spondylitic myelopathy -- limb fibs should go away within six months of fixing spine
inclusion body myositis pearl check finger flexors esp FPL FDP, forearm atrophy and check for absent fascics and symmetry
spinal cord AVM
heavy metal intoxication -- lead-- check for basophilic stippling and consider treatment with dmpa
lymphoproliferative disorder
multifocal motor neuropathy with conduction block (anti GM1 seen in about half) rsponds to IVIG
vasculitis
motor polyradiculopathy
infectious (polio, WNV)-- EMG shows poor recruitment, then fibs in 4-6 weeks
Bulbospinal mononeuropathy (Kennedy's syndrome) X linked, presents in men with symmetrical weakness, atrophy and fasciculations of bulbar and proximal limb muscles, testicular atrophy and gynecomastia.Occurs early, diagnosed by a CAG repeat, and is associated with a normal lifespan. Consider in males with slow course. Fascics are more pronounced in this disorder, but its all LMN.
Spinal muscular atrophy (Werdnig Hoffman, Kugelberg Welander)-- kids, young and old adults
Progressive lateral sclerosis (UMN variety) is associated with a normal lifespan
Benign fasciculations
cramp fasciculation syndrome-- easily treated with membrane stabilizers
Monomelic atrophy-- focal variant
frontotemporal dementia- identical path finding seen by Neumann ALS spectrun disorder?
Machado Jospeh disease= spinocerebellar atrophy tpe 3. See prominent anterior horn disorder plus ataxia and myelopathy
polyglucosan body disease-- rare UMN and LMN findings plus sensory changes and shite matter changes on MRI, with rare glucosan bodies seen on sural nerve biopsy.
Acid maltase deficiency may mimic PM abnormal respiratory muscles.
Syrinx
true neurologic TOS thoracic outlet syndrome -- shows decreased median CMAP, normal median SNAP, decreased ulnar SNAP, decreased medial antebrachial SNAP; needle shows median > ulnar hand involvement; lower trunk median/radial innervated forearm muscles (FPL, EIP)
myofibrillar myopathy-- = Desmond's myopathy-- mimics IBM. Get quad atrophy, medial gastroc, dysphagia, atrophy of VM/VL but rectus femoris is spared and is normal
Thursday, March 08, 2007
Thursday, February 15, 2007
Sensory Guillian-Barre Syndrome
Oh SJ, LaGanke C, Claussen GC. Sensory Guillian Barre. Neurology 2001; 56:82-82. Describes eight patients collected with a sensory equivalent of classical GBS. These patients had acute onset of a sensory ascending neuropathy that peaked within 4 weeks, generally had an antecedent viral illness, areflexia, CSF albumen-cytologic dissociation, demyelination in at least two nerves, (esp MOTOR nerves), favorable outcome. 3 patients were treated with immunomodulatory treatments.
Monday, February 12, 2007
Neck pain and calcium deposition
Nodera et al. Neurology 2007; 68:383 (Neuroimages). Japanese. A 43 year old man had neck pain, sore throat, dysphagia, and low grade fever upon awakening. He had limited ROM of the neck. CT showed calcification ANTERIOR to C1-2 with thickened prevertebral tissues due to effusion. Retropharyngeal calcific tendinitis is uncommon and confused with abscess but the characteristic calcium deposit is diagnostic. It is self limiting.
Paraneoplastic cerebellar degeneration antibodies
Anti Yo (PCA-1) ovarian and breast cancer; anti Tr antibodies -- Hodgkin's lymphoma; also for Hodgkin's lymphoma: Anti-mGluR1 and Anti Zic4 antibodies. Above are PREDOMINANTLY associated with PCD. SOMETIMES associated with PCD are anti VGCC (LEMS, small cell lung cancer); anti Hu (ANNA-1)(encephalomyelitis, PCD, sensory neuronopathy) (small cell lung and other CA); anti Ri (ANNA 2) (PCD, brainstem encephalitis; paraneoplastic opsoclonus/myoclonus)(breast, gyne and small cell CA);anti CV2/CRMP (encephalomyelitis, PCD, chorea, PN, uveitis)(small cell lung, thymoma, others); anti MA protein (limbic, hypothalamic, brainstem encephalitis, infrequently PCD)( testicular, lung and other cancers); antiamphiphysin (stiff person s, encephalomyelitis, PCD) (breast and small cell) CHART is FROM NEJM 2007; 356:612-620. MORE Tumors with anti Yo antibodies express Yo antigen which is a cytoplasmic protein called CDR2 that interacts with c-Myc. It is expressed in the Purkinje cells of the cerebellum and the large neurons of the brainstem. It sequesters c Myc and downregulates; disruption with antibodies may increase c Myc activity leading to apoptosis (although the T cell immune response may also be important).
rapidly progressive ataxia ddx
in immunocompentent patients:
stroke,
multiple sclerosis
sarcoidosis,
primary or metastatic diseases,
paraneoplastic disorders,
toxins/drugs (lead, anticonvulsants, salicyclates, aminoglycosides, sedatives, fluorouuacil, cytarabine),
Miller Fisher syndrome,
infection (HIV, viral/postviral cerebellitis, Creutzfeld Jacob disease, progressive multifocal leukoencephalopathy),
alcoholic cerebellar degeneration, thiamine deficiency,
autoimmune ( SLE, Sjogrens, Hashimoto's disease, cerebellar ataxia with anti GAD or antigliadin antibodies).
stroke,
multiple sclerosis
sarcoidosis,
primary or metastatic diseases,
paraneoplastic disorders,
toxins/drugs (lead, anticonvulsants, salicyclates, aminoglycosides, sedatives, fluorouuacil, cytarabine),
Miller Fisher syndrome,
infection (HIV, viral/postviral cerebellitis, Creutzfeld Jacob disease, progressive multifocal leukoencephalopathy),
alcoholic cerebellar degeneration, thiamine deficiency,
autoimmune ( SLE, Sjogrens, Hashimoto's disease, cerebellar ataxia with anti GAD or antigliadin antibodies).
