TTHM 12.2 “Weakness” Day (5/13/15) Tint. Ch. 53, 166, 167, pp.1545, 1546
10a ~ Multiple Sclerosis (Kolb)
Treated c high dose steroids, occasionally plasmapheresis.
…Rudolf Virchow discovered myelin, taught Kussmaul, Osler…
Charcot’s Neurologic Triad: Nystagmus, Intention Tremor, Staccato Speech (NOT charcot’s triad for cholangitis: fever, jaundice, RUQ pain)
Affects more women than men typically onset 15 to 45 yo.
Multiple types: Relapsing, Remitting, Secondary Progressive, Primary Progressive, Progressive Relapsing. Etiology somewhat uncertain, less incidence closer to equator.
Lhermitte’s sign: shooting electrical pain c neck flexion (“barber chair phenomenon”)
Optic Neuritis, scotomas, discolored vision
internuclear ophthalmoplegia (delayed abduction)
MS flares get high dose steroids.
*Case: MS Flare c Neurogenic Flash Pulmonary Edema
still treat c high dose steroids… MS flares also highly associated c UTI…
Dx made by MR, c LP sometimes used for exclusion of competing Dxs…
1030a ~ Movement Disorders (Pipas)
Definitions & Parkinsonism…
Tremor ~ rhythmic involuntary movements related to voluntary movements (rest, intent, posture)
(Alcohol withdrawal can have multiple involuntary movements)
Myoclonus ~ hiccoughs, nocturnal myoclonus, sudden rapid twitching, asterixis (can be found c etomidate)
Asterixis ~ within myoclonus, liver disease…
Chorea ~ Sydenham’s c Strep rheumatic heart Dsx, Huntington’s, Wilson’s Dsx, Levodopa…
Athetosis ~ slow writhing movements c antipsychotics, tardive dyskinesia…blinking, grimacing, tongue movements… can occur c long term Reglan use…
Hemiballism ~ unilateral Chorea, violent movements of proximal limb muscles
Dystonia ~ sustained abnormal postures
Torticollis ~ neck muscle spasm uncomfortable & anxiety producing
Akathisia ~ restlessness & agitation, shuffling…
Tics ~ frequent, often in childhood, repetitive coordinated movements, suppression causes anxiety…
PARKINSON’s ~ ?genetic predisposition ~ damage to dopamine pathways in substantia nigra… Resting pill rolling tremor, cogwheel rigidity, masked facies & bradykinesia, festinating shuffling gait c postural instability. 80% idiopathic. Benztropine for tremor, & Sinemet (carbidopa levodopa). OD of meds can have schizophrenic ~ like presentations, AMS…
Take advantage of YouTube Videos for characteristic movements!
11a ~ Small Groups (Lavoie, Farber)
*Radial nerve Palsy ~ Saturday Night Palsy, Honeymoon Palsy (snuffbox sensation, wrist extension) …Posterior Interosseous Nerve ~ Radial Nn branch, behind lateral epicondyle ~ tennis elbow
*Median Nerve ~ Phalens & Tinels signs for Carpal Tunnel Impingement (pad of 2nd digit, wrist flexion) …Anterior Interosseous Nerve ~ “ok” sign, thumb Adductor (can be isolated injury)
*Ulnar Neuropathy ~ Cyclists/Biker’s Palsy, affects Adducts fingers, “funny bone”, Guyon’s canal (sensory pad of 5th digit, spread fingers)
12p HTN & HTN Emergencies (Kajla) ~ How high is BP too high to send home?
No specific #? All clinical judgment?
Per ACEP policy: 140 to 159 followup… 160 to 179 followup… 180 to 209 HTN “Urgency” contact PCP & consider 2 drug therapy. >210 too high, strongly consider initiating 2 drug therapy! & if symptomatic, obviously workup end organ damage pathology!
Case Report: Emphysematous Cystitis (rare, found in elderly women c DM, thought to be related to bacterial fermentation process)
1230 ~ Jr/Sr Sessions (Prasad/Rodriguez)
*Case1) MMWR (Sept 2006) Dermacentor Adersoni (Rocky Mountain Wood Tick) Tick Paralysis dt toxin in tick saliva works on ACh receptor in motor neuron endplate. 6yo girl intubated!
*Case2) Trendelenburg Gait, Back Pain & weakness… LEMS presynaptic weakness vs Myasthenia Gravis (often c ocular muscle weakness)… often associated c cancer, but cancer may not be detected. LEMS over age 50, especially smokers, often associated c lungCA.
