?

A

At junction of gray matter and white matter

As infection develops, cerebritis appears and subsequently this focus of infection becomes necrotic and liquefies

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79
Q

Syphilis - long term sequelae

A

General paresis
- slow evolving

Early sx:
- subtle dementia (memory loss, impaired reasoning)

Late sx:

  • dysarthria
  • myoclonus
  • tremor
  • seizures
  • UMN signs
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80
Q

Structural brain changes with neurosyphilis

A

Meninges are thickened and opaque

Granular ependymitis develops

Degenerative changes throughout cerebral parenchyma

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81
Q

Rhombencephalitis - principal targets of this disease?

A

Pons

Medulla oblongata

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82
Q

Most common sx among those w/ brain abscesses?

A

Headache (3/4 of pts)

tx w/ surgical resection

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83
Q

Most common pathogen for brain abscesses

A

Aerobic +/anaerobic STREP bacteria (> 1/2)
Bacterioides

Staph aureus in pts w/ penetrating head wounds or undergone neurosurgical procedures

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84
Q

Old person + stiff neck + blurry vision + fever + headache

CSF = mild pleocytosis w/ no organisms

All blood + CSF cx negative

best med tx for organism likely responsible?

A

Could be listeria monocytogenes

Use ampicillin + gentamycin

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85
Q

Inclusion bodies in oligodendrocyte nuclei - what does this suggest for an infection?

How to dx?

A

JC virus = PML

Use MRI to dx
- multiple focal well-defined white matter lesions that DO NOT enhance or have mass effect

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86
Q

When are eye muscles affected in guillain Barre?

A

Miller Fisher variant

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87
Q

Guillain Barre vs. polio - how do you telll?

A

CSF analysis

GB = elevated protein count + normal or slightly elevated abnormal WBC + normal glucose

Polio = small increase protein + lymphocytes

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88
Q

Subacute sclerosing pancencephalitis

  • causes
  • CSF analysis
A

SSPE

Usually in children

Usually kids had measles
Death usually in 1-3 years

CSF like MS w/ oligoclonal bands (measles-specific antibody)

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89
Q

Regional adenitis usually in epitrochelar nodes

What is this assoc w/ ?
What does it look like?
Tropism?

A

Cat scratch disease
- B. henselae

Can produce self-limited aseptic meningitis

In immunocompetent, can get encephalitis + status epilepticus + neovascular proliferation (bacillary angiomatosis)

MRI may show characteristic increased signal intensity in the pulvinar (part of thalamus)

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90
Q

Calcification 2/2 brain tumor on skull xray suggests…

A

Astrocytoma
Meningioma
Oligodendroglioma
Metastatic tumor

Meningioma – hyperostosis may develop in bone adjacent to tumor even w/o infiltration of bone by tumor

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91
Q

1 source of metastatic tumors to brain?

A

Lung

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92
Q

Why is melanoma in brain esp bad?

A

Highly likely to bleed after it metastasizes to brain

Same is true of mets w/ choriocarcionma

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93
Q

Precocious puberty / acromegaly + visual field developments + no cancer

A

Hypothalamic hamartomas

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94
Q

Craniopharyngiomas

A

Epithelial neoplasms arising in sellar and 3rd venticular regions

Can cause HYPOpituitarism + visual field defects (vs hyper in hypothalamic hamartomas)

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95
Q

NF-1 clinical sequelae

A

Cafe au lait spots

Multiple cutaneous + subQ tumors

Bone cysts

Sphenoid bone dysgenesis

Precocious puberty

Pheo

Syringomyelia

Glial nodules

Cortical dysgenesis

Macrocephaly

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96
Q

Parinaud syndrome

A

Loss of vertical gaze, pupillary light reflex, lid retraction, covergence-retraction nystagmus

Usually due to lesion in dorsal midbrain in region of superio colliculus

CAn be caused by pineocytomas

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97
Q

Paraneoplastic cerebellar degeneration

A

Subacute, progresive ataxia, dysarthria, nystagmus

Usually assoc w/ Small cell carcinoma, ovarian carcinoma, lymphoma

50% pts have anti-Purkinje cell bodies (anti-Yo Abs)

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98
Q

Fabry disease

  • deficient enzyme
  • accumulated substrate
A

a-galactosidase A

Ceramide trihexoside

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99
Q

Gaucher’s disease

  • deficient enzyme
  • accumulated substrate
A

Glucocerebrosidase

Glucocerebroside

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100
Q

Krabbe’s disease

  • deficient enzyme
  • accumulated substrate
A

Gaucher and Krabbe are friends!

GALACTOcerebrosidease

Galactocerebroside

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101
Q

Neimann-Pick disease

  • deficient enzyme
  • accumulated substrate
A

Sphingomyelinase

Sphingomyelin

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102
Q

Tay Sach’s disaese

  • deficient enzyme
  • accumulated substrate
A

HeXosaminidase A

GM2 ganglioside

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103
Q

Metachomatic leukodystrophy

  • deficient enzyme
  • accumulated substrate
A

Arylsulfatase A

Cerebroside sulfate (galactosyl sulfatides)

Can use nerve biopsy to diagnose

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104
Q

Type of peripheral neuropathy most commonly developing w/ CRF

A

Symmetric
Distal
Mixed

Sensorimotor neuropathy

Legs affected 1st usually and most severly

Dialysis causes you to lose B vitamins –> neuropathy (thiamine depletion)

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105
Q

Akathisia

A

Restlessness occuring during daytime

vs restless leg syndrome is only at night

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106
Q

Most damaged areas in spinal cord w/ cobalamin deficiency

A

Lower cervical

Upper thoracic

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107
Q

Pathology of neuropathy in cobalamin deficiency

A

Starts as demyelinating lesion

Evolves into axonal loss

(Hyperreflexia –> hyporeflexia

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108
Q

Vitamin deficits in tobacco-alcohol amblyopia

A

B1
B12
Riboflavin

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109
Q

Vit E deficiency sequelae

A

Spinocerebellar degeneration
Polyneuropathy
Pigmentary retinopathy

Usually degen in :
Clarks columns
Spinocerebellar tracts
posterior columns
Nuclei of Goll and Burdach
Sensory roots

Get ataxia

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110
Q

Pickwickian syndrome

A

Obesity assoc w/ hypersomnia

Ex sleep apnea

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111
Q

Features of Alzheimer’s disease

A

Neuronal loss
Fibrillary tangles
Loss of synapses
Amyloid plaque formation

Tangles + neuronal loss most common in hippocampis + adjacent structure in hippocampus

Language deficit (decreased fluency, dysnomia, transcortical sensory aphasia)

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112
Q

EEG of Alzheimer’s pt

A

Slowing of posterior-dominant rhythm (8-12hz) of normal adult EEG

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113
Q

1 cause of dementia in gen pop

A

Alzheimer’s disease

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114
Q

NPH s/p ventriculoperitoneal shunting - complications?

A

Subdural hematoma
- reducing intracrainal P due to less CSF may cause brain to pull away from covering meninges and break bridging veins

Infections

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115
Q

EEG findings of CJD?