Sunday, February 04, 2007
Lack of utility/overuse of muscle biopsy
Filosto M et al. The role of muscle biopsy in investigating isolated muscle pain. Neurology 2007; 68:181-186.
editorial Kissel JT. Muscle biopsy in patients with myalgia. Neurology2007; 68:170-171.
Filosto et al. reviewed clinicals and neuropath on 240 patients presenting with muscle pain isolated or cramping. 80 % had biopsy abnormalities but only 20 %got a specific diagnosis and only 6 % a specific myopathy. Patients with obvious causes eg. statin use were excluded before analysis. CK levels and EMG did not predict pathology. Patients need to go under rigorous selection prior to biopsy.
editorial Kissel JT. Muscle biopsy in patients with myalgia. Neurology2007; 68:170-171.
Filosto et al. reviewed clinicals and neuropath on 240 patients presenting with muscle pain isolated or cramping. 80 % had biopsy abnormalities but only 20 %got a specific diagnosis and only 6 % a specific myopathy. Patients with obvious causes eg. statin use were excluded before analysis. CK levels and EMG did not predict pathology. Patients need to go under rigorous selection prior to biopsy.
Saturday, February 03, 2007
cryoglobulinemic vasculitis PN
Gemignani F et al. Clinical spectrum of cryoglobulinemic neuropathy. JNNP 2005; 76:1410-1414.
Most common presentation is middle aged female patients who develop small fiber sensory neuropathy (73%). Less often, then sensorimotor neuropathy (18 %) then least often, mononeuritis multiplex (8%) occurs. Symptoms were tingling paresthesias (55%), sensory ataxia (38%), thermal dysesthesias (42%) pain (42%) and RLS (45 %). 29 % were asymmetrical. Patients did not have autonomic dysfunction.
Study occurred of 71 patients referred to academic center. Cryoglobulnemic syndrome was defined as intense recurring purpura and cryocrit>5 %, and "mild" syndrome with minimal purpura and cryocrit <5%. 46 % presented with PN. Hepatitis C caused the cryoglobulinemia in more than 90 %.
Comment--most cases were noo biopsied.
Most common presentation is middle aged female patients who develop small fiber sensory neuropathy (73%). Less often, then sensorimotor neuropathy (18 %) then least often, mononeuritis multiplex (8%) occurs. Symptoms were tingling paresthesias (55%), sensory ataxia (38%), thermal dysesthesias (42%) pain (42%) and RLS (45 %). 29 % were asymmetrical. Patients did not have autonomic dysfunction.
Study occurred of 71 patients referred to academic center. Cryoglobulnemic syndrome was defined as intense recurring purpura and cryocrit>5 %, and "mild" syndrome with minimal purpura and cryocrit <5%. 46 % presented with PN. Hepatitis C caused the cryoglobulinemia in more than 90 %.
Comment--most cases were noo biopsied.
Wednesday, January 24, 2007
Mimics of GBS Part II
Chemical toxins
vacor (rodenticide)ingested due to homicidal or suicidal intent. Impairs fast axon transport. Clinical findings are initially abdominal pain, nausea, vomiting, necrosis of pancreas beta cells causes hyperglycemia, rapid distal motor/sensory/Autonomic neuropathy with weakness, areflexia, dysesthesias, OH, urine retention, and cranial neuropathy, then later stupor, EEG changes, DKA, EKG changes. Long term residua often include DM and ANS dysfunction. Acute tx is gastric lavage and induced emesis.
Organophosphates -- found in malathion, parathion, dursban. 10-20 days after exposure they produce a distal axonopathy, calf cramps, extremity paresthesias and distal weaknesss. A SEPARATE syndrome is NMJ disorder with cholinergic toxicity and respiratory insufficiency.
n-Hexane solvent associated with glue sniffing and industrial exposure. Clinical includes distal sensory loss and severe weakness that progresses for months after exposure ends.
Drugs
CV-- amiodorone, streptokinase
Hem-- cytarabine, suramin.
amiodoarone chronic progressive predominantly distal s-m neuropathy. NCS show distal latency prolongation and slowing to 11 m/s. Dose is 200-400 mg per day.
streptokinase-- 10-20 cases, 10-20 days after exposure, paresthesias of hands and feet, cndxn blocks, elevated CSF protein, coincident to peak SKN titers, dramatic response to IVIG.
cytarabine-- 0.6 % of cases with doses more than 200 mg/m2/d. progressive distal sensorimotor neuropathy, hours to days after last treatment.
Suramin -- PN in 40 % of patients with levels> 350 ug/ml Rarely GBS like presentation.
Vasculitis
Churg-Strauss s-- may resemble GBS. Eosinophilia is seen. Tx with pred/MTX. Asthma, vasculitic lung involvment, eosinophilia and mononeuropathy are common but not universal.
Other cryoglobulinemia, PAN.
AIP-- Motor PN.
Refsums
Diptheria ("extinct" in US, present in developing countries)
Poliomyelitis--
West Nile Virus
Botulism
WNV
CMV polyradiculopathy
critical illness myopathy
critical illness neuropathy
Myelopathy
vacor (rodenticide)ingested due to homicidal or suicidal intent. Impairs fast axon transport. Clinical findings are initially abdominal pain, nausea, vomiting, necrosis of pancreas beta cells causes hyperglycemia, rapid distal motor/sensory/Autonomic neuropathy with weakness, areflexia, dysesthesias, OH, urine retention, and cranial neuropathy, then later stupor, EEG changes, DKA, EKG changes. Long term residua often include DM and ANS dysfunction. Acute tx is gastric lavage and induced emesis.
Organophosphates -- found in malathion, parathion, dursban. 10-20 days after exposure they produce a distal axonopathy, calf cramps, extremity paresthesias and distal weaknesss. A SEPARATE syndrome is NMJ disorder with cholinergic toxicity and respiratory insufficiency.
n-Hexane solvent associated with glue sniffing and industrial exposure. Clinical includes distal sensory loss and severe weakness that progresses for months after exposure ends.
