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5 CNS: Neurocut-Misc
Step Up to Medicine, Chap 5: Neurocutaneous and Miscellaneous Syndromes
| Question | Answer |
|---|---|
| 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
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