A

Periodic sharp waves at 1-2 Hz frequency on EEEG

Elevated protein 14-3-3- in CSF

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116
Q

Neurosyphillis dx

A

Monocytic pleocytosis

+ serological tests for syphilis

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117
Q

Neuro sx of hypothyroidism

A

headache
dementia
psychosis
decreased consciouness

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118
Q

Neuro sx of Whipple disease

A

CNS infection can happen w/o GI disease

Seizures
Myoclonus
Ataxia
Supranuclear gaze disturbanes
hypothalamic dysfunction
dementia

Oculomasticatory myorhythmia (pendular convergence mvmts of eyes in assoc w/ contractions of masticatory muscles) = pathognomonic

Bx of jejunum to dx

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119
Q

Where is the substantia Nigra?

A

Brainstem

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120
Q

What is PML?

A

In immunocomp, usually AIDS pts

Affects subcortical white matter usually in

  • OCCIPITAL
  • PARIETAL regions

CSF normal
Lesions do not enchance on imaging
See foci of abnormality on MRI

Usually does not cause mass effect - onset is gradual

Most common presenting sx:

  • hemiparesis
  • disturbances in speech, vision, gait
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121
Q

Chorea gravidarum

A

Involuntary mvmt d/o during pregnancy

Rapid + FLUID but not rhythmic limb and trunk movements

Can appear w/ estrogen use too
Main problem is dramatic change in hormonal environment of brain

At end of preggers, or no more estrogen, movements stop

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122
Q

How do dopaminergic drugs affect Huntington’s in the beginning stages?

A

Can unmask chorea

NOT advisable b/c can contribute to premature symptom of chorea

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123
Q

Choreiform movement disorders

A

Huntington

Hereditary acanthocytosis

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124
Q

Theory of Parkinson’s

A

substantia nigra pars compacta has decreased dopamine production

Get overinhibition of thalamocortical pathways

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125
Q

Ways to tx Parkinson’s

A

Thalamus can eb directly intervened to decrease overinhibition

Globus pallidus interna can be lesioned/stimulated

Lesion/Stimulate subthalamic nucleus

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126
Q

Lewy Bodies

A

IN Parkinson’s

IntraCYTOPLASMIC inclusion bodies

Eosinophilic inclusions w/ poorly staining halos surrounding them

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127
Q

Effects of language in Parkinson’s

A

Not disturbed

Clarity and volume of speech deteriorate w/ development of hypophonia

Handwritting also gets smaller = micrographia

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128
Q

Meige syndrome

A

Form of focal dystonia characterized by:

Blepharospasm
Forceful jaw opening
Lip retraction
Necak contractions
Tongue thrusting

Can occur w/

  • phenothiazine
  • butyrophenone use
  • idiopathic (women > men)
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129
Q

Spasmodic torticollis

A

Focal dystonia

Starts early in adult life

Contraction of neck muscles –> painful –> hypertrophy

Standing and walking worsen contractions

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130
Q

Manifestation of Wilson’s disease

A

Renal tubular acidosis

Hepatic fibrosis

Heart + lung damage

Brain + liver disease

Dementia

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131
Q

Oligoclonal bands on CSF ddx

A

MS
Syphilis
Lyme
Subacute sclerosis pancencephalitis (SSPE)

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132
Q

Pt feels electrical sensation radiating down spine when neck passively flexed - what is this? What does it signify?

A

Lhermitte sign

Signifies spinal cord disease

Happens in MS pts

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133
Q

Composition of oligoclonal bands in MS pts

A

IgG

K-light chain composition

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134
Q

Jolly test

A

Evoked response involving muscles

Cannot usually be used to test VER in MS

Peripheral nerve shocked 5-15x per second and pattern of A/P is recorded
- Peripheral test normal in MS as is more a CNS disease

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135
Q

Canavan disease

A

Defect in N acetylaspartic acid metabolism

Can get developmental regression ~ 6mo age

Extensor posturing and rigidity
Myoclonic seizures

Increase in brain volume and weight

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136
Q

Bladder abnormality w/ MS

A

Spastic (UMN) bladder

Little or no residual urine in bladder after emptying – contractility good but distensibility poor

CAN GET MS EXACERBATION WITH EXERCISE! Because of the heat

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137
Q

Neuromylitis optica

A

Bilateral optic neuritis + transverse myelitis
- demyelinate optic N and spinal cord

Transverse myelitis = inflammatory demyelinating lesion transecting most of spinal cord

  • paraparesis
  • bladder and bowel dysfunction
  • sensory deficit

Can have cerebellar involvement, more cerebral involvement

Looks like MS but is not!

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138
Q

CSF profile of acute disseminated encephalomyelitis

A

Often fatal

MRI or CT shows rapidly evolving white matter damage assoc w/ high ESR

CSF:

  • increased P
  • elevated RBC, WBC
  • elevated protein
  • glucose NORMAL
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139
Q

Adrenoleukodystrophy

A

Adrenal dysfunction + progressive degenerative disease of white matter

Some types are X linked

  • mostly in boys
  • survival limited to ~3 yrs

Underlying defect in X linked = ATP binding transporter in peroxisomal system responsible for long chain fatty acid metabolism

LCFA accumulate in adrenals and other cells

Similar to adrenomyeloneuropathy in woemnn

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140
Q

Adrenomyeloneuropathy

A

More damage to spinal cord + peripheral nerves

Paraparesis

Problems w/ bowel and bladder control

Sensory distrubances in legs

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141
Q

Pelizaeus-Merzbacher disease

A

Demyelinating d/o part of disease known as sudanophilic leukodystrophies

Mostly males

affect growth of the myelin sheath

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142
Q

Leukodystrophy

A

disturbance of white matter

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143
Q

Sudanophilic

A

Sudan-staining characteristics of involved white matter

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144
Q

Lipid profile:

  • absent chylomicrons, VLDL, LDL
  • serum and red-cell lipid proteins normal

What is the disease? What is the defect/mutation? What does it look like?

A

Abetalipoproteinemia (Bassen-Kornzweig syndrome)

Mutation of gene: microsomal triglyceride transfer protein

RBC = acanthocytes

Ataxia
- posterior column + spinocerebellar tract degeneration (like Friedreich)

Vitamin E can help stop progression

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145
Q

VHL person has cyst + masses in cerebellar hemisphere on CT. What do you do?

A

Surgical resection of cerebellar lesions ASAP

Likely hemangioblastomas
- can bleed and produce potentially lethal intracranial hematomas

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146
Q

Fragile X phenotype

A

Females - normal

Men -
hyperextensible joints
prominent thumbs

Can detect abnormal chromosome in fetal lyphocytes + fibroblasts for prenatal screening

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147
Q

With PKU, what does the child have dangerously high levels of?

A

Phenylalanine

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148
Q

Railroad track pattern on plain xray of skull - intracranial calcification pattern indicative of?

