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MTB 1 Flashcards

1
Q

Tuberous sclerosis presentation?

A

Neuro: seizures, psychomotor retardation, mental deterioration
Skin: Adenoma sebaceum, shagreen patches, ash leaf patches
Retinal lesions
Cardiac rhabdomyomas

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2
Q

What do skin lesions with tuberous sclerosis look like?

A

Adenoma sebaceum - reddened facial nodules
shagreen patches - leathery plaques on trunk
ash leaf patches - hypopigmented patches

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3
Q

How does McCune-Albright present?

A

3 P’s:
Precocious puberty
Pigmentation - large, irregular cafe au lait spots in axillary/genital area
Polyostotic fibrous dysplasia - abnormal bone formation that is weak and deformable

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4
Q

What dz’s is McCune Albright ass’d with

A

Hyperthryoidism
GH secreting tumors
Adrenal hypercortisolism

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5
Q

What are neurofibromas?

A

Soft, flsh colored lesions attached to peripheral nerves

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6
Q

Type I Neurofibromatosis (Von Recklinghausen)?

A

Neurofibromas
CN tumor - 8
Cafe au lait spots = hyperpigmented
Meningiomas and gliomas

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7
Q

Tx for Type I Neurofibromatosis?

A

None.

8th CN lesions - surgical decompression to preserve hearing

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8
Q

Type II Neurofibromatosis?

A

BL Acoustic neuromas - hearing loss

Schwanommas

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9
Q

Sturge Webe Syndrome Presentation

A

Seizures
Port wine stain - face
CNS: homonymous hemianopsia

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10
Q

What is seen on skull xray with Sturge Webe Syndrome?

A

Calcification of angiomas

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11
Q

What is the Tx for essential tremor?

A
  1. Propranolol
  2. Primidone, Alprazolam, Clozapine
  3. Ineffective -> Thalatomy
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12
Q

Gait disturbance + Head trauma from boxing?

A

Parkinson Dz

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13
Q

Drugs that cause Parkinson?

A

Antipsychotics
Resperine
Metoclopromide

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14
Q

MOA of atypical AP’s?

A

COZ RAQ

Block dopamine and serotonin

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15
Q

What is the tremor like in PD?

A

pronation/supination tremor

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16
Q

What is Shy Drager syndrome?

A 
Multiple system atrophy
PD + orthostasis 
1. Parkinsonism
2. Autonomic Dysfnc - postural HypoTN, Impotence, Incontinence, Abnormal salivation, lacrimation gastroparesis
3. Widespread neuro si's
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17
Q

What is the Tx for Shy Drager syndrome?

A

Intravascular volume expansion
Fludrocortisone
Salt supplementation
A-Agonists

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18
Q

MOA of Typical AP’s?

A

Block D2 receptors only

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19
Q

Facial expression and writing in PD?

A

Hypomimia - limited

Micrographia - small writing

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20
Q

What is the tx for mild PD (intact functional status)?

A

Anticholinergics: Benztropine, trihexyphenidyl = relieve tremor and rigity

Amantidine: pts >60 intolerant of anticholinergics

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21
Q

AE’s of Anticholinergics?

A 
Dry mouth
Worsening of prostate hypertrophy
Constipation
N/V
Blurred vision
Urinary retention
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22
Q

When are anticholinergics CI?

A

BPH

Glaucoma

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23
Q

PD tx for severe dz (inability to care for self, orthostasis)?

A
  1. Dopamine Agonist: Pramipexole and ropinirole
    Bromocriptine, cabergoline - older, used less b/c of AE’s
  2. Levodopa/Carbidopa - “on-off” phenomenon
  3. COMT inhibitors - Tolcapone, entacapone
  4. MAO Inhibitors - Rasalgiline, selegiline
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24
Q

AE’s of Levodopa/Carbidopa

A

Hallucinations
Somnolence
Confusion
Dyskinesia

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25
Q

AEs of bromocriptine?

A

Cardiotoxic

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26
Q

Which drugs are adjuncts to levodopa/carbidopa?

A

COMT inhibitors

MAOIs

27
Q

How do COMT inhibitors work?

A

Extend duration of L/C by blocking metabolism of dopamine

28
Q

AE’s of COMT inhibitors?

A 
Dyskinesia
Hallucinations
Confusion
Nausea
Orthostatic HypoTN
29
Q

How do MAOIs work?

A

Block metabolism of dopamine

Potential to slow dz progression

30
Q

AEs of MAOIs?