Drugs
CV-- amiodorone, streptokinase
Hem-- cytarabine, suramin.
amiodoarone chronic progressive predominantly distal s-m neuropathy. NCS show distal latency prolongation and slowing to 11 m/s. Dose is 200-400 mg per day.
streptokinase-- 10-20 cases, 10-20 days after exposure, paresthesias of hands and feet, cndxn blocks, elevated CSF protein, coincident to peak SKN titers, dramatic response to IVIG.
cytarabine-- 0.6 % of cases with doses more than 200 mg/m2/d. progressive distal sensorimotor neuropathy, hours to days after last treatment.
Suramin -- PN in 40 % of patients with levels> 350 ug/ml Rarely GBS like presentation.
Vasculitis
Churg-Strauss s-- may resemble GBS. Eosinophilia is seen. Tx with pred/MTX. Asthma, vasculitic lung involvment, eosinophilia and mononeuropathy are common but not universal.
Other cryoglobulinemia, PAN.
AIP-- Motor PN.
Refsums
Diptheria ("extinct" in US, present in developing countries)
Poliomyelitis--
West Nile Virus
Botulism
WNV
CMV polyradiculopathy
critical illness myopathy
critical illness neuropathy
Myelopathy
AIDP atypical forms and mimics PT I
Levin KH. Variants and mimics ocf Guillian Barre Syndrome.The Neurologist 2004;10:61-74
typical case AIDP: febrile antecedent esp. campylobacter jej, CMV, EBV, infl, mycoplasma, coxsackie, hepatitis virus. Other noninfectious causes include Hodgkins dis, and events such as surgery, childbirth, and immunization. Within 2 weeks, get symmetric leg paresthesiae, tingling, crawling sensations, pain esp in back (50%), ascending weakness. 50 % develop weakness diaphragmand cranial nn. > 50 % have autonomic findings. CSF dissociation acellularity, increased protein, and typical NCS (not detailed here).
Variants: asymmetric; pure motor; prominent sensory loss; preserved DTR's; regional presentations (pharyngeal/brachial/cervical); paraparetic; facial diplegia with paresthesias; pure sensory neuropathy; pure autonomic neuropathy; Miller Fisher variant; axon loss variants (AMAN, AMSAN).
Miller Fisher syndrome-- triad opthalmoplegia, areflexia, ataxia (follows sensory loss). NCS show loss of SNAP amplitude. Course is often benign,maynot require IVIG/Plasmapx. may include facial weakness, dysarthria, dysphagia, abnormal pupils, limb weakness. 95 % have ANTI GQ1B antibodies.
AMAN-- Chinese disease-- NCS show motor amplitude loss without demyelination. High correlation with campylobacter. Antibodies to GD1a and GM1 at node of Ranvier. Involves complement activation and macrophage infiltration.
AMSAN-- cannot differentiate from AMAN until enough time has elapsed to show NCS findings. Typically this does not respond to IVIG/plasmapx.
Mimics:
tick paralysis-- notoriously overlooked in children, esp preadolescent girls. Ticks may infest scalp. Symptoms develop 2-4 days later including paresthesias, gait ataxia, diplopia, dysarthria, rapid weakness. Tick is dermocenter andersonii. Paralysis reverses within 24 hours of tick removal. Australian ticks cause paralysis that lasts longer. NCS show reduced CMAP amplitudes.
marine toxins-- ciguatoxin (diflagellates eaten by algae), tetrodotoxin (pufferfish) and saxitoxin
(dinoflagellates eaten by shellfish)cause paresthesias and weakness within hours. NCS show slow motor/sensory responses (mimics GBS) and dispersed CMAP.
buckthorn berry-- in SW USA and Mexico. Affects children and cattle causing rapid weakness within a few weeks. Limited amt published.
heavy metals (esp arsenic, gold, thallium).
Arsenic-- 7-14 days after ingestion. presents with N/V/D paresthesias, burning, then stocking glove loss small and large fibers. Weakness is distal to proximal and lead to footdrop. Predominant axon loss, Occassional ans dysfunction.
Gold-- usually secondary to RA treatment. PN, with pain, may be sudden or slow, with or without dermatitis and stomatitis.
Thallium-- usually deliberate ingestion (may be accidental in kids) or due to mal intent. Causes dermatitis, alopecia (usually a LATE finding), GI (N/V/D), CNS (ON, confusion, movement disorders, psych), acute and chronic PN. Starts with painful paresthesias and painful joints, ascending weakness, possibly normal DTR's, autonomic dysfunction
typical case AIDP: febrile antecedent esp. campylobacter jej, CMV, EBV, infl, mycoplasma, coxsackie, hepatitis virus. Other noninfectious causes include Hodgkins dis, and events such as surgery, childbirth, and immunization. Within 2 weeks, get symmetric leg paresthesiae, tingling, crawling sensations, pain esp in back (50%), ascending weakness. 50 % develop weakness diaphragmand cranial nn. > 50 % have autonomic findings. CSF dissociation acellularity, increased protein, and typical NCS (not detailed here).
Variants: asymmetric; pure motor; prominent sensory loss; preserved DTR's; regional presentations (pharyngeal/brachial/cervical); paraparetic; facial diplegia with paresthesias; pure sensory neuropathy; pure autonomic neuropathy; Miller Fisher variant; axon loss variants (AMAN, AMSAN).
Miller Fisher syndrome-- triad opthalmoplegia, areflexia, ataxia (follows sensory loss). NCS show loss of SNAP amplitude. Course is often benign,maynot require IVIG/Plasmapx. may include facial weakness, dysarthria, dysphagia, abnormal pupils, limb weakness. 95 % have ANTI GQ1B antibodies.