A

sturge weber

Calcifications follow gyral pattern of cerebral cortex

Abnormal blood vessels overlying brain allow Ca, Fe across defective blood-brain barrier –> calcifications

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149
Q 
Erythrocytosis + 
Cerebellar signs + 
Microscopic hematuria + 
Hepatosplenomegaly
= ?
A

von Hippel Lindau syndrome

Has:
polycystic liver + kidney disease
reitnal angiomas (telangiectasia)
cerebellar tumors

AD w/ variable penetrance
More men affected

Hemangiomas in bones, adrenals, ovaries
Hemangioblastomas in SC, brain stem, cerebellum

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150
Q

Infection causing congenital aqueductal stenosis –> hydrocephalus

A

Mumps

Rubella

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151
Q

Tuberous sclerosis

- characteristics

A 
Retinal phakomas
Adenoma sebaceum
Periventricular tubers
Ash leaf spots
Shagreen patches
CNS calcifications
Renal tumors
Cardiac rhabdomyomas
Epilepsy
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152
Q

By 5 years of age, more than 1/2 of pts w/ tuberous sclerosis will have…

A

Subependymal glial nodules that have calcified

Can get big enough to cause obstructive hydrocephalus

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153
Q

Child w/ congenital weakness, hypotonia, muscle atrophy

A

Werdnig-Hoffman disease

congenital motor neuro disease
Anterior horn cell disease

Life expectancy = weeks - months

154
Q

Older child w/ weakness, hypotonia, muscle atrophy

A

Kugelberg-Welander disease (like Werdnig-Hoffman but in older kids)

Part of class known as spinal muscular atrophies

Anterior horn cell disease

155
Q

Static encephalopathy

A

Brain damage stopped by neuro problems persist

156
Q

Static motor disorder

A

Child w/ this has cerebral palsy

Will have static brain lesion but deficits may evolve as child matures

157
Q

Alcohol abuse in preggers

  • fetal abnormalities?
  • mech of action?
A

1) Intrauterine + postpartum growth retardation
2) dysmorphic facies in newborn
3) effects on development of CNS

EtOH teratogenic at high doses
- impairs neuronal migration

Commonly causes
- mental retardation, learning disabilities, hyperactivity, micocephaly (not MACRO)

158
Q

Prenatal CMV infection - problems developing?

A

Retardation
microcephaly
Seizures
Hearing deficits

Chorioretinitis
Optic atrophy

159
Q

What’s a skin paraneoplastic syndrome?

A

Dermatomyositis! (15% of cases of derm are paraneoplastic)

Lung, ovarian, GI, breast tumors can cause this )not CNS)

Always check for cancer when pt has dermatomyositis

160
Q

HIgh CPK in woman w/ relatives affected by Duchenne dystrophy = ?

A

High prob that she is carrier of abnormal dystrophin

normal CPK does not rule out carrier status

161
Q

Duchenne dystrophy incidence?

A

1 in 3,000 male infants

162
Q

Myotonic dystrophy

A 
Problems relaxing grip
Hypersomnolence
Premature baldness
Testicular atrophy
Cataracts
Cardiac defect needing pacemaker

EMG:
dive bomber pattern
repetitive discharges w/ minor stimulation

163
Q

Polymyalgia rheumatica

A

ONLY in persons older than 60 yo

Inc ESR
Anemia
Wt loss
Malaise
Normal CPK
164
Q

Signs of brainstem disease in ALS

A

ALS has loss of

  • anterior horn cells (LMN)
  • motor nuclei of brainstem + loss of large motor neurons (Betz cells) (UMN)

Brainstem disease:
Diaphragmatic weakness
Facial fasiculations

Brainstem disease signs early in disease is poor prognosis (<1 yr survival)

165
Q

What indicates graver prognosis in ALS?

A

Brainstem disease
= early involvement of musculature supplied by cranial nerves

Ex:
disturbed swallowing
recurrent aspiration
distrubed ventilatory activity
fasiculations of tongue
166
Q

How does EtOH abuse damage brain?

A

Superior vemis of cerebelllum:

  • loses purkinje cells
  • atrophy off molecular layer

White matter is cerebellum usually OK

167
Q

Lathyrism

A

Slow or subacute onset of spastic paraparesis
- too much chickling pea diet (L sativus)

Toxin through to be B-N-oxalylamino-L-alanine = excitatory NT that can induce disease in primae models

Damage in CNS usually in SC tracts (corticospinal, spinocerebellar tracts)
Demyelination in lateral and posterior columns of SC

168
Q

Tick paralysis

A

Can happen esp w/ australian tick producing holocyclotoxin = interfere w/ presynpatic release at NMJ

Remove tick, get dramatic improvement

169
Q

Risk w/ radiation for Hodgkin disease

A

Thyroid cancer

Carotid stenosis b/c of accelerated atherosclerosis from ionizing radiation

Surgical tx more difficult b/c radiation causes scarring of tissues around vessels –> harder to get to for surgery

170
Q

Hepatic encephalopathy

- sx

A 
Initially:
Decrease in level of alertness
irritability
Depression
tremor
asterixis
Later:
Lethargy
Paranoia
Bizzare behavior
Dysarthria
Nystagmus
Pupillary dilatation
171
Q

Sturge weber syndrome

A 
Port wine spots on faces
Contralateral hemiparesis
Retardation
Seizures
Glaucoma
Leptomeningeal angiomatosis causing intracranial calcifications
Angioma of choroid of eye
172
Q

Pseudotumor cerebri

A

Increased CSF pressure
- not at risk of herniation

Can happen in obese or preggers woman - thought to rise from hormonal problems

W/o tx, increased ICP will produce optic N damage w/ loss of VA

173
Q

reversible posterior leukoencephalopathy

A

Looks like HTN encephalopathy

Involves more than white matter, occur in anterior frontal regions

caused by cyclosporine and tacrolimus

174
Q

Do you get complete hearing loss on one side w/ one side temporal lobe infarction?

A

No

Hearing in each ear is represented bilaterally (even at brainstem)

If unilateral hearing loss, eval if loss is conductive or sensorineural

175
Q

Mastoditis can extend…

A

Supratentorially –> temporal lobe –> fluent aphasia (Wernicke)

Infratentorially –> cerebellum –> ataxia

176
Q

Mechanical trauma hearing loss - what does it look like?

A

High tone conductive loss

Perforates eardrum

177
Q

Cerebellar damage + severe vertigo

- where is the lesion?

A

PICA lesion

Vertigo caused by:

  • vestibular nuclei infarction
  • cerebellar flocculonodular lobule injury
178
Q

Vertigo on extreme extension or rotation of head –> insufficiency of?

A

Vertebrobasilar system

179
Q

Transient dizziness + ringing + decreased hearing in one ear

Early hearing loss in this disease is?

A

Meniere’s disease!
- can also have fullness in ear

Lower frequency hearing loss

Thought to be due to distension of semicircular canal and increase in volume of endolymph fluid

180
Q

Presbycusis

A

High frequency hearing loss

181
Q

Acoustic schwannomas usually tumors on what?

