stents dangerous

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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
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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. ******* *******



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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.

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

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.

transfusion related infections

Blajchman MA and Vamvakas EC. The Contining risk of transfusion-transmitted infections. NEJM 355:13: 1303-1305 Sept 28 2006

Authors remind us of risks to the blood supply. Neurologically speaking, culprits include West Nile Virus which is now mandated to be screened. vCJD (Variant CJD) with an incubation period oo up to 50 years has probably been transmitted in three cases in Britain. HHV8 a herpesvirus akin to CMV causes lifelong infections with periodic reactivations during which virus can circulate in WBC's and be transmitted. Risk factors probably overlap with HBV HCV and HIV andmayinduce Kaposi's sarcoma. No simple screening assay exists. Transfusing leukocyte reduced PRBC's may reduce transmission but this is unproved.

Discussion in blood bank circles includes whether to use pathogen reduction technologies such as methylene blue or solvent detergent, amotosalen for platelets, and UVA for RBC's.

Neuropathy treatments

MMN distal> proximal, +GM1, +IVIG, Cytoxan, ppx

MADSAM Lewis Sumner syndrome= MMN with sensory symptoms, distal>proximal +IVIG, + prednisone, ?cytoxan, ? Ppx

DADS -- rituxan role?

Mestinon for OH

Singer et al. Pyridostigmine treatment trial in neurogenic orthostatic hypothension. Arch Neurol 2006; 63:513-518.

Pyridostigmine may improve OH especially diastolic BP without affecting systolic BP due to effect on efferent limb of baroreflex in a 2 neuron system synapsing at the autonomic ganglion.

Study was a 4 way crossover study at Mayo Clinic in MN on 56 patients. Finding was that alone or with low dose midodrine alleviates OH without exacerbating HTN

CTX signs and lack thereof

Verrips et al. Presence of diarrhea and absence of tendon xanthomas in patients with cerebrotendinous xanthomatosis. Arch Neurol 2000; 57:520-524

Summary: study of 27 adults and 5 children at Netherlands. Only 41 % had tendon xanthomas at diagnosis. 97 % had premature cataracts and 81 % had long tract signs, 66 % had low intelligence and 56 % had cerebellar signs. Half had severe intractable diarrhea that started in childhood. Suggests CTX is underdiagnosed and should be sought in patients with cataracts and early diarrhea irrespective of neurologic disease.

Late onset Fabry's disease

Nance et al. Later onset Fabry disease. An adult variant presenting with cramp-fasciculation syndrome. Arch Neurol 2006; 63:453-457.

Fabry disease is an X linked recessively inherited disease due to deficient/absent lysosomal alpha galactosidase and accumulation of globotriaosylceramide (GL-3) and glycososphingolipids in plasma and cellular lysosomes. Classically, male onset occurs in childhood with episodic painful burning sensations in the hands and feets (acroparesthesias), typical skin lesions (angiokeratomas), hypohydrosis, and corneal opacities. Later, patients develop CRF, cardiac valvular disease and strokes, and die by 50s.

Late onset variants typically have renal or cardiac disease without typical neurologic stigmata in sixth decade or later.

Case report is of 34 year old man with activity induced cramps and fasciculations. Nerve biopsy, and pan MRI were negative. Mother had similar syndrome, with painful walking, and had a stroke at 37. Patient's alpha Gal A was deficient, leading to diagnosis.

Other features
Dolichoectasia of cerebral vessels
TIA
Vertigo
Hearing loss
Tinnitus
Cognitive disorders
Small fiber neuropathy
Painful acroparesthesias
Hypohidrosis
Impaired temperature sensation
Intestinal dysmotility
Cardiomyopathy
Cardiac conduction abnormality
Renal failure
Angiokeratomas
Corneal dystrophy
Feathery opacities

Autoantibodies in MG

Autoantibodies in thymoma-associated myasthenia gravis with myositis or neuromyotonia.