AMAN-- Chinese disease-- NCS show motor amplitude loss without demyelination. High correlation with campylobacter. Antibodies to GD1a and GM1 at node of Ranvier. Involves complement activation and macrophage infiltration.
AMSAN-- cannot differentiate from AMAN until enough time has elapsed to show NCS findings. Typically this does not respond to IVIG/plasmapx.
Mimics:
tick paralysis-- notoriously overlooked in children, esp preadolescent girls. Ticks may infest scalp. Symptoms develop 2-4 days later including paresthesias, gait ataxia, diplopia, dysarthria, rapid weakness. Tick is dermocenter andersonii. Paralysis reverses within 24 hours of tick removal. Australian ticks cause paralysis that lasts longer. NCS show reduced CMAP amplitudes.
marine toxins-- ciguatoxin (diflagellates eaten by algae), tetrodotoxin (pufferfish) and saxitoxin
(dinoflagellates eaten by shellfish)cause paresthesias and weakness within hours. NCS show slow motor/sensory responses (mimics GBS) and dispersed CMAP.
buckthorn berry-- in SW USA and Mexico. Affects children and cattle causing rapid weakness within a few weeks. Limited amt published.
heavy metals (esp arsenic, gold, thallium).
Arsenic-- 7-14 days after ingestion. presents with N/V/D paresthesias, burning, then stocking glove loss small and large fibers. Weakness is distal to proximal and lead to footdrop. Predominant axon loss, Occassional ans dysfunction.
Gold-- usually secondary to RA treatment. PN, with pain, may be sudden or slow, with or without dermatitis and stomatitis.
Thallium-- usually deliberate ingestion (may be accidental in kids) or due to mal intent. Causes dermatitis, alopecia (usually a LATE finding), GI (N/V/D), CNS (ON, confusion, movement disorders, psych), acute and chronic PN. Starts with painful paresthesias and painful joints, ascending weakness, possibly normal DTR's, autonomic dysfunction
Saturday, January 06, 2007
CNS complications of HPCT
Denier et al. Spectrum and prognosis of neurologic complications after hematopoeitc transplantation. Neurology 2006;67: 1990-1997.
Modern study. Ablation of bone marrow with allogeneic grafts are treated with prophylaxis against previously common events such as veno-occlusive disease and pulmonary complications. Now neurologic disease is a major cause of M/M. They occurred in about 16 % of patients receiving HPCT, usually opportunistic infections. Toxoplasmosis was the most common cause (33%) with others getting viral encephalitis, HSVE, and less commonly, metabolic encephalopathy, stroke, peripheral nervous system dysfunction.
Modern study. Ablation of bone marrow with allogeneic grafts are treated with prophylaxis against previously common events such as veno-occlusive disease and pulmonary complications. Now neurologic disease is a major cause of M/M. They occurred in about 16 % of patients receiving HPCT, usually opportunistic infections. Toxoplasmosis was the most common cause (33%) with others getting viral encephalitis, HSVE, and less commonly, metabolic encephalopathy, stroke, peripheral nervous system dysfunction.
Spinal epidural abscess
Darouiche RO. Current Concept: Spinal epidural abscess NEJM 35;19 2012 Nov 9 2006
Article concentrates on bacterial causes. Most patients have predisposing risk factors: DM, ETOH, HIV, drug abuse intravenous, DJD, surgery with instrumentation, placement of stimulators, or potential local source of infection (skin, soft tissue, indwelling catheter, UTI, sepsis). Entry is contiguous spread (1/3) or hematogenous (1/3). Two thirds are staph aureus, 40 % of those Methicillin resistant (MRSA). These are especially common a few weeks after implantation of devices.
Other causes: St epidermidis, e coli, Ps aer., rarely actinomyces, nocardia, mycobacteria, fungi, (candida, aspergillus) parasites (echonococcus and dracunculus). A letter writer added brucellosis as a cause in Spain, Italy and the Near East.
Spinal cord injury can occur either by mechanical compression of vascular occlusion due to septic thrombophlebitis.
Clinical staging system: stage 1, back pain at level; stage 2, nerve root pain of affected level; stage 3, motor weakness, sensory loss, or B/B dysfunction at affected level; stage 4, paralysis. 3/4 have back pain, fever present in half, neurologic dysfunction in one third. Stage 2 is "enigmatic" in thoracic cases. Duration/rate of progression is highly variable but can be extremely rapid.
Abscesses favor large epidural spaces with infection prone fat, and therefore are more common in posterior than anterior and thoracolumbar than cervical. Lumbar even more common due to epidural injections. Generally they extend over 3-4 levels or more.
Diagnosis is by drainage. Sed rate is always high. Differential diagnostic conditions ( osteomyelitism discitis, sepsis, endocarditis) also have S aureus bacteremia. CSF cultures are negative in 75%. Blood cultures are usually positive. Risks of LP includes causing meningitis by introducing infection through the meninges or causing deterioration if tapped below a block.
MRI with contrast is preferred procedure although myelography has > 90 % sensitivity. Spine CT can be done in conjunction with and may suggest changes of osteopmyelitis.
Frequent misdiagnoses include: osteomyelitism discitis, meningitis, UTI, sepsis, endocarditis, disc prolapse, DJD, spinal tumor, transverse myelitis, spinal hematoma. These are made because patient is neurologically intact.
Treatment: medical v. surgical. No trials done. Medical treatment indicated if patient has high risk of surgery, has panspinal infection, or paralysis for more than 24 hours, or if agent is identified and his condition is monitored closely. Surgery can be a limited laminectomy if patient has a panspinal infection. Empiric coverage should include coverage against MRSA, against gram negative bacilli (with a third or fourth generation cephalosporin such as ceftazidime). Letter writer added that medical management was safe as long as the patient didnot deteriorate.
Statistics: 4-22% get irreversible paralysis. 11-75 % are initially misdaignosed.
The most important predictor of outcome is patient's status just before surgery. Patients who undergo surgery in stage 1 or 2 are expected to remnain neurologically intact.