A

vestibular division of nerve

182
Q

Olfactory cortex located…

A

Olfactory tract –> medial + lateral striae
Medial stria –> anterior commissure –> opposite hemi
Lateral stria –> medial adn cortical nuclei of amygdaloid complex + prepiriform area

Primary olfactor cortex = area 34 of Brodmann
- hippocampal gyrus + uncus

NO FIBERS THROUGH THALAMUS

183
Q

Foster Kennedy syndrome

A

Ipsilateral optic atrophy + Contralateral papilledema in assoc w/ intracranial tumor

Meningioma of olfactory groove can produce this syndrome

184
Q

Benign Positional vertigo

A

Usually in middle aged ppl

Possibly due to otolith material in posterior semicircular canal

Attacks of recurrent rotational vertigo

  • change in head position
  • lying down
  • turn head onto side of affected ear

Use nylan-barany or hallpike maneuvers to confirm peripheral cause of vestibulopathy rather than central

  • will get rotary nystagmus
  • fatigability in sx (decrease them w/ increased provocation)
185
Q 
Progressive vertigo
Ataxia
Sensory loss
Dysphagia
Hiccups
A

Sx of lateral medullary syndrome

Usually due to distal vertebral artery occlusion

186
Q

Low lying vermis or cerebellar tonils + syringomyelia

A

Chiari malformation

Posterior fossa abnormally small
Tentorium cerebelli relatively low on cranium

187
Q

Tx syringomyelia

A

Laminectomy
- reduces damage to SC from pressure that develops

Cyst aspiration

Marsupilization
- slice open adn leaving open cyst

Shunting

188
Q

Spinal shock

A

Transient phenomenon

Occurs w/ damage to fibers from UMN

Usually within a few DAYS of spinal cord injury

Represents exaggeration of normal stretch reflexes in limbs disconnected from UMN control

189
Q

Lumbar spine injury in MVA - how did it happen?

A

Extreme flexion

190
Q

Syringomyelia

A

Pain and temp loss - bilateral

Tactile sensation ok

191
Q

Artery of Adamkieqicz enters at what level of SC?

A

At risk for occlusion w/ ab aortic aneurysm repair

Supplies lower 2/3 of spinal cord

T10-L1

192
Q

Hemisection of SC - at what level do you get STT effects?

A

Contralateral 2 levels below the spinal level hemisection

193
Q

Periumbilical area innervated by

A

T10

194
Q

Charcot joints

A

Result of cumulative damage from loss of reflexes and diminished pain awareness

Classically assoc with syphilis or diabetes

195
Q

Ulnar nerve supplies (motor)

A

C8-T1

All interosseus muscles - finger adduction + abduction

Lumbricals on ulnar metacarpals - extend digits

196
Q

Schistosomiasis

A

Use MRI to see lesions!

Eggs embolize to CNS

Itching of skin
Lancinating pains in legs + toes
Paraparesis
Loss bladder and bowel control
Unable to stand

S masoni is endemic to Puerto Rico + S. America
- embolizes to SC

S. japonicum also embolizes eggs

197
Q

Lateral Corticospinal tract decusates where?

A

At junction of medulla and SC

198
Q

Spinal claudication

A

IS NOT THE SAME AS LEG CLAUDICATIOn

Leg pains b/c shunt blood to leg muscles and pain happens due to ischemia of sensory neurons in SC

199
Q

Spondylolisthesis

A

Slippage of vertebral elements

200
Q

CSF findings w/ spinal cord infarction or cerebral infarction

A

Usually normal

Sometimes get elevated protein

201
Q

Achilles reflex

A

S1, 2

202
Q

Patellar reflex

A

L3, L4

203
Q

Biceps reflex

A

C5, C6

204
Q

Triceps reflex

A

C7, C8

205
Q

1 cause mononeuropathy multiplex

A

Diabetes mellitus

206
Q

Friedreich disease

  • what it affects
  • associations
A

Defect on chromosome 9

  • protein frataxin defect
  • AR defect - trinucleotide repeat

Affects:

  • Spinocerebellar
  • Dorsal columns
  • Lateral corticospinal tract
  • peripheral neuropathy w/ degeneration of dorsal root ganglia
Associations:
HOCM
Diabetes 
- can cause visual problems w/ hyperglycemia
Optic atrophy

Can see effects at juvenile period

207
Q

Parsonage-Turner syndrome

A

Acute brachial plexopathy

Acute onset pain in neck, shoulder, or upper arm

3-10 days later: weakness affecting proximal arm muscles
Sensory loss too

Cause unknown; can happen w/ vaccination or viral infection

Can have familial cases w/ AD pattern

208
Q

Riley Day disease

A

Familial dysautonomia

AR d/o usually in Jewish kids

Small fiber neuropathy affecting mylinated and unmyelinated small fibers
–> impair pain and temp sensation

SNS and PSNS also affected –> autonomic effects

  • no tears on crying
  • corneal ulceration
  • no pupillary reactivity
  • poor temp regulation
  • lots of perspiration
  • blood pressure control abnormality
  • dysphagia
  • recurrent vomiting
  • gastric and intestinal dilation

NO papillae of tongue

NO tx

209
Q

Chronic inflammatory demyelinating polyneuropathy (CIDP)

A

Like G-B but slower

Affects proximal portions of nerves where they exit SC @ root level
–> causes increase in protein –> inflammatory d/o –> demyelinates

Get paresis, sensory loss proximally and distally

210
Q

Porphyric polyneuropathy

A

assoc w/ AIP

GI paresis
Ab pain
Constipation

Psychosis

Axonal motor neuropathy

Autonomic instability

Seizures, SIADH

211
Q

Acute Wernicke lesions where?

A

Periaqueductal and mamillary bodies
- hemorrhagic necrosis

Get autonomic failure

212
Q

Botulinism electophsiologic testing results

A

Posttetanic potentiation of compound muscle A/P

  • decremental response of muscles to repetitive stim at low freq
  • incremental response to repetitive stim at high freq

Same profile as Lamber Eaton

213
Q

Acute disseminated encephalomyelitis vs MS

A

ADEM

  • demyelinates brain, brainstem, SC
  • monophasic –> only 1 occasion (vs recurrent MS)

MS
- recurrent

214
Q

When do emergency surgery w/ disk herniation?

A

If hurts cauda equina

- if see evolving focal motor deficit in legs (eg footdrop) + spihincter dysfunction –> surgery ASAP!

215
Q

Wernickes encephalopathy

A

Deteriorating level of consciousness
Autonomic disturbances
Ocular motor problems
Gait difficulty

Lethal HYPOTN
Hypothermia

216
Q

Head trauma + periorbital ecchymosis + echymosis over mastoid region + hemotympanum (blood behind eardrum)

A

Basilar skull fracture

217
Q

Diffuse axonal injury

A

Pathology:

diffusely spread axonal swellings of white matter, corpus callosum, upper brainstem that are usually hemorrhagic

218
Q

What should you do for urinary incontinence during status epilepticus?

A

Condom catheter

Keeps pt dry
allows collection urine
monitor I/Os

219
Q

Parotid surgery for parotid tumor can cause what kind of CN palsy?

A

Facial Nerve

220
Q

Acute shoulder pain after forceful abduction and external rotation - nerve injured? What happened?