Thymomas are associated with red cell aplasia and in 50 %, with MG. Some patients with MG have inflammatory myopathy of striated and cardiac muscle. Diagnosis is with prosimal muscle weakness, high CPK levels, and myopathic EMG. Cardiac myositis leads to CHF, arrythmia, and sudden death. Patients with thymoma also may have neuromyotonia (NMT) with hyperactive peripheral motor nerves, myokymia, muscle stiffness, cramps, hypertrophy. EMG shows bursts of high frequency motor unit discharges. Antibodies against VGKC have been detected in NMT with or without thymoma. Other antibodies seen are against skeletal muscle calcium release channel (ryanodine receptor RyR) of sarcoplasmic reticulum; and antibodies to cytoplasmic filaments titin or neurofilaments.

There are 3 subgroups of thymoma associated MG with different spectrum of antibodies. 19/19 had AchR antibodies and 17/19 had anti-titin antibodies that did not correlate with symptoms (although anti-titin was higher in patients with antiRyR). Antititin is more or less synonymous with striated muscle antibodies and correlates with the presence of a thymoma.

10/19 had anti RyR, of which five had myositis and 1 had NMT. Myositis is associated with a poorer prognosis, occassionally rippling muscle disease, electrically silent muscle contractions.

7/16 patients tested had anti VGKC, and 4 had NMT, 1 myositis and 2 nothing. RyR and VGKC coexisted only in 2 patients.

Thymic pathology was not correlated, and included thymic ca and carcinoid.

DADS

more pn miscellany

rituxan is useful? in DADS

distal acquired demyelinating sensory neuropathy (DADS),  M protein differentiates from classical CIDP as does (lack of ) response to treatment. Is large fiber neuropathy in men in fifties or above.  Half have anti MAG antibodies, poor response to IVIG.  Differentiate DADS-M (monoclonal paraproteinemia present) from DADS no M which is sensory CIDP. 

Miscellany on neuropathy & tests

from Saperstein
B12--may cccur suddenly, 11% hands, 75 % hands and feet, 42 % PN. In evaluating, recall that MMA is increased in renal disease and volume depletion, homocystein in hypothyroidism, age, genetic syndromes, pyridoxine deficiency.

MGUS incidence of monoclonal proteins in population above 50 is 1 %, above 70 is 3 %, in patients with PN is 10 %. SPEP misses 10 percent caught by SIFE. 30 % of MGUS patients eventually develop something (MM, WM, Amyloid). Quantify M protein should be less than 3 g/dl. +/- BM biopsy. Axonal EMG not as bad. DADS also called demyelinating PN, "CIDP" (wrong) IGM neuropathy and anti MAG neuropathy. Usually occurs in older males (> 60). Sensory ataxia, PROLONGED LATENCIES ON NCS are typical. Consider fat biopsy if cardiac,renal, or disease is present.

Celiac disease-- note there are cases with negative duodenal biopsies. Antigliadin antibodies are present in 5-12 percent of normals, but transglutaminase antibodies are more specific. No response to IVIG or poor response to gluten free diet but do it anyway.

antiHU antibodies are best saved for patients with cerebellar ataxia or encephalopathy. NCS show sensorimotor pattern. Less known antibody is CD@ also known as CRMP (collapsing response mediator protein 5). Consider lung cancer, thymoma.

GM1 antibody associates with multifocal motor neuropathy, or at low titers other AI neuropathies not seen in MADSAM.

antiMAG-- small pct dont have igm

Barohn's PN phenotypes

1. Symmetric proximal and distal weakness with sensory loss. GBS or CIDP, is treatable

2. Symmetric distal sensory loss with or without weakness. This is untreatable with immunomodulators and is the most common PN. Within category the most common types are CSPN (cryptogenic sensory peripheral neuropathy) and metabolic, a category that includes diabetic neuropathy and alcoholic neuropathy. Rarer causes include hereditary sensory neuropathies.

3. Asymmetric distal weakness with sensory loss. This includes mononeuritis multiplex, consider vasculitis, motor sensory variant and HNPP (hereditary nerve pressure palsies) or MADSAM (multifocal acquired distal sensory and motor neuropathy, or Lewis Sumner syndrome) which is diagnosed with EMG. Rarer causes are infection (HIV, sarcoid, lyme) or involvement of single nerves or roots (eg. CTS).