Article concentrates on bacterial causes. Most patients have predisposing risk factors: DM, ETOH, HIV, drug abuse intravenous, DJD, surgery with instrumentation, placement of stimulators, or potential local source of infection (skin, soft tissue, indwelling catheter, UTI, sepsis). Entry is contiguous spread (1/3) or hematogenous (1/3). Two thirds are staph aureus, 40 % of those Methicillin resistant (MRSA). These are especially common a few weeks after implantation of devices.
Other causes: St epidermidis, e coli, Ps aer., rarely actinomyces, nocardia, mycobacteria, fungi, (candida, aspergillus) parasites (echonococcus and dracunculus). A letter writer added brucellosis as a cause in Spain, Italy and the Near East.
Spinal cord injury can occur either by mechanical compression of vascular occlusion due to septic thrombophlebitis.
Clinical staging system: stage 1, back pain at level; stage 2, nerve root pain of affected level; stage 3, motor weakness, sensory loss, or B/B dysfunction at affected level; stage 4, paralysis. 3/4 have back pain, fever present in half, neurologic dysfunction in one third. Stage 2 is "enigmatic" in thoracic cases. Duration/rate of progression is highly variable but can be extremely rapid.
Abscesses favor large epidural spaces with infection prone fat, and therefore are more common in posterior than anterior and thoracolumbar than cervical. Lumbar even more common due to epidural injections. Generally they extend over 3-4 levels or more.
Diagnosis is by drainage. Sed rate is always high. Differential diagnostic conditions ( osteomyelitism discitis, sepsis, endocarditis) also have S aureus bacteremia. CSF cultures are negative in 75%. Blood cultures are usually positive. Risks of LP includes causing meningitis by introducing infection through the meninges or causing deterioration if tapped below a block.
MRI with contrast is preferred procedure although myelography has > 90 % sensitivity. Spine CT can be done in conjunction with and may suggest changes of osteopmyelitis.
Frequent misdiagnoses include: osteomyelitism discitis, meningitis, UTI, sepsis, endocarditis, disc prolapse, DJD, spinal tumor, transverse myelitis, spinal hematoma. These are made because patient is neurologically intact.
Treatment: medical v. surgical. No trials done. Medical treatment indicated if patient has high risk of surgery, has panspinal infection, or paralysis for more than 24 hours, or if agent is identified and his condition is monitored closely. Surgery can be a limited laminectomy if patient has a panspinal infection. Empiric coverage should include coverage against MRSA, against gram negative bacilli (with a third or fourth generation cephalosporin such as ceftazidime). Letter writer added that medical management was safe as long as the patient didnot deteriorate.
Statistics: 4-22% get irreversible paralysis. 11-75 % are initially misdaignosed.
The most important predictor of outcome is patient's status just before surgery. Patients who undergo surgery in stage 1 or 2 are expected to remnain neurologically intact.
Thursday, December 28, 2006
Psychotropic effects of New AED's
Ettinger a. Neurology 2006;67: 1916-1925. Review paper. Random notes
Gabapentin--
1. aberrant/aggressive behavior noted in children and patients with developmental disorders. 2. Ineffective for BPAD 3. It does help behaviors in institutionalized patients with AD 4. Useful in epileptic patients with anxiety
Lamotrigine-- 1. It does help mood in depressed patients with epilepsy. 2. Effective in controlling impulsive aggression in one study.
Leviteracetam-- 1. Causes depression or anxiety in up to 15 % of those treated, especially those with preexisting disorders that are exacerbated with the drug. 2. Reversible psychotic symptoms occur in children treated. 3. May be ameliorated with pyridoxine. 4. Tested for treatment of mania and social anxiety syndrome
oxcarbazepine-- too limited information to make conclusions.
pregabalin--similar to gabapentin. Useful for epilepsy patients with anxiety.
topiramate-- related to psychomotor slowing. Used in bPAD for weight reducing side effect. Not proven efffective in BPAD. May be effective for anger/aggression in those with borderline personalities. Other side effects in 1-2 % of patients (7 % of kids) are psychotic symptoms, agitation, aggression, hallucinations, auditory and visual, paranoid and mystical delusions. Does not stabilize moods.
tiagabine-- possibly anxiolytic but not a mood stabilizer.
Gabapentin--
1. aberrant/aggressive behavior noted in children and patients with developmental disorders. 2. Ineffective for BPAD 3. It does help behaviors in institutionalized patients with AD 4. Useful in epileptic patients with anxiety
Lamotrigine-- 1. It does help mood in depressed patients with epilepsy. 2. Effective in controlling impulsive aggression in one study.
Leviteracetam-- 1. Causes depression or anxiety in up to 15 % of those treated, especially those with preexisting disorders that are exacerbated with the drug. 2. Reversible psychotic symptoms occur in children treated. 3. May be ameliorated with pyridoxine. 4. Tested for treatment of mania and social anxiety syndrome
oxcarbazepine-- too limited information to make conclusions.
pregabalin--similar to gabapentin. Useful for epilepsy patients with anxiety.
topiramate-- related to psychomotor slowing. Used in bPAD for weight reducing side effect. Not proven efffective in BPAD. May be effective for anger/aggression in those with borderline personalities. Other side effects in 1-2 % of patients (7 % of kids) are psychotic symptoms, agitation, aggression, hallucinations, auditory and visual, paranoid and mystical delusions. Does not stabilize moods.
tiagabine-- possibly anxiolytic but not a mood stabilizer.
Fludaribine for MGUS
Niermeijer JMF et al. Neurologic and hematologic response to fludaribine treatment in IgM MGUS polyneuropathy. Neurology 2006:67:2076-2079.
Uncontrolled trial of 16 patients with IgM with progressive IgM MGUS treated with fludaribine.
Patients: 6 were MAG positive, 14 had kappa light chain, 2 had lambda light chain, 3 had axonal neuropathy and 13 had demyelinating neuropathy. 9 used ambulation aids.