A

Anterior dislocation of humerus

Axiallary N injured
Artery can be injured too

221
Q

Peripheral neuropathy classifications

A

Mononeuropathy

Mononeuropathy multiplex

Axonal polyneuropathy

Demyelinating polyneuropathy

222
Q

Mononeuropathy

  • distribution/pattern
  • examples
A

Focal

Ex:
Carpal tunnel
Bell palsy

223
Q

Mononeuropathy multiplex

  • distribution/pattern
  • examples
A

Asymmetric, multifocal (several noncontiguous nerves)

Ex:
Vasculitis
DM
Lymphoma
Amyloidosis
Sarcoidosis
Lyme
Acute HIV
Leprosy
224
Q

Axonal polyneuropathy

  • distribution/pattern
  • examples
A

Symmetric, distal
Mostly sensory

Ex:
EtOH
Drugs
Chronic arsenic exposure
DM
Uremia
B12 deficiency
Folate deficiency
HypoThyroid
Paraproteinemia
Paraneoplastic
chornic HIV
Charcot-Marie-Tooth
225
Q

Demyelinating polyneuropathy

  • distribution/pattern
  • examples
A

Symmetric, often PROXIMAL
Mostly motor
Ascending spread

Ex:
acute arsenic exposure
Guillain Barre
Chronic inflammatory demyelinating polyneuropathy
Paraprotenemia
226
Q

DDx dementia

A 
Alzheimer
CJD
Delirium
Dementia w/ Lewy bodies
Depression
Frontotemporal dementia (Picks disease)
Mild cognitive impairment
Normal pressure hydrocephalus
Vascular dementia
227
Q

Alzheimer’s Disease

A

Gradual memory loss
Preserved consciousness

Progression of disease:
aphasia
apraxia
agnosia
inattention
L-R confusion
228
Q

CJD

A

Rapid progression
Prominent myoclonus
EEG: triphasic sharp waves
CSF protein 14-3-3 has good specificity

Diffusion weighted MRI is more sensitive and specific

229
Q

Delirium

A

Altered level of alertness and attention often w/ globally impaired cognition

Abrupt onset

Fluctuating level of alertness common

230
Q

Dementia w/ Lewy Bodies

A

Early signs:
Mild parkinsonism
Hallucinations
Delusions

231
Q

Depression

A

SIGECAPS

Sleep changes
Interest lost
Guilty
Energy lack
Cognition/concentration reduced
Appetite decrease
Psychomotor retardation
Suicidal ideation
232
Q

Frontotemporal dementia

A

Picks disease is example

Often < 60 yo
Language difficulties
Behavioral disturbances
Impulsive
Aggressive
Apathetic

Diminished function in frontal and/or temporal lobes

233
Q

Normal Pressure hydrocephalus

A

Dementia
Gait ataxia
Urinary incontinence

Psychomotor slowing
Apathy

Can cure w/ ventriculoperitoneal shunting

234
Q

Vascular dementia

A

Loss of function may be temporally correlated w/ cerebrovascular events

May be assoc w/ silent strokes, CV risk factors

235
Q

EEG pattern of absence seizures

A

Generalized, symmetrical 3-Hz spike and wave activity on normal background

Finding from hyperventiation of pt during EEG

236
Q

Tx epidural hematoma

A

Emergent craniotomy

237
Q

Restless leg syndrome

A

4 cardinal sx:

Uncomfortable sensation or urge to move legs
Discomfort worsens in eve or sleep
Discomfort worse at rest
Discomfort relieved by mvmt of affected limbs

238
Q

Sx of heat stroke

A 
Temperature > 40C (105 F)
AMS
HYPOTn
Tachy
Tachypnea

Can get rhabdo

239
Q

How to assess for Delirium?

- 4 questions/things to find out

A

Need 1 & 2 + 3 or 4

1) Onset
- from family member
- is there evidence of acute change in MS from pt’s baseline?
- Did abnormal behavior fluctuate during day, or increase and decrease in severity?

2) Inattention
+ response to : Did pt have difficulty focusing attention?

3) Disorganized thinking
+ response to: Was pt’s thinking disorganized or incoherent (rambling, illogical, switch subject to subject)?

4) Altered level of consciousness
- Overall, how would you rate pts level of consciousness?
- alert
- hyperalert
- drowsy, easily aroused
- difficult to arouse
- unarousable

240
Q

CPP =

A

MAP - ICP

241
Q

Cushing reflex

A

TRIAD: Increased systolic, bradycardia, irregular respiration

Increase in ICP causes a decrease in CPP

SNS will first kick in - vasoconstrict to increase MAP and tachycardia

Baroreceptors in aortic arch will then detect increase in BP and activate PSNS via vagus
- Causes bradycardia

These all can cause increased P in brainstem –> affect respiratory centers –> apnea

242
Q

Cord compression dx

A

spinal MRI

243
Q

Charcot Marie Tooth disease

A

PNS disease, demyelinating

progressive loss of muscle tissue and touch sensation across various parts of the body

244
Q

How long can it take to see blood on CT without contrast?

A

24 hrs

245
Q

Peripheral demyelinating disorders in kids

A

Charcot Marie Tooth

Diptheria

246
Q

3 causes of subacute facial bilateral palsy

A

Sarcoid
Lyme
Guillain Barre

247
Q

1 cause of status epilepticus

A

Epileptics that don’t take meds

248
Q

Most likely dx: essential tremor

  • next dx step
  • next step in therapy
A

MRI of brain and spine

Primidone, propanolol

Can see cogwheel effect in ET vs cogwheel rigidity in PD

249
Q

Spinal muscular atrophy type I

A

Caused by excessive programmed cell death

Hypotonic and weak baby but appears alert
—this means it is periph rather than central defect
LMN defects
Pure motor disorder
Extra ocular muscles usually spared
Moro and tonic neck reflexes present
Tongue fibrillations common

Next step dx: molecular testing for SMN1 gene

Tx: supportive

250
Q

Remember these causes of stroke

A

Ischemia from atherosclerosis

Afib throwing clot emboli

Septic emboli from endocarditis

251
Q

Spasticity vs rigidity

A

Spasticity is velocity dependent
- if move arm faster, less spastic

Rigidity is NOT velocity dependent
- will get same rigidity w/ fast or slow mvmt

252
Q

MCA stroke of L hemi

A

Aphasia
Contralateral hemiparesis (right side)
Contralateral hemisensory loss

253
Q

MCA stroke of R hemi

A

If this is nondominant hemi,

Apraxia
Contralateral neglect
Confusion

Contralateral hemiparesis
Contralateral hemisensory loss

254
Q

ACA stroke

A

Contralateral lower extremity and face weakness (paresis)

Contralateral motor and/or sensory deficits more in the lower limb
Urinary incontinence
Gait apraxia
Primitive reflexes 
Abulia (lack of will/initiative)
Paratonic rigidity
255
Q

Vertebral/basilar stroke

A 
Ipsilateral
Ataxia
Diplopia
Dysphagia
Dysarthria
Vertigo
Contralateral
Homonymous hemianopia (PCA)
256
Q

PCA stroke

A

Contralateral Homonymous hemianopia

Alexia w/o agraphia (dominant hemi)
Visual hallucinations (Calcarine cortex)
Sensory sx (thalamus)
3rd nerve palsy w/ paresis of vertical eye mvmt
Motor deficits *cerebral peduncle, midbrain)
257
Q

Lacunar stroke

A

Internal capsule = pure motor hemiparesis

Thalamus = pure hemisensory loss
Also ataxic hemiparesis due to cerebrllar fibers through thalamus

Pons = dysarthria, clumsy hand

258
Q

Pupillary findings in ICH and corresponding level of involvement

A

Pinpoint pupils = pons

Poorly reactive pupils = thalamus

Dilated pupils = putamen

259
Q

CT scan of ischemic vs hemorrhagic stroke

A

Ischemic appears dark

Hemorrhagic looks white

260
Q

When is surgery good for ICH?

