Term
| Most common progressive motor neuron dz that causes muscle weakness, disability, and eventually death. |
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Definition
| Amyotrophic Lateral Sclerosis (ALS) |
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Term
| What is the median survival rate of ALS? |
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Definition
|
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Term
| What are the 2 forms of ALS? |
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Definition
| 1)Sporadic forms (unknown cause) 2)Familial forms (autosomal dominant inherited) |
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Term
| What is the most common of the 2 forms of ALS? |
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Definition
| Sporadic forms (90% of cases) |
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Term
| What is the peak age of onset of ALS? |
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Definition
|
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Term
| What are the 2 est. risk factors for ALS? |
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Definition
|
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Term
| Name 3 suggested risk factors for ALS. |
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Definition
| 1)Smoking 2)Factory workers 3)Welding or soldering exposure |
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Term
| ALS is characterized by loss of motor neurons in the _____ and loss of corticospinal fibers in the _________. |
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Definition
| Anterior horn, brain stem, and motor cortex. Lateral and anterior columns of the spinal cord. |
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Term
| What is the clinical hallmark of ALS clinical findings? |
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Definition
| A combination of upper motor neuron and lower motor neuron signs |
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Term
| Name 4 upper motor neuron signs. |
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Definition
| 1)Hyperreflexia 2)Spacticity 3)Extensor plantar response (+Babinski's sign) 4)Positive jaw jerk |
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Term
| Name 4 lower motor neuron signs. |
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Definition
| 1)Weakness 2)Muscle atrophy 3)Cramps 4)Fasiculations |
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Term
| What is the most common clinical presentation of ALS present in ~80% of cases? |
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Definition
|
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Term
| What is the 2nd most common clinical presentation of ALS present in ~20% of cases? |
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Definition
| Bulbar sx - dysarthria or dysphagia |
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Term
| Do pts w/ ALS have relapsing or remitting sx or are sx progressive? |
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Definition
|
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Term
| What is the diagnostic criteria for ALS? |
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Definition
|
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Term
| Name the 4 clinical findings required for El Escorial criteria. |
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Definition
| 1)LMN in at least 2 limbs 2)UMN in at least 1 region 3)EMG finding of fibrillation potentials for evidence of LMN involvement 4)Exclusion of ddx by electro, neuroimaging, and lab |
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Term
| What is the only med. approved for the tx of ALS? |
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Definition
| Riluzole (Rilutek) glutamate inhibitor |
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Term
| The most common autoimmune inflammatory demyelinating dz of the CNS. |
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Definition
|
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Term
| Who is MS most common in? (Age, gender, race) |
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Definition
| 20-45yrs, Female 2:1, Northern European descent |
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Term
| What is the etiology of MS? |
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Definition
|
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Term
| What are the 4 main classes of MS? |
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Definition
| 1)Relapsing-remitting 2)Primary-progressive 3)Secondary-progressive 4)Progressive-relapsing |
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Term
| What is the most common class of MS? |
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Definition
| Relapsing - remitting (85%) |
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|
Term
| What is the pathogenesis of MS? |
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Definition
| Breakdown of the myelin sheath of nerve cells seen around basal ganglia, medulla, and cerebellum, on optic nerves and in spinal cord |
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Term
| What is the difference b/t preimary and secondary progressive MS? |
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Definition
| Primary - (rare, MC in men)slow but continuous decline in neurological fxn. Secondary - begins as relapsing-remitting followed by steady decline. |
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Term
| Describe sensory loss seen in pts w/ MS. |
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Definition
| Numbness, tingling, impaired vibratory and joint position sense, dec. pain/light touch sensation |
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Term
| Describe pain expereinced by pts w/ MS. |
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Definition
| Trigeminal neuralgia, Lhermitte's sign, Back pain, mus. cramping d/t spasticity |
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Term
| Describe cerebellar sx experienced by pts w/ MS. |
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Definition
| Dysarthria, ataxia, and tremor |
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Term
| WHat is Uhthoff's phenomenom? |
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Definition
| Heat sensitivity (hot flashes)seen in MS |
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Term
| What are constitutional sx seen in pts w/ MS? |
|
Definition
| Fatigue and dizziness or vertigo |
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Term
| 15% of pts w/ MS have this condition and 50% of pts w/ this condition have MS. |
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Definition
| Optic neuritis (flashes of light and deterioration of vision) |
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Term
| What type of tremor is present in pts w/ MS? |
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Definition
|
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Term
| What is the diagnostic test of choice for MS? |
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Definition
| MRI of brain or spine (ovoid appearance of lesions) |
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Term
| What meds are used to tx MS when stiffness, spasms, or clonus interfere w/ function. |
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Definition
| Muscle relaxanats (Baclofen, Zanflex, Neurotin, or benzos) |
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Term