10/16 were first treated with cyclophosphamide/prednisone
Uncontrolled trial of 16 patients with IgM with progressive IgM MGUS treated with fludaribine.
Patients: 6 were MAG positive, 14 had kappa light chain, 2 had lambda light chain, 3 had axonal neuropathy and 13 had demyelinating neuropathy. 9 used ambulation aids.
10/16 were first treated with cyclophosphamide/prednisone
4-H syndrome
Timmons et al. Peripheral and central hypomyelination with hypogonadotropic hypogonadism and hypodontia. NIH. Neurology 2006; 67:2066-2069.
Pubertal onset dysmyelinating leukodystrophy. Characteristics: young adults, absent spontaneous puberty, normal till age 7-12, borderline iq's (60s-70s), dysarthria, absent pursuit, upbeat or other nystagmus, normal sensory exam, dysmetria, long tract signs.
Sural nerve biopsies showed membraneous and other debris, loss of major and minor dense lines, increased neurofilaments. Decreased galactocerebrosidase and sphingomyelin. Immunostaining for PO protein showed clefted disruption of myelin sheaths and laminar deposits in circumferential distribution. MAG staining was similar but less definitive. Nerve conductions were normal.
MRI showed supratentorial white matter signal approximated cortex on T1, hyperintense WM on T2 (demyelination) atrophic cerebellum and thin corpus callosum.
Heterogenous Holmes-Adie syndrome has described the combination of cerebellar ataxia and hypogonadotropic hypogonadism.
Pubertal onset dysmyelinating leukodystrophy. Characteristics: young adults, absent spontaneous puberty, normal till age 7-12, borderline iq's (60s-70s), dysarthria, absent pursuit, upbeat or other nystagmus, normal sensory exam, dysmetria, long tract signs.
Sural nerve biopsies showed membraneous and other debris, loss of major and minor dense lines, increased neurofilaments. Decreased galactocerebrosidase and sphingomyelin. Immunostaining for PO protein showed clefted disruption of myelin sheaths and laminar deposits in circumferential distribution. MAG staining was similar but less definitive. Nerve conductions were normal.
MRI showed supratentorial white matter signal approximated cortex on T1, hyperintense WM on T2 (demyelination) atrophic cerebellum and thin corpus callosum.
Heterogenous Holmes-Adie syndrome has described the combination of cerebellar ataxia and hypogonadotropic hypogonadism.
Radiation retinopathy
Grimm et al. Retinopathy in survivors of primnary CNS lymphoma. Neurology 2006:67:2060-2062.
Study reports specifically five survivors of PCNSL who developed syndrome, 1.5 % of database. Patients received high dose MTX then WBRT then post-XRT chemo including procarbazine, vincristine, and carmustine. Mean latency to developing syndrome was 27 months. Note- syndrome is also reported in other diseases treated with WBRT.
Presentation was blurred vision (2/5), partial visual loss (3/5) and floaters (1/5). Fluroscein agio confirmed bilateral disease in 3. Treatments given include intraocular steroids and laser therapy. No patients had progressive ocular disease although one had radiation dementia and another had ongoing PCNSL.
Study reports specifically five survivors of PCNSL who developed syndrome, 1.5 % of database. Patients received high dose MTX then WBRT then post-XRT chemo including procarbazine, vincristine, and carmustine. Mean latency to developing syndrome was 27 months. Note- syndrome is also reported in other diseases treated with WBRT.
Presentation was blurred vision (2/5), partial visual loss (3/5) and floaters (1/5). Fluroscein agio confirmed bilateral disease in 3. Treatments given include intraocular steroids and laser therapy. No patients had progressive ocular disease although one had radiation dementia and another had ongoing PCNSL.
dopa responsive disorders
Friedman J, Hyland K, Blau N, MacCollin M. Dopa-responsive hypersomnia and mixed movement disorder due to seriapterin reductase deficiency. Neurology 2006:67:2032-2035.
Defects in monoamine synthesis are associated with phenotypes of developmental delay, diurnal variation in symptom severity, and dopa-responsive dystonia. A subset also with cognitive delay due to SPR (seriapterin reductase ) gene have dopa and serotonergic responsive hypersomnolence and movement disorder responsive.
Case report-- 27 year old woman with "CP" had delayed milestones, abnormal gait, incoordination, and hypersomnolence. Other maternal relatives had abnormal limb posturing. She had FSIQ 60, punctate cataracts, oculomotor apracis, dysarthria, bradykinesia, , PTOSIS, generalized dystonia, myoclonus, normal MRI/PET.
CSF showed decreased 5HIAA and HVA and increased 7,8 dihydropterin c/w SRD. Treatment with Sinemet and selegeline and later combined with sertraline helped.
The same patient was FIRST treated with sertraline alone (akathisia), melatonin (dystonic neck spasms), 5 hydroxytriptophan (helped but led to increased LFT's), levodopa-carbidopa (caused dyskinesias; these are rare in DRD or dopa responsive dystonias).
Inheritance is AR. Further information available at the BIODEF database (www.bh4.org)
Defects in monoamine synthesis are associated with phenotypes of developmental delay, diurnal variation in symptom severity, and dopa-responsive dystonia. A subset also with cognitive delay due to SPR (seriapterin reductase ) gene have dopa and serotonergic responsive hypersomnolence and movement disorder responsive.
Case report-- 27 year old woman with "CP" had delayed milestones, abnormal gait, incoordination, and hypersomnolence. Other maternal relatives had abnormal limb posturing. She had FSIQ 60, punctate cataracts, oculomotor apracis, dysarthria, bradykinesia, , PTOSIS, generalized dystonia, myoclonus, normal MRI/PET.
CSF showed decreased 5HIAA and HVA and increased 7,8 dihydropterin c/w SRD. Treatment with Sinemet and selegeline and later combined with sertraline helped.