A

Cerebrllar hematomas

Usually not helpful most

261
Q

Shy Drager syndrome

A

Multiple system atrophy

Parkinsonian sx
autonomic insufficiency (hypoTN, abnormal sweating, impotence, gastroparesis, etc)
Widespread neuro signs (cerebellar, pyramidal or LMN)

ALWAYS CONSIDER when pt w/ parkinsonism experiences orthostatic hypotension or other autonomic sx

262
Q

Where are neurons lost in PD?

A

Substantia Nigra

Locus ceruleus

263
Q

Huntington pathology

A

Loss if GABA producing neurons in striatum

264
Q

Dx MS

A

Clinically
2 episodes of symptoms
2 white matter lesions on MRI

265
Q

Primary generalized Dystonia

A

GAG deletion of DYT1 (TOR1A gene)
No sensory loss

AD

Work up: MRI brain

Tx: DBS of globus pallidus, pars interna to stimulate

266
Q

What are lewy bodies made of and where do they deposit?

A

a-synuclein

Nucleus basalis of meynert
Locus ceruleus
dorsal raphe
dorsal motor nucleus of CN 10

267
Q

Does familial PD often have lewy bodies?

A

No!

But it is early onset

268
Q

Mimics PD

A

Multiple System atrophy
(shy Drager, striataonigral degeneration)

  • less tremor
  • symmetrical

Dementia w/ Lewy Bodies
- cognitive dysfunction

269
Q

What can you have impaired temp sensation in?

A

Riley Day disease

Spinocerebellar ataxia type 3

270
Q

Is parkinsonism of SCA responsive to levadopa?

A

Yes

271
Q

What on MRI is characteristic of SCA?

A

High T2 signal in cerebellum

272
Q

How to tell difference between T1 and T2 MRI

A

T1 - white matter is white, gray is dark

T2 - white matter is dark, gray is bright

273
Q

Anterior cerebral artery supplies

A

Medial frontal lobe

Anterior thalamus

Anterior limb of internal capsule

Caudate nucleus

Olfactory bulb and tract

274
Q

Middle cerebral artery supplies

A

Lateral cerebral hemispheres

Choroid plexus of lateral ventricles

Internal capsule

Some basal ganglia

275
Q

Posterior cerebral artery supplies

A

Thalamus

Posterior internal capsule

Occipital lobe

276
Q

Mechanism tardive dyskinesia

A

Unknown

Maybe cascade of responses develop in response to blockade of receptors by dopamine agonists

277
Q

What percent of ppl on dopamine receptor antagonists get TD?

A

1/3

278
Q

What’s characteristic of TD?

A

Spasms of back and neck

279
Q

How long to develop tardive dyskinesia?

A

1 month
If more acute, is acute Dystonia

Need to have for 3 months for dx

280
Q

Tardive dyskinesia due to?

A

Dopamine receptor blocking drugs

Not caused by dopamine depleting drugs

281
Q

Where is dopamine in basal ganglia?

A

Substantia Nigra pars compacta

282
Q

What is rate limiting step of dopamine synthesis?

A

Tyrosine hydroxylase

Converts tyrosine to dopa

283
Q

Parkinson genetics

A

Mutation in a-synuclein on chromosome 4q21

284
Q

CT vs MRI is better at what?

A

CT better for bone

MRI better to see soft tissue

285
Q

Sleep is mediated by

A

Serotonin

Norepinephrine

286
Q

Tx rem sleep behavior disorder

A

Clozapine

287
Q

Only drugs safe to use for antipsychotics in PD

A

Clozapine

Quietiapine

288
Q

What reflex still works in brain death?

A

Deep tendon

289
Q

What kind of CT do you get for blood? For abscess or tumor?

A

noncontrast = blood

abscess or tumor = contrast

290
Q

Narcolepsy pathophys

A

Get into REM sleep too fast

Sleep paralysis because wake up in REM sleep but still paralyzed

Orexin is decreased in narcolepsy (which usually helps in alertness)

291
Q

Cataplexy pathophys

A

Happens a lot in narcolepsy

In REM sleep, you are paralyzed so you don’t act out your dreams in REM. With narcolepsy, you get loss of muscle tone but you’re still awake because body wants to get into REM sleep.

292
Q

Rigidity vs. Spasticity –> how to tell difference?

A

Rigidity is LEAD PIPE rigidity. Still rigid for as long as you move it

Spasticity

  • flexors in arm and extensors in leg worse off
  • Clasp knife spasticity
293
Q

Bulbar vs. pseudobulbar dysarthria

A

Bulbar = LMN lesion for CN 12
- can happen in ALS (classic example)

Pseudobulbar dysarthria = UMN lesion for CN 12

294
Q

Density on non-contrast ct of blood, WM, bone, GM, CSF?

A

Bone > blood > WM, GM, CSF

295
Q

How big to aneurysms have to be to detect on CT?

A

> 5mm

296
Q

Tx meningioma

A

Resection

297
Q

Where are astrocytomas in adults?

A

NOT IN POSTERIOR FOSSA

298
Q

How to test for dysdiadochokinesia in cerebellar damage?

A

Rapid alternating movements (eg tap heel)

299
Q

What is the frequency on EEG of awake person?

A

8-13 Hz

300
Q

Lateral medullary syndrome

A

Wallenberg syndrome
Vertebral artery occlusion

DAMAGE:
CN5, 9, 10
Nucleus ambiguous
Lateral STT
Descending SNS fibers

PRESENTATION:
Ipsilateral ataxia
Ipsilateral horners

301
Q

1 cause of lobar hemorrhage in old person w/o HTN

A

Cerebral amyloid angiopathy

- deposit beta amyloid in blood vessel

302
Q

Mycotic aneurysm

A

Very small usually, can be missed on CT

Multiple aneurysms (not fungal etiology)

Occur w/ Gm +/- infections w/ low virulence

Mycotic aneurysms form over cerebral convexities w/ subacute bacterial endocarditis

Bleeding mostly in subarachnoid space

303
Q

Charcot Bouchard aneurysms

A

Small

In chronic HTN

Mostly in lenticulostriate arteries

Dentate nucleus very prone to forming these

304
Q

Hemangioblastomas are seen in?