4. Asymmetric proximal and distal weakness with sensory loss. Proximal means it may include elbow extensors, hip girdle muscles, neck muscles, knees, covering c5-T1 or L2-S1). Consider polyradiculopathy or plexopathy, due to DM, plexopathies, polyradiculopathy due to neoplasia, carcinoma, lymphoma, and hereditary neuropathy

5. Asymmetric distal weakness without sensory loss eg. progressive footdrop. Category includes ALS (especially with UMN signs), progressive muscular atrophy including regional forms (brachial or leg) juvenile monomelic atrophy, multifocal acquired motor neuropathy

6. Symmetric sensory loss (like number 2) but with UMN signs (crossed adductors, Babinski) think B12 deficiency, ALD, MLD or combination of diabetic/CSPN with cervical spondylosis diagnosis of exclusion)

7. Symmetric weakness without sensory loss-- consider SMA (Kennedy s. includes bulbar, chromosome five includes neck weakness). With bilateral footdrop may have normal EMG. CMT SNAPs are abnormal. overlaps MD and NMJ

8. Focal midline proximal symmetric weakness with neck extensor weakness (ALS, myopathy, MG, oculopharyngeal d., Kennedy s.)

9. Sensory asymmetric proprioceptive loss without weakness. Looks like neuronopathy (CA) but NORMAL SNAPS, abnormal roots on MRI suggest autoimmune, +/- treat with IVIG

10. Autonomic dysfunction

Exceptions to above
1) Mononeuritis multiplex including vasculitis presents symmetrically in one third

2) CIDP -- MMN and MADSAM may be asymmetric distal

3) Demyelinating CMT has no proximal weakness

4) DADS phenotype looks like pattern 2 at bedside but on exam has prolonged latiencies.

