MTB 1 Flashcards
Tuberous sclerosis presentation?
Neuro: seizures, psychomotor retardation, mental deterioration
Skin: Adenoma sebaceum, shagreen patches, ash leaf patches
Retinal lesions
Cardiac rhabdomyomas
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
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
What dz’s is McCune Albright ass’d with
Hyperthryoidism
GH secreting tumors
Adrenal hypercortisolism
What are neurofibromas?
Soft, flsh colored lesions attached to peripheral nerves
Type I Neurofibromatosis (Von Recklinghausen)?
Neurofibromas
CN tumor - 8
Cafe au lait spots = hyperpigmented
Meningiomas and gliomas
Tx for Type I Neurofibromatosis?
None.
8th CN lesions - surgical decompression to preserve hearing
Type II Neurofibromatosis?
BL Acoustic neuromas - hearing loss
Schwanommas
Sturge Webe Syndrome Presentation
Seizures
Port wine stain - face
CNS: homonymous hemianopsia
What is seen on skull xray with Sturge Webe Syndrome?
Calcification of angiomas
What is the Tx for essential tremor?
- Propranolol
- Primidone, Alprazolam, Clozapine
- Ineffective -> Thalatomy
Gait disturbance + Head trauma from boxing?
Parkinson Dz
Drugs that cause Parkinson?
Antipsychotics
Resperine
Metoclopromide
MOA of atypical AP’s?
COZ RAQ
Block dopamine and serotonin
What is the tremor like in PD?
pronation/supination tremor
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
What is the Tx for Shy Drager syndrome?
Intravascular volume expansion
Fludrocortisone
Salt supplementation
A-Agonists
MOA of Typical AP’s?
Block D2 receptors only
Facial expression and writing in PD?
Hypomimia - limited
Micrographia - small writing
What is the tx for mild PD (intact functional status)?
Anticholinergics: Benztropine, trihexyphenidyl = relieve tremor and rigity
Amantidine: pts >60 intolerant of anticholinergics
AE’s of Anticholinergics?
Dry mouth Worsening of prostate hypertrophy Constipation N/V Blurred vision Urinary retention
When are anticholinergics CI?
BPH
Glaucoma
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
AE’s of Levodopa/Carbidopa
Hallucinations
Somnolence
Confusion
Dyskinesia
AEs of bromocriptine?
Cardiotoxic