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5 CNS: Demy-Neuromus

Step Up to Medicine, Chap 5: Demyelinating Dz, Neoplasms, Trauma, Neuromusc Dz

Clinical definition of definite MS? 2 episodes of sx (transient sensory deficits, fatigue, weakness/spasticity, visual disturbances, incontinence, neuropathic pain) + evidence of 2 white matter lesions (imaging or clinical)
Lab-supported definition of definite MS? 2 episodes of sx + evidence of 1 white matter lesion on MRI + abnormal CSF (oligoclonal IgG bands in CSF)
Definition of probable MS? 2 episodes of sx + 1 white matter lesion or oligoclonal IgG bands in CSF
Classic location of demyelination (plaques) in MS? Angles of lateral ventricles
What is internuclear ophthalmoplegia? A lesion in which area leads to this deficit? ipsilateral medial rectus palsy (unable to adduct eye on lateral gaza) and horizontal nystagmus of abducting eye (contralateral to side of lesion); lesion is in medial longitudinal fasciculous
Tx for acute MS attacks? High dose IV corticosteroids. Tx does not alter course, however.
Disease modifying therapy for MS? Interferon therapy (start early in course b/f disability becomes irreversible) and immunosuppressives like cyclophosphamide (reserved for rapidly progressive dz due to toxic SE)
Symptomatic therapy for MS? Baclofen for m spasticity and carbamazepine or gabapentin for neuropathic pain
Guillan Barre is typically preceded by what type of event? A viral or mycoplasma infection of the upper respiratory or GI tract
Usual 3 causes of death in Guillan Barre? Respiratory failure, pneumonia, or arrhythmias
How do you diagnose Guillan Barre? CSF analysis shows elevated protein with normal cell ct and EMG shows decreased motor nerve conduction velocity. If suspected, ADMIT PT IMMEDIATELY for observation.
Tx for Guillan Barre? Which drug should never be given? Tx: IV immunoglobulin for significant weakness with plasmapheresis if weakness progresses, mechanical ventilation if necessary. NEVER GIVE STEROIDS!
MC intracranial neoplasms? Brain mets
Name 2 important indications for ordering an MRI to rule out an intracranial mass. 1. New-onset seizure in an adult 2. New and persistent or progressive headache
Specific risk of radiation therapy in intracranial neoplasms? May cause new primary tumors such as meningiomas
MC primary CNS neoplasms in adults? astrocytomas
Primary lymphomas are seen in which pt populations? Immunosuppressed, esp AIDS and transplant recipients
5 MC primary sites of mets to brain (in order of frequency)? 1. Lung (MC) 2. Breast 3. Skin (melanoma) 4. Kidney 5. GI tract
Ddx of a ring-enhancing brain lesion? Metastatic cancer, brain abscess, glio, lymphoma, and toxoplasmosis
CSF findings in meningeal carcinomatosis (mets to meninges, usually via bloodstream)? Malignant cells, elevated protein and lymphocytes, and decreased glucose
Bilateral Schwannomas are pathognomonic for what disease? Neurofibromatosis, type II
3 determinants of intracranial pressure? Volume of brain, volume of blood, volume of CSF
MCC death after a severe head injury? Elevated ICP (normal is 5-15; anything over 20 is worrisome)
What ophthalmologic finding suggests elevated ICP? Bilateral fixed and dilated pupils
4 signs of basilar skull fracture? 1. Raccoon eyes (periorbital ecchymoses) 2. Battle's sign (postauricular ecchymoses) 3. Hemotympanum 4. CSF rhinorrhea/otorrhea
What is a coup injury? Injury at site of impact (contrecoup is injury at site opposite the point of impact)
What is the normal cerebral perfusion pressure? What happens if ICP increases to the level of MAP? >50 is normal. If ICP increases to level of MAP, cerebral perfusion pressure is lost and brain cell death ensues.
ICU pressure goal for head trauma pts? MAP >80 and ICP <20. Ensures cerebral perfusion pressure of at least 60.
What is Cushing's triad? What does it signify? Triad is hypertension, bradycardia, and respiratory irregularity seen in response to increased ICP. Signifies life-threatening levels of ICP.
Most important regulator of cerebral vessel dilation? PaCO2. Increase PaCO2 causes cerebral vasodilation.
Techniques for lowering ICP? Reverse Trendelenburg (raise head of bed), intubation + hyperventilation, maintain PaCO2 30-35, mannitol, narcotics (morphine or fentanyl), neuromusc paralysis (vencuronium or pancuronium), lower body temp, drain CSF with ventricular catheter
Epidural hematoma is usually caused by injury to what structure? Middle meningeal a when temporal bone is fractured
Classic presentation for epidural hematoma? Brief LOC followed by a lucid interval--> coma (transtentorial herniation). May also see ipsilateral blown pupil.
Diagnosis for epidural hematoma? Convex mass on CT
Tx for epidural hematoma? Rapid surgical decompression
Cause of subdural hematoma? Venous bleeding after blunt head trauma (rupture of bridging vv)
Typical findings in subdural hematomas? Decreased LOC, headache, cortical dysfunction
Diagnosis of subdural hematoma? Cresent-shaped concave hematoma on head CT.
Tx for subdural hematoma? Acute: surgical evacuation Chronic: may resolve on its own depending on size
Why should a CT scan of thorax be ordered in suspected myasthenia gravis? To rule out thymoma
Which malignancy is associated with Lambert-Eaton Myasthenic syndrome? Small cell lung cancer
Difference between myasthenia gravis and Lambert-Eaton syndrome? In Lambert-Eaton, symptoms improved with repeated m stimulation. In myasthenia gravis, weakness is exacerbated by repeated m stimulation
Tx for myasthenia gravis? Anticholinesterase drugs like pyridostigmine (symptomatic), thymectomy (may provide remission), corticosteroids (for poor response to AChE inhibitors), plasmapheresis, IgG (for acute exacerbations)
Indication for intubation in myasthenia crisis? FVC of 15. Do NOT wait until pt is hypoxic-- they have a low threshold for intubation.
Inheritance of Duchenne's Muscular Dystrophy (DMD)? Mutation? X-linked recessive. Mutation on gene that codes for dystrophin protein.
Is there an inflammatory response in DMD? NO! Mm die in absence of dystrophin with no inflammation.
Diagnosis of DMD? Elevated serum creatine phosphokinase and m biopsy (replacement of necrotic m tissue by fat and CT)
Tx for DMD? None. Prednisone may slow progression of disease for ltd period of time.
AR inheritance of disease causing m cramping after exercise. Diagnosis? Cause? McArdle's disease. Caused by deficiency of glycogen phosphorylase.
Which medications exacerbate the symptoms of myasthenia gravis? Abx (aminoglycosides, tetracyclines), beta blockers, antiarrhythmics (quinidine, procainamide, and lidocaine)
Created by: sarah3148



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