SE III

Status epilepticus is a neurologic emergency. The first item is medical stablization airway-breathing-circulation. Confused patients should be given thiamine, then glucose (50 grams_, thiamine (100 mg i-v), Narcan (0.4-2.0 mg i-v), and flumazenil (if indicated) 0.2 mg i-v. Labs should be sent, including electrolytes, CBC, diff, Ca, Mg, PO4, extra red top, AED levels, tox screen/ ETOH level.
Several drugs may be used for the acute treatment of SE, but the key is knowing in detail the pharmacokinetic proporties of the chosen drugs. Lorazepam is often given first line, and is considered the most rapidly effective. The dose is 0.5-1.0 mg/kg, Unlike diazepam, is not metabolized by the liver and has a longer half life. Typically, if lorazepam is used a longer acting drug needs to be added immediately. Cerebyx can be given i-v. Unlike i-v Dilantin, Cerebyx can be given rapidly, without cardiac monitoring, can be given i-m if no i-v access is obtained, and does not cause purple hand syndrome (safer and more effective), Dosing is identical to Dilantin. IT IS NOT ONE GRAM. The dosing is 20 mg/kg, about 1500 grams in an average sized patient, and underdosing can cause recurrent seizures. In patients with verified diagnosis of overt GCSE, response rates were as follows: lorazepam, 64.9%; phenobarbital, 58.2%; diazepam and phenytoin, 55.8%; and phenytoin alone, 43.6%. In statistical comparison of the pairs, only the difference between lorazepam and phenytoin alone was significant.
If the patient has a single seizure and is able to swallow pills, they may be orally loaded with Dilantin Kapseals, 100 mg tablets in the 20 mg/kg dosing schedule. Typically for a 80 kg man, 400 mg po q 3 hours times four doses is reasonable. A Dilantin level should be checked the following morning and a daily dose ordered. LIQUID DILANTIN GIVEN THROUGH DOBHOFFS NEVER ACHIEVES THERAPEUTIC LEVELS! (Give Kapseals or i-v Cerebyx).
Alternatives: Depacon (= i-v Depakote) 20 mg/kg i-v over five minutes, phenobarbital 10-20 mg/kg i-v with monitoring over an hour. In refractory SE (ie, that which does not respond to either regimen above), a commonly used protocol is intravenous pentobarbital. A loading dose of 5 mg/kg is followed by 0.5-3 mg/kg/h titrated to cessation of seizures or a burst-suppression pattern on EEG. In a recent study of patients with refractory SE, Krishnamurthy and Drislane concluded that the survival rate was better in patients whose EEG was more suppressed. Hypotension is a risk of pentobarbital infusion. In patients who cannot tolerate pentobarbital, alternatives include continuous infusion of benzodiazepines (eg, midazolam or propofol).
Pitfalls
1. Failure to perform EEG-- may miss nonconvulsive SE and the chance to treat. May miss pseudoseizures and overtreat patient.
2. Failure to perform lumbar puncture-- may miss meningitis
3. Failure to consider a diagnosis of herpes encephalitis-- must be treated early
4. Using above medications incorrectly
5. Failure to order EEG monitoring on admission if patient is not back to normal-- similar to 1-- may miss ongoing SE
6. Discharge from ER prior to patient returning to baseline cognitively-- may miss HSVE
7. Failure to consider differential diagnosis-- intoxication, locked in syndrome, psychogenic SE etc.
Complex partial status epilepticus
Favorable neurologic outcomes of CPSE have been reported regardless of whether medical treatment was successful. Few reports indicate serious sequelae complicating CPSE. Thus, the question of how aggressively to treat CPSE remains controversial. In general, pending a good, randomized trial, CPSE should be treated similarly to GCSE, except that treatment should stop before the use of general anesthesia (eg, pentobarbital coma).
In acute stages and for diagnosis, treatment with an intravenous benzodiazepine may be helpful. Often, out-of-hospital treatment with rectal or oral benzodiazepines aborts an episode. Williamson believes that, since most patients with CPSE have a history of epilepsy, concomitant AED therapy should be optimized.
Walker and Shorvon reported that, although most episodes of CPSE are self-terminating, recurrent episodes are encountered, and medical treatment is often disappointing. Patients who have medically refractory localization-related epilepsy should be evaluated for surgical therapy.
Absence status epilepticus
Walker and Shorvon reported that ASE responds rapidly to intravenous benzodiazepines. D'Agostino and coworkers believe that valproate is the medication of choice for ASE. Although effective, this treatment may result in complications such as sedation and respiratory depression. Kaplan summarized a case of a female with known absence epilepsy in which hospitalization was avoided by treating ASE with intravenous valproate. Snead et al stated that the more atypical the SE, the more difficult it is to control with benzodiazepines and other forms of therapy. Patients with primary generalized epilepsy should have optimized valproate or ethosuximide therapy to prevent recurrent episodes of ASE. Thomas et al reported that long-term anticonvulsant therapy might not be necessary in adults who are middle-aged or older at the onset of de novo ASE.