A

Polycystic disease of kidney

von Hippel LIndau

305
Q

Leptomeningeal angiomas seen in?

A

Sturge Weber

306
Q

Transcortical motor aphasia

A

Like Brocas….

But repetition is ok

307
Q

Transcortical sensory aphasia

A

Like Wernickes…

But repetition is ok

308
Q

Mesial temporal sclerosis

A

closely related to temporal lobe epilepsy, a type of partial (focal) epilepsy in which the seizure initiation point can be identified within the temporal lobe of the brain.

Mesial temporal sclerosis is the loss of neurons and scarring of the temporal lobe associated with certain brain injuries.

309
Q

Jacksonian march

A

a phenomenon where simple partial seizure spread from distal part of limb to face ipsilaterally (on same side of body).

They involve a progression of the location of the seizure in the brain, which leads to a “march” of the motor presentation of symptoms

They are unique in that they travel through the primary motor cortex in succession, affecting the corresponding muscles, often beginning with the fingers. This is felt as a tingling sensation. It then affects the hand and moves on to more proximal areas on the same side of body.

310
Q

Where are the reticular neurons of the ascending reticular activating system? What is it for?

A

Between thalamus AND midbrain

for wakefulness

311
Q

Classification of concussion

A

grade 1 = no LOC, < 15 mins sx
grade 2 = no LOC, > 15 min sx
grade 3 = LOC

312
Q

How long is the amnesia usually for grade 3 concussion?

A

Proportional to duration of unconsciousness

313
Q

What imaging is more sensitive to diagnose acute ischemia?

A

MRI

314
Q

Apraxia

A

loss of ability to execute or carry out learned purposeful movement

315
Q

Agnosia

A

Loss of ability to recognize objects, persons, sounds, shapes, or smells

316
Q

Time to tx brain ischemia w/ thrombolytics

A

Within 4.5 hrs

317
Q

Common labfindings for SAH

A

Hyponatremia (increased ANP, or SIADH)

EKG - WT prolongation, T wave inversion, arrhythmias

318
Q

1 place for aneurysm in ADPKD

A

Anterior communicating

319
Q

Best way to evaluate hyperacute onset of hemiparesis ~ 1 wk after SAH incident

A

Transcranial doppler

  • measures velocity, finding caused by vasospasm

Angiography usually needed to confirm

320
Q

HTN Hx + unilateral weakness + no changes on CT

A

Lacunar infarct

microatheroma and lipohyalinosis

Most commonly in internal capsule

321
Q

1, #2 muscles involved for myasthenia gravis

A

1 - extraocular muscles

2 - muscles of jaw (bulbar muscles)

CPK usually normal in myasthenia gravis

322
Q

Myasthenia gravis vs. primary muscle problem - what lab value is helpful to distinguish?

A

CPK normal in MG

323
Q

Myasthenia gravis vs. ALS

A

normal reflexes in MG

324
Q

What test do you use to evaluate spinal stenosis?

A

MRI

325
Q

Electrolyte risk with immobilization (eg paralysis)

A

Hypercalcemia

  • possibly due to increased osteoclastic bone resorption
  • tx w/ hydration + bisphosphonates
326
Q

Medial medullary syndrome

  • artery responsible
  • syndrome
A

Vertebral A
ASA

Ipsilateral
paralysis + fasciculations of tongue

Contralateral
paralysis of arm and leg
loss of tactile, vibratory and position sense

Deviation of tongue to injured side

327
Q

Wallenberg syndrome

  • Injured artery
  • syndrome
A

PICA
Vertebral A

Lateral medulla injury

Contralateral
:loss of pain and temperature sensation on body

Ipsilateral:
Loss of pain and temp of face
Horner

Dysphagia
Hoarseness
Deviation of uvula to contralateral side
Loss of gag reflex

cerebellar ataxia
Nystagmus

328
Q

Internal carotid stroke

A

MCA stroke + amaurosis fugax

329
Q

Proximal weakness of lambert eaton vs. polymyositis

A

Both have proximal weakness

CPK high in polymyositis
CPK normal in LE

Polymyositis - anti Jo1 and ANA
Lambert eaton - anti voltage gated Ca channels

330
Q

Cavernous sinus thrombosis

  • symptoms
  • how do you tell the difference w/ orbital cellulitus?
A

CST happens b/c facial/ophthalmic venous sys is valveless

Headache
Binocular palsies
Periorbital edema
Hypoesthesia or hyperesthesia in V1/V2 distribution
CAN BECOME BILATERAL

Dx w/ magnetic resonance venography

Orbital cellulitus DOES NOT have headache, bilateral cranial nerve findings, or bilateral periorobital edema

331
Q

Progressive supranuclear palsy

A

Degenerative condition of

  • brainstem
  • basal ganglia
  • cerebellum

Like parkinson’s:

  • bradykinesia
  • limb rigidity
  • cognitive decline

NOT like parkinson’s

  • no tremor
  • ophthalmoplegia
332
Q

Herniated disc

A

Low back pain sciatica presentation
- radiates to thighs adn below knee

Pain worsens w/ sitting

+ straight leg test

333
Q

Spinal stenosis

A

Pain is posture-dependent

Flexion of spine = widening of canal
Extension - narrowing of canal

Pain exacerbated by standing still, walking
Pain improved by sitting, lying down

Normal arterial pulses
Straight leg test negative

Dx w/ MRI

Tx laminectomy

334
Q

MS HLA?

A

HLA DR2

50% are this

335
Q

Neurons in tuberoinfundibular pathway secrete what?

A

Dopamine

336
Q

What path is nigostriatal path?

A

Substantia nigra –> basal ganglia

337
Q

Lateral pontine stroke

  • artery responsible
  • symptoms
A

AICA

Cerebellar:
Ataxia, nystagmus

Ipsilateral 
deafness, tinnitus
loss of pain and temp on face
Horners
facial weakness, dysarthria

Contralateral
loss of pain and temp on body

Jaw weakness, dysarthria

Vertigo, nystagmus, ataxia

338
Q

Medial pontine stroke

  • artery responsible
  • symptoms
A

Basilar A

Contralateral
hemiparesis – body and face; dysarthria
Ataxia (usually contralateral)
loss of touch, vibration and position sense

Ipsilateral
face weakness, dysarthria
horizontal gaze palsy, diplopia
Horners

INO

339
Q

Midbrain stroke

  • artery responsible
  • symptoms
A

PCA

Contralateral hemiparesis – body and face; dysarthria

Ipsilateral CN III nerve palsy (down and out)

340
Q

What tumors have periventricular structure predilection?