| In paroxysmal d/o of MS, what meds are used to tx dystonic spasms? Pain? |
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Definition
| Carbamazepine. Anticonvulsants or amitriptyline. |
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Term
| What meds are used to tx failure to store urine in MS? |
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Definition
| Anticholinergics (Ditropan or Detrol) |
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Term
| What meds can be used for pts w/ a flaccid bladder in MS? |
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Definition
|
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Term
| What can be used to tx nocturnal urinary production in MS? |
|
Definition
| Inhaled desmopressin (DDAVP) |
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|
Term
| What is the definitive tx for urinary retention in MS pts? |
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Definition
|
|
Term
| What is a common bowel sx seen in pts w/ MS? |
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Definition
|
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Term
| 50% of pts w/ MS have what neurobehavioral manifestation? |
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Definition
|
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Term
| What med can be used to tx depression in MS pts? |
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Definition
| SSRI or amytriptyline (for depression + insomnia, pain, or HA) |
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Term
| What med is used to tx relapsing-remitting MS for acute exacerbation + optic neuritis? |
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Definition
|
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Term
| What are the 4 approved drugs to tx relapsing-remitting MS? |
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Definition
| 1)IM interferon beta-1a (Avenox) 2)SQ Glatiramer acetate (Copaxone) 3)SQ interferon beta-1a (Rebif) 4)SQ Interferon beta-1b (Betaseron) |
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Term
| What labs are usuaaly elevated on CSF? What finding is seen in 85% of pts? |
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Definition
| IgG index. Oligoclonal bands. |
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|
Term
| What meds are used to tx primary and secondary progressive MS? |
|
Definition
| IV glucocorticoid + Cyclophosphamide OR Methotrexate |
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Term
| Which has a better prognosis, the relapsing or progressive form of MS? |
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Definition
| Relapsing has a better prognosis |
|
|
Term
| What is the life expectancy of a pt w/ MS? |
|
Definition
| 5-7 yrs shorter than general population |
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|
Term
| Acute inflammatory demyelinating polyneuropathy char. by progressive symmetric ascending muscle weakness, paralysis, and hyporeflexia w/ or w/o sensory sx. |
|
Definition
| Guillain-Barre syndrome (GBS) |
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Term
| What is the prognosis of GBS? |
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Definition
| Up to 85% of pts achieve full recovery w/i 6-12mths |
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Term
| Name 4 viral illnesses that may precipitate GBS. |
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Definition
| HZV, Mumps, Measles, or EBV |
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|
Term
| Name 3 bacterial infections that may precipitate GBS. |
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Definition
| Mycoplasma pna, Campylobacter jejuni, or enteritis |
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Term
| Name 4 other conditions/procedures that may precipitate GBS. |
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Definition
| Immunizations, trauma, surgery, or Hodgkin's dz |
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Term
| Name 4 cardinal clinical features of GBS. |
|
Definition
| 1)Progressive symmetric muscle weakness 2)Absent or dec. DTR 3)Severe low-back pain 4)Dysautonomia |
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Term
| What is dysautonomia seen in GBS pts? |
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Definition
| Tachycardia, HTN alternating w/ hypotension, bradycardia, urinary retention, loss of sweating |
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Term
| What diagnostic finding is present in 90% of GBS pts at 1 wk /p onset of sxs? |
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Definition
| Elevated CSF proteins w/ normal CSF/WBC count |
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Term
| Name 2 disease-modifying tx for GBS. |
|
Definition
| Plasmapheresis and IV immune globulin |
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Term
| Name 5 poor prognostic factors for GBS. |
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Definition
| 1)Older age 2)Rapid onset (<7d) 3)Need for vent 4)NCS 5)Preceeding diarrheal illness |
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Term
| The most common d/o of neuromuscular transmission. |
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Definition
|
|
Term
| What is the etiology of MG? |
|
Definition
| Autoimmune d/o of peripheral nerves (AB form a/g Ach receptors) |
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|
Term
| Which gender is MG more common in the adult peak (20-30yo)? Older adult peak (>50yo)? |
|
Definition
|
|
Term
| 75% of pts w/ MG have some sort of ____ abn. |
|
Definition
| Thymus (suspected to be site of autoantibody production) |
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|
Term
| What is the hallmark clinical finding in pts w/ MG? |
|
Definition
| Fluctuating skeletal muscle weakness |
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|
Term
| 50% of pts w/ MG present w/ ocular sx, what are they? |
|
Definition
| Ptosis, diplopia, orbicularis oculi weakness (expresionless) |
|
|
Term
| What are the 3 phases of MG? |
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Definition
| 1)Active phase (most severe/fluctuations) 2)Stable second phase (stable but persistent sx) 3)Third phase (remission possible on immunotherapy) |
|
|
Term
| What is myasthenia or cholinergic crisis? |
|
Definition
| Bronchospasm w/ wheezing, bronchorrhea, resp. failure, diaphoresis and/or cyanosis |
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Term
| Name 2 bedside tests used to dx MG. |
|
Definition
| 1)Ice pack test (used w/ ptosis) 2)Tensilon challenge test (IV injection of Edrophonium) |
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Term
| Name 2 confirmatory tests for MG. |
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Definition
| 1)Serologic (detect Ab a/g Ach receptor) 2)Electrodiagnostic (repetitive nerve stim. and EMG) |
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|
Term
| What meds are used as symptomatic tx for MG? |
|
Definition
| Anticholinesterase (Pyridostigmine) |
|
|
Term
| What is the surgical tx for MG? |
|
Definition
|
|
Term
| What tx is reserved for myasthenia crisis or b/f thymectomy? |
|
Definition
| Plasma exchange + IV immune globulin |
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|
Term
| Name 2 complications of MG. |
|
Definition
| Respiratory failure or aspiration pna/CAP |
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|
Term
| What is the prognosis for MG? |
|
Definition
| <4% mortality (thymectomy typ. results in complete remission) |
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