Li

SE II

Status epilepticus is a neurologic emergency. The first item is medical stablization airway-breathing-circulation. Confused patients should be given thiamine, then glucose (50 grams_, thiamine (100 mg i-v), Narcan (0.4-2.0 mg i-v), and flumazenil (if indicated) 0.2 mg i-v. Labs should be sent, including electrolytes, CBC, diff, Ca, Mg, PO4, extra red top, AED levels, tox screen/ ETOH level.
Several drugs may be used for the acute treatment of SE, but the key is knowing in detail the pharmacokinetic proporties of the chosen drugs. Lorazepam is often given first line, and is considered the most rapidly effective. The dose is 0.5-1.0 mg/kg, Unlike diazepam, is not metabolized by the liver and has a longer half life. Typically, if lorazepam is used a longer acting drug needs to be added immediately. Cerebyx can be given i-v. Unlike i-v Dilantin, Cerebyx can be given rapidly, without cardiac monitoring, can be given i-m if no i-v access is obtained, and does not cause purple hand syndrome (safer and more effective), Dosing is identical to Dilantin. IT IS NOT ONE GRAM. The dosing is 20 mg/kg, about 1500 grams in an average sized patient, and underdosing can cause recurrent seizures. In patients with verified diagnosis of overt GCSE, response rates were as follows: lorazepam, 64.9%; phenobarbital, 58.2%; diazepam and phenytoin, 55.8%; and phenytoin alone, 43.6%. In statistical comparison of the pairs, only the difference between lorazepam and phenytoin alone was significant.
If the patient has a single seizure and is able to swallow pills, they may be orally loaded with Dilantin Kapseals, 100 mg tablets in the 20 mg/kg dosing schedule. Typically for a 80 kg man, 400 mg po q 3 hours times four doses is reasonable. A Dilantin level should be checked the following morning and a daily dose ordered. LIQUID DILANTIN GIVEN THROUGH DOBHOFFS NEVER ACHIEVES THERAPEUTIC LEVELS! (Give Kapseals or i-v Cerebyx).
Alternatives: Depacon (= i-v Depakote) 20 mg/kg i-v over five minutes, phenobarbital 10-20 mg/kg i-v with monitoring over an hour. In refractory SE (ie, that which does not respond to either regimen above), a commonly used protocol is intravenous pentobarbital. A loading dose of 5 mg/kg is followed by 0.5-3 mg/kg/h titrated to cessation of seizures or a burst-suppression pattern on EEG. In a recent study of patients with refractory SE, Krishnamurthy and Drislane concluded that the survival rate was better in patients whose EEG was more suppressed. Hypotension is a risk of pentobarbital infusion. In patients who cannot tolerate pentobarbital, alternatives include continuous infusion of benzodiazepines (eg, midazolam or propofol).
Pitfalls
1. Failure to perform EEG-- may miss nonconvulsive SE and the chance to treat. May miss pseudoseizures and overtreat patient.
2. Failure to perform lumbar puncture-- may miss meningitis
3. Failure to consider a diagnosis of herpes encephalitis-- must be treated early
4. Using above medications incorrectly
5. Failure to order EEG monitoring on admission if patient is not back to normal-- similar to 1-- may miss ongoing SE
6. Discharge from ER prior to patient returning to baseline cognitively-- may miss HSVE
7. Failure to consider differential diagnosis-- intoxication, locked in syndrome, psychogenic SE etc.
Complex partial status epilepticus
Favorable neurologic outcomes of CPSE have been reported regardless of whether medical treatment was successful. Few reports indicate serious sequelae complicating CPSE. Thus, the question of how aggressively to treat CPSE remains controversial. In general, pending a good, randomized trial, CPSE should be treated similarly to GCSE, except that treatment should stop before the use of general anesthesia (eg, pentobarbital coma).
In acute stages and for diagnosis, treatment with an intravenous benzodiazepine may be helpful. Often, out-of-hospital treatment with rectal or oral benzodiazepines aborts an episode. Williamson believes that, since most patients with CPSE have a history of epilepsy, concomitant AED therapy should be optimized.
Walker and Shorvon reported that, although most episodes of CPSE are self-terminating, recurrent episodes are encountered, and medical treatment is often disappointing. Patients who have medically refractory localization-related epilepsy should be evaluated for surgical therapy.
Absence status epilepticus
Walker and Shorvon reported that ASE responds rapidly to intravenous benzodiazepines. D'Agostino and coworkers believe that valproate is the medication of choice for ASE. Although effective, this treatment may result in complications such as sedation and respiratory depression. Kaplan summarized a case of a female with known absence epilepsy in which hospitalization was avoided by treating ASE with intravenous valproate. Snead et al stated that the more atypical the SE, the more difficult it is to control with benzodiazepines and other forms of therapy. Patients with primary generalized epilepsy should have optimized valproate or ethosuximide therapy to prevent recurrent episodes of ASE. Thomas et al reported that long-term anticonvulsant therapy might not be necessary in adults who are middle-aged or older at the onset of de novo ASE.

ADEM in pediatrics

ADEM A Long term followup study of 84 pediatric patients.

Argentinian series of patients admitted to the National Hospital. Preceding illnesses occurred in 74 %: 35 % nonspecific RTI 12 % immunization , 11 % GI illness, rest nonspecific febrile,varicella, HSVE, mumps, rubells or undefined.