*Case3) 29M sudden paralysis, K 1.6 ~ Mobitz 1 block. Periodic Paralysis (Familial Hypokalemic). IV Potassium c dramatic symptomatic improvement! Occurs dt rapid intracellular shift. Often occurs concurrent c hyperthyroid, autosomal dominant condition. Treat c K supplementation, acetazolamide, spironolactone…
*Case4) 17M weak, difficulty swallowing… no ocular problems, no neck or backache, PMHx 1 month prior c URI, Bell’s palsy & 9th/10th cranial nn. problems c swallowing… Systemic leukocytosis of 25K! GBS!!! Can be dt B vitamin deficiencies, Toxic, Metabolic, Postinfectious, RA, SLE, hereditary/Idiopathic… LP albuminocytologic dissociation…
130p ~ GBS (Joslin) Life in the Fast Lane
aka AIDP (Acute Inflammatory Demyelinating Polyneuropathy)
*Case1) Transverse Myelitis started with difficulty urinating
*Case2) GBS sp needlestick exposure
2p ~ Myelitis (MacConaghy)
Which one is transverse myelitis? Sensory deficits most common, often bladder, may be asymmetric, specific level deficits!
*Case1) 70F proximal muscles & urination weak, no incontinence, some hyperreflexia & POS babinski, positive romberg, but good cerebellar coordination.
*Case2) 51F midback pain, ascending numbness & weakness in legs, went to chiropractor, can’t walk, hyperreflexic, good rectal tone.
*Case3) 30M c 6 days numbness below the neck, difficulty c hot cold discrimination, & can’t feel pain in feet, sensory is only deficit!
“Transverse” myelitis refers to bandlike sensory deficit!
Criteria for diagnosis: ro other causes, *clearly defined sensory level!, analogous to spinal cord compression symptoms, sensory motor or autonomic dysfunction attributable to spinal cord!
Most have neck or back pain & recent illness.
(ADEM ~like MS in a kid with fever & encephalitis)
MRI with contrast is needed for diagnosis. LP may be needed to exclude DDx…
Labs ~CRP/ESR, lyme, TB, fungal, B12, ANA things to consider… NMO~Ig new test…
Treatment c Steroid & Abx (high dose Decadron or Solumedrol), consider plasma exchange…
Prognosis 1/3’s: 1/3 complete recovery, 1/3 partial recovery, 1/3 significant permanent deficits.
Case 1: Cervical Myelopathy
Case 2: Bony Metastatic disease, BRCA
Case 3: Focal Abnormality, though sensory deficits ONLY. This case 2* to MS.
230p ~ Myasthenia Gravis (Maley)
Autoimmune disorder ~ antibodies against ACh~Receptor (Anti~AChR Ab)
PostSynaptic, T lymphocyte role can be associated c thymic inflammation…
ED what do you need to know? DX: clinical.
Meds to AVOID? & How to treat EXACERBATION?
Bimodal distribution of age (20s to 30s female & 60s to 80s male)
Juvenile MG Ab can cross placenta… common presenting diplopia & pstosis
Airway protection is big priority!
Tensilon Test (edrophonium)… Some meds make it worse: Cipro/Levoflox, Azith/Gentamycin…
Treat c high dose steroids. For paralysis DO NOT USE SUX!
LEMS has LE proximal muscle weakness, often associated c small cell lungCA…
Snake venoms may cause similar symptoms c Coral snake (elapidae) & Mojave rattlesnake (crotalid, CroFab?)
3p ~ Bell’s Palsy (Lavoie)
Cranial Nerve VII inflammation, can be Lyme, HSV, VZV…
Most cases idiopathic, commonly treated c steroid & acyclovir for empiric & symptomatic management. Hyperacusis often precedes, dt stapedius tensor tympani paralysis. Loss of taste & dry mouth also common findings… Facial “numbness” report very common dt altered proprioception, although sensation will be present on physical exam testing.
Smaller deficit, bigger problem? Sparing of the forehead typically ~ CVA!
Bell’s can be b/L! Eye exam c Hutchinson sign c zoster. In endemic region, consider Lyme testing…
Eye patch or tape, eye ointment, acyclovir, steroid may have role in treatment…
Steroids help 10% of the time per Cochrane, relatively safe, NNT 11 to 12…
Antivirals no significant benefit or harm…
Imaging not routine, though MRI occasionally useful, might have falsePOS… ro CVA if necessary, i.e. atypical presentations.