The same patient was FIRST treated with sertraline alone (akathisia), melatonin (dystonic neck spasms), 5 hydroxytriptophan (helped but led to increased LFT's), levodopa-carbidopa (caused dyskinesias; these are rare in DRD or dopa responsive dystonias).
Inheritance is AR. Further information available at the BIODEF database (www.bh4.org)
Wednesday, November 15, 2006
stents dangerous
-----------------
Forwarded Message:
Subj: Endarterectomy versus stenting in patients with symptomatic severe carotid stenosis
Date: 11/13/2006 1:42:12 PM Eastern Standard Time
From: BestEvidence@mp.medscape.com
To: djacobs272@aol.com
Sent from the Internet (Details)
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Dear Dr. JACOBS,
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Neurology
Article(s) Relevance Newsworthiness
1 Endarterectomy versus stenting in patients with symptomatic severe carotid stenosis.
N Engl J Med. 2006 Oct 19;355(16):1660-71. ******* *******
2 Efficacy and safety of pramipexole in restless legs syndrome.
Neurology. 2006 Sep 26;67(6):1034-9. Epub 2006 Aug 23. ******* *******
3 Efficacy of cabergoline in restless legs syndrome: a placebo-controlled study with polysomnography (CATOR).
Neurology. 2006 Sep 26;67(6):1040-6. Epub 2006 Aug 23. ******* *******
4 Treatment with interferon beta-1b delays conversion to clinically definite and McDonald MS in patients with clinically isolated syndromes.
Neurology. 2006 Oct 10;67(7):1242-9. Epub 2006 Aug 16. ******* *******
5 Effect of donepezil on motor and cognitive function in Huntington disease.
Neurology. 2006 Oct 10;67(7):1268-71. ******* *******
6 Unification of the revised trauma score.
J Trauma. 2006 Sep;61(3):718-22; discussion 722. ******* *******
Medscape Best Evidence, powered by the McMaster Online Rating of Evidence ("MORE") program, vastly improves the signal-to-noise ratio in keeping up to date. By employing filters for research quality and clinical relevance, these alerts can shrink the approximate 50,000 articles per year that appear in more than 110 journals and might be relevant to your clinical practice down to a manageable amount that is likely to be truly important to your clinical practice.
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Forwarded Message:
Subj: Endarterectomy versus stenting in patients with symptomatic severe carotid stenosis
Date: 11/13/2006 1:42:12 PM Eastern Standard Time
From: BestEvidence@mp.medscape.com
To: djacobs272@aol.com
Sent from the Internet (Details)
Medscape Best Evidence
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Dear Dr. JACOBS,
We hope you enjoy this trial issue of the Medscape Best Evidence Neurology weekly newsletter.
Click here to subscribe today.
Neurology
Article(s) Relevance Newsworthiness
1 Endarterectomy versus stenting in patients with symptomatic severe carotid stenosis.
N Engl J Med. 2006 Oct 19;355(16):1660-71. ******* *******
2 Efficacy and safety of pramipexole in restless legs syndrome.
Neurology. 2006 Sep 26;67(6):1034-9. Epub 2006 Aug 23. ******* *******
3 Efficacy of cabergoline in restless legs syndrome: a placebo-controlled study with polysomnography (CATOR).
Neurology. 2006 Sep 26;67(6):1040-6. Epub 2006 Aug 23. ******* *******
4 Treatment with interferon beta-1b delays conversion to clinically definite and McDonald MS in patients with clinically isolated syndromes.
Neurology. 2006 Oct 10;67(7):1242-9. Epub 2006 Aug 16. ******* *******
5 Effect of donepezil on motor and cognitive function in Huntington disease.
Neurology. 2006 Oct 10;67(7):1268-71. ******* *******
6 Unification of the revised trauma score.
J Trauma. 2006 Sep;61(3):718-22; discussion 722. ******* *******
Medscape Best Evidence, powered by the McMaster Online Rating of Evidence ("MORE") program, vastly improves the signal-to-noise ratio in keeping up to date. By employing filters for research quality and clinical relevance, these alerts can shrink the approximate 50,000 articles per year that appear in more than 110 journals and might be relevant to your clinical practice down to a manageable amount that is likely to be truly important to your clinical practice.
All alerts are pre-assessed for scientific merit (based on the criteria for Evidence-Based Medicine and ACP Journal Club) and qualifying articles are then rated by at least three clinicians in each pertinent discipline for relevance to clinical practice and how new or "newsworthy" the information is likely to be to practicing clinicians.
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Saturday, November 11, 2006
Prevention of meningococcal disease
Gardner P. NEJM 355: 1466-1473. Clinical Practice section.
N meningitidis colonizes the nasopharyx in 18 % of the population. Transmission is by droplet. Most adults have antibodies to the pathogenic subgroups (A,B,C,Y w-135). Classically infection occurs in crowded conditions, eg. military recruits. Infection occurs within 7-10 days of transmission and can be fatal. Occurrence is 0.5-1.1 per 100,000 population. Case fatality rate is 10 %. Sequelae occur in 11-19 % due to neurologic effects or DIC residua. 62 % occur in kids < 11. Other risk factors include crowding, RTI, active and passive smoking, asplenia, terminal complement deficiency. Travel to endemic areas such as Saudi Arabia or sub Saharan Africa also are risk factors. In year one of college rate is 5.1/100,000 but by year two its 1.4 or almost normal.
Chemoprophylaxs of close contacts: "Close contacts" are defined as people in 3 foot range (droplet range) or exposure through oral secretions including ventilatory tubing. For adults chemoprophylaxis is Copro 500 mg once, rifampin 600 q 12 for two days, or ceftriaxone 125 mg im onc if <15, 250 mg im once if older than 15. Chemoprophylaxis is indicated for vaccinated since vaccine does not cover all strains. Chemoprophylaxis should be undertaken within 24 hours.