Age at onset was 5.3 years +/- 3.8 years with a male predominance. The presentation was usually hemiparesis (76 %) with unilateral or bilateral long tract signs (85 %0 and mental status changes (69%). None had oligoclonal banding. High dose coricosteroids use was associated with a good recovery and minimal/no disability. 90 % were monophasic, 10 % were biphasic. Disability was often based on optic nerve involvement at presentation.

Xyrem facts

Gamma hydroxybutyrate (GHB) was approved for oral treatment of cataplexy in patients with narcolepsy, and also in excessive daytime somnolence in the same patients. Xyrem is a rapid acting hypnotic with a short half life, increasing the duration of stage 3 and 4 sleep. It does not cause tolerance or dependence upon abrupt cessation, but abuse has been reported with craving and severe dependence. It can cause respiratory depression and death if taken with other sedatives. It can worsen sleep apnea, cause HA/N/V/dizziness, depression, nocturnal urinary incontinence, sleepwalking, and has sodium which could be a problem in patients with CHF, CRF or HTN.

Dosing: begin at 4.5 g per night: 2.25 g at bedtime when in bed 2-4 hours after a meal and repeat 2.5-4 hours later. Titrate by 1.5 grams per night eavery 2 weeks to a total of 9 grams. Xyrem is a available as an oral solution in 180 ml bottles with 500 mg/ml.

Central pharmacy prescriptions are available at the Xyrem success program, 1866 997 3688

Nelarabine (Arranon) for ALL causes neurotoxicity

Nelarabine is a nucleoside analog approved by the FDA for treatment of patients with refractory T-cell ALL and T-cell lymphoblastic lymphoma (T-LBL). These diseases are usually treated aggressively with combination chemotherapy (vincristine, prednisone, anthracycline, asparaginase, cyclophosphamide and cytarabine). It is demythylated to ara-G (ara-GTP) which is incorporated into dna, causing apoptosis and fragmentation. 18 % of refractory patients (5/28) has a complete response. Median survival was 20 weeks. Among children, 9/39 (23%) had a CR with median survival of 13 weeks. The most common AE's are anemia, leukopenia, thrombocytopenia, HA, somnolence and GI effects, dyspnea and peripheral neuropathy. Neurotoxicity, which can be fatal, is dose limiting; paresthesia, ataxia, ocnfusion, convulsions, and coma.

Obturator and combined obturator/femoral neuropathy

Obturator neuropathy-- sensory symptoms, including paresthesias and pain, possibly radiating in medial thigh. Motor involvement leads to trouble walking, due to inability to adduct the thigh, so thigh will be abducted and the gait is wide based. Examination-- weakness of thigh adductors is seen. There are no deep tendon reflex changes but the adductor tendon reflex may be absent.Etiology-- extension or lateral movements of leg that stretch it through the pelvis. Most cases are due to severe trauma: gunshot wounds, pelvic fracture or major trauma, and very rarely, childbirth.

Combined obturator/femoral neuropathy-- much more common, occurs in upper plexus lesions near psoas muscle. Causes include retroperitoneal hematoma, metastatic cancer and lymphoma. Clinically the quadriceps and the adductors are both paralyzed leading to a functional deficit of the leg.

Within pelvis the obturator nerve is medial to femoral or sciatic lesions and rare cases occur due to cement extrusion after hip surgery and after pelvic fracture.

Arsenic poisoning affects upgaze

Nakamagoe et al. Upward gaze-evoked nystagmus withorganoarsenic poisoning.

The authors report 3 patients who drank well water contaminated with very high levels of an organoarsenic compound, diphenyarsenic acid, believed to originate in emetic agents used in WWII.

Clinical presentation included articulation disorder, twitching and tremors of the hands and feet, gait disturbance and oscillopsia. The patient had upbeat nystagmus and abnormal vertical smooth pursuit movements, and impaired vertical gaze-holding. The patients also had myoclonus with long tract signs, truncal and limb ataxia. MRI showed subtle midbrain changes. Symptoms improved with removal of exposure.The authors speculate that the toxin hits the rostral interstitial nucleus of Cajal.