MTB 1 Flashcards by Nadia Mahmud

Tuberous sclerosis presentation?

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

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What do skin lesions with tuberous sclerosis look like?

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

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How does McCune-Albright present?

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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What dz’s is McCune Albright ass’d with

Hyperthryoidism
GH secreting tumors
Adrenal hypercortisolism

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What are neurofibromas?

Soft, flsh colored lesions attached to peripheral nerves

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Type I Neurofibromatosis (Von Recklinghausen)?

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

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Tx for Type I Neurofibromatosis?

None.

8th CN lesions - surgical decompression to preserve hearing

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Type II Neurofibromatosis?

BL Acoustic neuromas - hearing loss

Schwanommas

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Sturge Webe Syndrome Presentation

Seizures
Port wine stain - face
CNS: homonymous hemianopsia

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What is seen on skull xray with Sturge Webe Syndrome?

Calcification of angiomas

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What is the Tx for essential tremor?

Propranolol
Primidone, Alprazolam, Clozapine
Ineffective -> Thalatomy

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Gait disturbance + Head trauma from boxing?

Parkinson Dz

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Drugs that cause Parkinson?

Antipsychotics
Resperine
Metoclopromide

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MOA of atypical AP’s?

COZ RAQ

Block dopamine and serotonin

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What is the tremor like in PD?

pronation/supination tremor

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What is Shy Drager syndrome?

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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What is the Tx for Shy Drager syndrome?

Intravascular volume expansion
Fludrocortisone
Salt supplementation
A-Agonists

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MOA of Typical AP’s?

Block D2 receptors only

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Facial expression and writing in PD?

Hypomimia - limited

Micrographia - small writing

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

Anticholinergics: Benztropine, trihexyphenidyl = relieve tremor and rigity

Amantidine: pts >60 intolerant of anticholinergics

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AE’s of Anticholinergics?

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

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When are anticholinergics CI?

BPH

Glaucoma

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

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

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AE’s of Levodopa/Carbidopa

Hallucinations
Somnolence
Confusion
Dyskinesia

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AEs of bromocriptine?

Cardiotoxic

Which drugs are adjuncts to levodopa/carbidopa?

COMT inhibitors | MAOIs

How do COMT inhibitors work?

Extend duration of L/C by blocking metabolism of dopamine

AE's of COMT inhibitors?

``` Dyskinesia Hallucinations Confusion Nausea Orthostatic HypoTN ```

How do MAOIs work?

Block metabolism of dopamine | Potential to slow dz progression

AEs of MAOIs?

Insomnia | Confusion

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

Start Clozapine - AP w/fewest EPS AE's | Severe PD cannot stop meds b/c of 'locked in' possibility

Tx for spasticity?

Baclofen Dantrolene Tizanidine

Tx for Restless Leg Syndrome?

Dopamine agonists: Pramipexole | Ropinirole

Tx for dyskinesia in Huntington dz?

Tetrabenazine - VMAT inhibitor

Tx for psychosis in Huntington dz?

Haloperidol | Quetiapine

CT findings in Huntingtons?

Early - cerebral atrophy | Late - caudate atrophy = box car ventricles

What do we see in Creutzfeld-Jakob Dz?

``` 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 ```

How to dx Creutzfeld-Jakob Dz?

Gold std: Brain Bx = spongiform changes | or demonstrate PRNP gene mutation w/ genetic testing

Tx for Tourettes?

Haloperidol Clonazepam Pimozide

Multiple Sclerosis etiology

White woman Colder climates - Northern European CNS white matter disorder

MC presentation of MS?

Blurry vision/visual disturbance - optic neuritis

Characteristic eye abnormality in MS?

Internuclear Opthalmoplegia (INO) from MLF demyelination

Best initial test in MS?

MRI w/GAD brain and spine

Most accurate test in MS?

MRI w/GAD brain and spine

LP of MS?

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

Best initial tx for MS?

High dose steroids | 500-1000 mg IV Methylprednisone 7 days in exacerbation

Drugs to prevent MS relapse/progression?

``` Glatiramer B-IFN - Must stop both in pregnancy MTX Natalizumab Azathioprine Cyclophosphamide ```

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

Plasma exchange

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

Natalizumab | Ass'd with PML

ALS dx?

Upper and motor neurons lost | Difficulty chewing, swallowing, decreased gag reflex, weak cough

Are spinchters affected in ALS?

No. they are spared.

How is ALS diagnosed?

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

Tx for ALS?

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

What is Charcot-Marie-Tooth Dz?

Genetic loss of both motor and sensory innervation

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

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

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

EMG | No tx

Spinal cord lesion of upper thoracic presentation?

Paraplegia Bladder and bowel incontinence Absent sensation from nipple downward

Spinal cord lesion of lower thoracic presentation?

Absent sensation from umbilicus downward

Supratentoriral lesion?

Hemiparesis

Lesion at posterior column?

Ataxia

Otitis media and mastoiditis can spread to what brain area?

Direct spread to temporal lobe and cerebellum

Frontal and ethmoid sinuses can affect what area of brain?

Direct spread to frontal lobe

Dental Infxn can affect what area of brain?

Direct spread to frontal lobe

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

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

MTB 1 Flashcards

(64 cards)