Vaccines cover strains A,C, Y W-135 but not B. Two vaccines exist. Number one , Menomune (Sanofi) lasts 3 years and is good for travellers, people with limited risk (army recruits, college kids). Second one Menactra (also Sanofi) just released last two years, is more durable (lasts longer) and revaccination results in booster response. There is a warning on Menactra about GBS but it is not clear the 8 cases were more than would be expected in the population.
N meningitidis colonizes the nasopharyx in 18 % of the population. Transmission is by droplet. Most adults have antibodies to the pathogenic subgroups (A,B,C,Y w-135). Classically infection occurs in crowded conditions, eg. military recruits. Infection occurs within 7-10 days of transmission and can be fatal. Occurrence is 0.5-1.1 per 100,000 population. Case fatality rate is 10 %. Sequelae occur in 11-19 % due to neurologic effects or DIC residua. 62 % occur in kids < 11. Other risk factors include crowding, RTI, active and passive smoking, asplenia, terminal complement deficiency. Travel to endemic areas such as Saudi Arabia or sub Saharan Africa also are risk factors. In year one of college rate is 5.1/100,000 but by year two its 1.4 or almost normal.
Chemoprophylaxs of close contacts: "Close contacts" are defined as people in 3 foot range (droplet range) or exposure through oral secretions including ventilatory tubing. For adults chemoprophylaxis is Copro 500 mg once, rifampin 600 q 12 for two days, or ceftriaxone 125 mg im onc if <15, 250 mg im once if older than 15. Chemoprophylaxis is indicated for vaccinated since vaccine does not cover all strains. Chemoprophylaxis should be undertaken within 24 hours.
Vaccines cover strains A,C, Y W-135 but not B. Two vaccines exist. Number one , Menomune (Sanofi) lasts 3 years and is good for travellers, people with limited risk (army recruits, college kids). Second one Menactra (also Sanofi) just released last two years, is more durable (lasts longer) and revaccination results in booster response. There is a warning on Menactra about GBS but it is not clear the 8 cases were more than would be expected in the population.
Saturday, October 21, 2006
hypothermia after arrest
Bernard et al. Treatment of comatose survivors of our of hospital cardiac arrest with induced hypothermia. NEJM 2002 346:557-63 (Australia) Study done 1996-1999. Candidates men>18, women >50, v fib, coma, cardiogenic shock (BP<90 despite pressors). Basic cooling in ambulance, core cooling in ER to 33 degrees, usual measueres used eg. heparin tpa, lidocaine, versed, pancuronium. Life support withdrawn at 72 hours. n=77 patients, 39/43 assigned to hypothermia received it. 21/43 patients assigned to hypothermia had a good outcome, 9/34 in normothermia group (49 v. 26 %)defined as discharge home or to rehab. Normal disability was achieved in 15 v 7 patients (35 v 21 %). Mortality (51 v. 68 % ) did not reach significance. Factors affecting outcome included age (two years led to 9 % less chance of good outcome)and time till return of circulation also was important.
Paper 2: Hypothermia study group. Mild therapeutix hypothermia to improve the neurologic outcome after cardiac arrest. NEJM 346:549-556. (Austria). Primary endpoint is favorable neurologic outcome at 6 months by Pittsburgh cerebral performance (1 good recover, 2, mod disability, 3 severe disability, 4 veg, 5 death). Criteria: witnessed arrest, VF, no hypotension, comatose. 1996-2001. 275/35551 patients enrolled. 137 to hypothermia group, 138 to control. Of treated group, 75/136 (55%) had good outcome v. 54/137 (39 %) NTT to get one additional good outcome = 6. Death was less in treated group: NTT=7.
Notes
Paper 2: Hypothermia study group. Mild therapeutix hypothermia to improve the neurologic outcome after cardiac arrest. NEJM 346:549-556. (Austria). Primary endpoint is favorable neurologic outcome at 6 months by Pittsburgh cerebral performance (1 good recover, 2, mod disability, 3 severe disability, 4 veg, 5 death). Criteria: witnessed arrest, VF, no hypotension, comatose. 1996-2001. 275/35551 patients enrolled. 137 to hypothermia group, 138 to control. Of treated group, 75/136 (55%) had good outcome v. 54/137 (39 %) NTT to get one additional good outcome = 6. Death was less in treated group: NTT=7.
Notes
Wednesday, September 27, 2006
Tuberous sclerosis complex
Crino et al. NEJM 355:13: 1345-1356 The Tuberous Sclerosis Complex [Review article}
TSC is a multisystem autosomal dominant disorder affecting children and adults, resulting from mutations in one of two genes, TSC1(encoding hamartin) or TSC2 (encoding tuberin).
Neurologic disorders include epilepsy, mental retardation, and autism. Other features are facial angiofibromas (formerly adenoma sebaceum), renal angiomyolipomas, and pulmonary lymphangiomyomatosis. TSC has a wide spectrun of disease ranging from subclinical to severely affected. Clinical trials utilizing tuberin and hamartin are underway.
The diagnosis is made clinically using major and minor criieria. Genetic tests are currently considered corroborative. Diagnosis may be made by developmental stage specific findings.
Renal disease (angiomyolipomas) occur in up to thre fourths, may be vascular, may sometimes be treated with embolization, and are most dangerous if greater than 3 cm in size.
TSC is a multisystem autosomal dominant disorder affecting children and adults, resulting from mutations in one of two genes, TSC1(encoding hamartin) or TSC2 (encoding tuberin).
Neurologic disorders include epilepsy, mental retardation, and autism. Other features are facial angiofibromas (formerly adenoma sebaceum), renal angiomyolipomas, and pulmonary lymphangiomyomatosis. TSC has a wide spectrun of disease ranging from subclinical to severely affected. Clinical trials utilizing tuberin and hamartin are underway.
The diagnosis is made clinically using major and minor criieria. Genetic tests are currently considered corroborative. Diagnosis may be made by developmental stage specific findings.
Renal disease (angiomyolipomas) occur in up to thre fourths, may be vascular, may sometimes be treated with embolization, and are most dangerous if greater than 3 cm in size.
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