A

Tuberous sclerosis periventricular tubers

Primary brain lymphoma

341
Q

Subclavian steal syndrome

A

Stenosis of subclavian artery proximal to vertebral artery (before it branches off)

Exercise of ipsilateral arm causes blood to flow in reverse down vertebral artery to fill subclavian distal to block

Decrease cerebral blood flow
BP in left arm less than right arm
Upper extremity claudication

Tx with surgical bypass

342
Q

Astrocytomas in cerebellum are usually…

A

cystic

343
Q

Absence vs complex partial seizure

A

Absence seizure does NOT have:
- post ictal state

Absence seizure CAN be stimulated by hyperventilation (vs not in complex partial) – makes a 3Hz spike and wave pattern on normal background

344
Q

Cluster headache

A

Unilateral retrorobital pain starting suddenly

Redness of ipsilateral eye
tearing, stuffed or runny nose
ipsilateral horners

345
Q

Transtentorial (uncal) herniation signs

A

Compress:

Contralateral crus cerebri —–> ipsilateral hemiparesis

Ipsilateral oculomotor N ——-> mydriasis, down and out, ptosis

Ipsilateral posterior cerebral A ————> ischemic visual cortex ———> contralateral homonymous hemianopia

Reticular formation ————-> Alt mental consciousness, coma

346
Q

Can you expect fever in any intracranial hemorrhage?

A

YES!

347
Q

Sudden onset of vertigo, vomitting, occipital headache in person w/ HTN

A

Cerebellar hemorrhage

348
Q

What do you do to monitor respiratory fxn in person w/ Guillain Barre?

A

Serial vital capacity measurements

349
Q

What is a pt in tonic clonic seizure at risk for?

A

Cortical laminar necrosis

  • hallmark of prolonged seizures
  • can lead to persistent neuro deficits and more seizures

Can see on MRI

350
Q

Basal ganglia hemorrhage - neuro findings

A

Hemiplegia
Hemisensory loss

Homonymous hemianopia
gaze palsy

Stupor, coma

351
Q

Cerebellum hemorrhage - neuro findings

A

Neck stiffness
Facial weakness
NO hemiparesis
Stupor/coma from brainstem herniation

352
Q

Thalamus hemorrhage - neuro findings

A

Hemiparesis
Hemisensory loss

Upgaze palsy
Nonreactive miotic pupils
Eyes deviate Towards hemiparesis

353
Q

Cerebral lobe hemorrhage - neuro findings

A

Can be assoc w/ seizures

Contralateral homonymous hemianopia (occipital)
Contralateral plegia/paresis (frontal)
Contralateral hemiparesis (parietal)
Eyes deviated AWAY from hemiparesis

354
Q

Pons hemorrhage - neuro findings

A

Deep coma, total paralysis w/in minutes

Pinpoint reactive pupils

355
Q

Hypertensive lacunar stroke mostly in…

A

Lacunar arteries

Putamen (#1)
Thalamus
Pons
Cerebellum (more HTN hemorrhage!!!!! vs stroke)
Cortex
356
Q

What test can detect endogenous depression?

A

Dexamethasone suppression test

357
Q

Dx alzheimer’s disease

A

Made clinically by assessing:

MMSE
Neurophysch testing

2 or more of the following:
2 or more areas of cognitive deficits
Progressively worsening memory and other cognitive function
no disturbance of consciousness
onset after age 60
absence of other systemic or neurologic disorder causnig progressive cognitive defects

358
Q

Duret hemorrhages

A

are small areas of bleeding in the ventral and paramedian parts of the upper brainstem, (midbrain and pons)

They are secondary to raised intracranial pressure with formation of a transtentorial pressure cone involving the cerebral peduncles (crus cerebri) and other midbrain structures caused by raised pressure above the tentorium

359
Q

Diffuse axonal injury

A

is the result of traumatic shearing forces that occur when the head is rapidly accelerated or decelerated, as may occur in auto accidents, falls, and assaults

Can also be due to metabolic causes

Only edema will cause diffuse axonal injury (esp in metabolic)

360
Q

Areas of brain most susceptible to hypoxic injury

A

Hippocampus
Purkinje cells of cerebellum
4th layer of cortex

361
Q

Dystrophic calcification

A

Cells are dying and will uptake calcium

362
Q

Primary generalized seizures

A
  • will induce w/ sleep deprivation, hyperventillation
  • –usually induce the seizures so you can figure out how much restriction to put on a kid’s life (like in absence)
  • runs in families
  • starts in reticular activating system and spreads
  • usually less than 30s (vs > 40s in partial)
  • only motor activity impaired
363
Q

When does teratogenicity dose of valproic acid start at?

A

750 mg

364
Q

Is febrile seizure genetic?

A

Somewhat!

Always put these children w/ FH of febrile seizures on Tylenol to lower fever

20% Japanese can have febrile seizures

365
Q

Do ppl who have acidosis seize?

A

No!

Moment you are aciddic, you will stop seizing

366
Q

Resons for intraprenchymal hemorrhage

A

HTN
Trauma
Aneurysm/AVM
Malignancy, hemorrhage

367
Q

Internal capsule - where are the motor fibers?

A

Genu - face and arm

Posterior capsule - more leg

368
Q

How can you tell the central sulcus on MRI?

A

Omega sign

369
Q

1 type seizure in adults

A

Complex partial

370
Q

Which muscles are affected more in UMN lesions?

A

A pattern of weakness in the flexors (lower limbs) or extensors (upper limbs), is known as ‘pyramidal weakness’

371
Q

What does extensor posturing mean?

A

Lesion above red nucleus

372
Q

Meralgia paraesthetica

A

aka Bernhardt-Roth syndrome

numbness or pain in the outer thigh not caused by injury to the thigh, but by injury to a nerve that extends from the thigh to the spinal column

involves only 1 nerve: lateral cutaneous nerve of thigh

The lateral femoral cutaneous nerve most often becomes injured by entrapment or compression where it passes between the upper front hip bone (ilium) and the inguinal ligament near the attachment at the anterior superior iliac spine

373
Q

Complex Regional Pain Syndrome (CRPS), formerly Reflex Sympathetic Dystrophy (RSD)

A

Clinical features of CRPS have been found to be neurogenic inflammation, nociceptive sensitisation, vasomotor dysfunction, and maladaptive neuroplasticity

374
Q

What is first sign of weakness on PE?

A

Pronator drift!

The very first sign is extensor digiti minimi sign

375
Q

What are most common seizures 2/2 to EtOH?

A

Trauma-induced

Withdraw

376
Q

What is the following typical of?
Occipital lobe hypometabolism

Medial temporal lobe atrophy and parietal hypometabolism

Atrophy of midbrain

A

Occipital lobe hypometabolism is most typical of DL

Medial temporal lobe atrophy and parietal hypometabolism are characteristic if AD

Atrophy of midbrain is characteristic of progressive supra nuclear palsy

377
Q

Top 3 places for hypertension stroke

A

Basal ganglia
Cerebellum
Pons

378
Q

Number 1 cause of sixth nerve palsy

A

Diabetes

379
Q

What is first nerve affected by increased intracranial pressure?

A

CN 6 since it has the longest course.

380
Q

Intracranial HTN symptoms

A 
Diffuse HA worse in AM
N/V in AM
vision changes
Papilledema
CN deficits
Somnolenece
Confusion
Unsteadiness
Cushings reflex

Evaluate via CT or MRI

Neuro (3 decks)

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