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5 CNS: Neurocut-Misc

Step Up to Medicine, Chap 5: Neurocutaneous and Miscellaneous Syndromes

QuestionAnswer
AD dz characterized by cafe au lait spots, neurofibromas, and CNS tumors (glios, meningiomas), axillary or inguinal freckling, iris hamartomas (Lisch nodules), and bony lesions. Neurofibromatosis type I (von Recklinghausen's disease)
Complications of neurofibromatosis type I? Scoliosis, pheos, optic n gliomas, renal a stenosis, and erosive bone defects.
AD dz with multiple meningiomas, cafe au lait spots, neurofibromas, cataracts, and bilateral acoustic neuromas. Neurofibromatosis, type II
AD cognitive impairment, epilepsy, an dskin lesions. May also have retinal hamartomas, renal angiomyolipomas, and rhabdomyomas of heart. Tuberous sclerosis
Acquired dz with capillary angiomatoses of pia mater. Classic feature is port wine stain (facial vascular nevi). Epilepsy and MR also present. Sturge-Weber Syndrome
AD dz with cavernous hemangiomas of brain/brainstem, renal angiomas, and cysts in multiple organs. Malignant association? Von Hippel-Lindau Disease. Assoc'd with renal cell CA
Central cavitation of the cervical cord due to abnormal collection of the fluid within the spinal cord parenchyma. Syringomyelia
Presentation of syringomyelia? Bilateral loss of pain and temp sensation over shoulders in a cape-like distribution with preservation of touch.
Diagnosis and tx of syringomyelia? MRI. Tx is surgical: syringosubarachnoid shunt
Spinal cord hemisection usually at level of spinal cord 2/2 trauma, tumor, or abscess. Brown-Sequard syndrome
Presentation of Brown-Sequard? Contralateral loss of pain and temperature, ipsilateral hemiparesis, and ipsilateral loss of position/vibration sense.
Clinical features include lower extremity weakness or paralysis, back pain, sensory deficits below level of lesion, and sphincter disturbance (especially urinary retention). Dz? Transverse myelitis. Affects tracts across horizontal aspect of spinal cord at given level, usually thoracic spine.
Vertical nystagmus suggests which type of vertigo? Central
What is the progression of hearing loss in Meniere's disease? Eventually becomes permanent
Name a class of abx that is very ototoxic. Aminoglycosides
Which demyelinating disease is associated with central vertigo? MS
Which test should be ordered on all pts presenting with syncope? EKG
Which test should be ordered if syncope episodes are recurrent and unexplained and there is no evidence of underlying heart disease? Table tilt test
Difference in level of consciousness between simple partial, complex partial, and generalized seizures? Simple: intact Complex: impaired Generalized: LOC
Difference between course in absence (petit mal) seizures and complex partial seizures? Absence (petit mal) seizures tend to disappear in adulthood whereas complex partial do not.
Which lab values should be checked immediately in an unfamiliar seizing pt? Serum calcium, serum sodium, serum glucose, and BUN.
Tx for chronic seizures? Give anticonvulsant for at least 2 years. If pt remains seizure-free, taper dose cautiously. Confirm with lack of seizure activity on EEG.
Drugs of choice for generalized tonic clonic seizures and partial seizures? Phenytoin and carbamazepine. Can also use phenobarbital, valproate, and primidone.
Drug of choice for petit mal (absence) seizures? Ethosuximide and valproic acid.
Management of pt in status epilepticus? Establish airway, give IV diazepam, IV phenytoin, and 50mg dextrose. Treat resistant cases with IV phenobarbital.
What drug may delay death in ALS/Lou Gehrig's disease by 3-5 months? What is its mechanism of action? Riluzole. Glutamate-blocking agent.
MCC of aphasia? Stroke
Name that aphasia!: impaired comprehension of written or spoken language. Speech is grammatically correct and fluid, but doesn't make sense. Wernicke's (word salad) aphasia
Name that aphasia!: slow speech requiring effort. Few words with no grammatical composition. Good comprehension of written and spoken language. Broca's (broken tongue) aphasia
Name that aphasia!: disturbance in repetition Conduction aphasia (lesion btwn Broca's and Wernicke's areas)
Name that aphasia!: disturbances in all areas of language f'n (comprehension, speaking, reading, fluency) Global aphasia
Where is the lesion in relation to the central sulcus?: fluent aphasia posterior
Where is the lesion in relation to the central sulcus?: non-fluent aphasia anterior
Common preceding event for Bell's palsy (CN 7)? Upper respiratory infection
What diagnosis should you consider before treating Bell's palsy with steroids? Lyme disease (if in endemic areas). Do NOT give steroids if you suspect Lyme disease!
Tx for Bell's palsy? Usually resolves on its own in 1 month. May give short course of prednisone (NOT with Lyme disease tho) or acyclovir if necessary
Tx for trigeminal neuralgia (tic douloreux)? Carbamazepine is drug of choice. Other choices include baclofen and phenytoin, either alone or in combination with carbamazepine.
Created by: sarah3148