A

Insomnia

Confusion

31
Q

Pt w severe PD comes to ED for psychosis and confusion. He is on L/C, ropinirole, tolcapone. Next step?

A

Start Clozapine - AP w/fewest EPS AE’s

Severe PD cannot stop meds b/c of ‘locked in’ possibility

32
Q

Tx for spasticity?

A

Baclofen
Dantrolene
Tizanidine

33
Q

Tx for Restless Leg Syndrome?

A

Dopamine agonists: Pramipexole

Ropinirole

34
Q

Tx for dyskinesia in Huntington dz?

A

Tetrabenazine - VMAT inhibitor

35
Q

Tx for psychosis in Huntington dz?

A

Haloperidol

Quetiapine

36
Q

CT findings in Huntingtons?

A

Early - cerebral atrophy

Late - caudate atrophy = box car ventricles

37
Q

What do we see in Creutzfeld-Jakob Dz?

A 
Myoclonus, sharp triphasic synchronous discharges on EEG, positive 14-3-3 CSF Assay
spongiform encephalopathy - prion dz
2 of 4 to Dx: 
1. myoclonus
2. Akinetic 
3. Cerebellar/visual disturbance
4. Pyramidal/EPS dysfnc
38
Q

How to dx Creutzfeld-Jakob Dz?

A

Gold std: Brain Bx = spongiform changes

or demonstrate PRNP gene mutation w/ genetic testing

39
Q

Tx for Tourettes?

A

Haloperidol
Clonazepam
Pimozide

40
Q

Multiple Sclerosis etiology

A

White woman
Colder climates - Northern European
CNS white matter disorder

41
Q

MC presentation of MS?

A

Blurry vision/visual disturbance - optic neuritis

42
Q

Characteristic eye abnormality in MS?

A

Internuclear Opthalmoplegia (INO) from MLF demyelination

43
Q

Best initial test in MS?

A

MRI w/GAD brain and spine

44
Q

Most accurate test in MS?

A

MRI w/GAD brain and spine

45
Q

LP of MS?

A

CSF w mild elevation in protein
<50-100 WBCs
Oligoclonal bands

46
Q

Best initial tx for MS?

A

High dose steroids

500-1000 mg IV Methylprednisone 7 days in exacerbation

47
Q

Drugs to prevent MS relapse/progression?

A 
Glatiramer
B-IFN
- Must stop both in pregnancy
MTX
Natalizumab
Azathioprine
Cyclophosphamide
48
Q

MS tx in severe dz that fails to respond to steroids?

A

Plasma exchange

49
Q

MS pt develops worsening neuro deficits while on meds. MRI = new, multiple, white matter hypodense lesions. What is the cause?

A

Natalizumab

Ass’d with PML

50
Q

ALS dx?

A

Upper and motor neurons lost

Difficulty chewing, swallowing, decreased gag reflex, weak cough

51
Q

Are spinchters affected in ALS?

A

No. they are spared.

52
Q

How is ALS diagnosed?

A

EMG
- loss of neural innervation = fibrillation potentials in multiple muscles of multiple extremities
Increased CPK

53
Q

Tx for ALS?

A

Riluzole - reduces glutamate buildup
Baclofen, Tizanidine - for spasticity
CPAP/BiPAP - respiratory difficulty
Tracheostomy and Ventilator - advanced dz

54
Q

What is Charcot-Marie-Tooth Dz?

A

Genetic loss of both motor and sensory innervation

55
Q

How do pts present with Charcot-Marie-Tooth Dz?

A 
Distal weakness and sensory loss
Wasting in legs - look like inverted Champagne bottles
Decreased DTRs
Tremor
High arch = Pes Cavus
56
Q

Most accurate test and tx for Charcot-Marie-Tooth Dz?

57
Q

Spinal cord lesion of upper thoracic presentation?

A

Paraplegia
Bladder and bowel incontinence
Absent sensation from nipple downward

58
Q

Spinal cord lesion of lower thoracic presentation?

A

Absent sensation from umbilicus downward

59
Q

Supratentoriral lesion?

A

Hemiparesis

60
Q

Lesion at posterior column?

61
Q

Otitis media and mastoiditis can spread to what brain area?

A

Direct spread to temporal lobe and cerebellum

62
Q

Frontal and ethmoid sinuses can affect what area of brain?

A

Direct spread to frontal lobe

63
Q

Dental Infxn can affect what area of brain?

A

Direct spread to frontal lobe

64
Q

Bacteremia, cyanotic heart disease can affect what area of brain?

A

Hematogenous spread to form multiple abscesses along grey/white junction (MCA)

Neuro (3 decks)

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