Clinical Care of ALS patients

based on Radunovic Am Mitsumoto H, Leight PN Review article in Lancet Neurology October 2007. Based on the idea that basic medical management is underutilized. Major points:
1. Diagnosis (correct diagnosis) necessitates evaluation initially including brain and spine imaging (most of the time), CXR, lab tests, EMG. Use El Esocorial criteria http://www.wfnals.org/guidelines/1998elescorial/elescorial1998criteria.htm. Prior links to other important ALS information including EMG criteria, mimics and others. Dx is hardest with only UMN or only LMN signs. Mean survival is 30 months from diagnosis. Long survivors were not necessarily the ones taking riluzole. The ALS Functional Rating Scale http://www.outcomes-umassmed.org/als/alsscale.cfm and vital capacity are the most useful clinical measures.

Authors emphasize team approach and palliative care. Some of the treatments especially medicines are well known and obvious and are not reblogged. Some "tricks" are listed below.

excess saliva-- non medicine treatments include home suction, dark grape juice, sugar free citrus lozenges, nebulizers, botox, parotid radiation, steam inhalers. Medicines include elavil glycopyrrholate, hyoscine.

Excess broncial secretions-- propanolol, metoprolol, carbocysteine?, assisted cough insufflator/exsufflator. rehydration, pineapple or papaya juice, butter, decreased intake dairy products, alcohol and caffeine.

excess yawning-- baclofen

laryngospasm-- baclofen

emotional lability-- TCA's, Sinemet, dextromethrophan and quinidine

Noninvasive ventilation at onset of respiratory insufficiency among patients with no/moderate bulbar dysfunction improves survival. It also improves quality of life. Initiate when FVC is less than 40 % that of predicted and it doubles survival. Authors state measure of sniff nasal pressure is better than FVC and that measure of 32 % (= 25 cm H2O) or less predicts respiratory failure whereas FVC of 40-50 % does not do so as well . SNP less than 40 cm H20 predict survival. Other measures such as inspir/expir mouth pressures, PSM, sniff transdiaphragmatic pressureand diaph EMG provide more info. Begin noninvasive ventilation when patient has nocturnal hypoventilation including dyspnea and orthopnea. , low SNP, nocturnal desaturation (< 90 % for more than 5% of sleep) or AM hypercapnia more than 6.5 kPa.

Discuss tracheotomy with patient in advance. It prolongs life but is "beyond means" of many patients, who might not want it. Lorazepan, morphine are OK but not oxygen which (unless patient is hypoxic) worsens mouth dryness and hypercapnia.

Nutrition can be assessed with dietary history, BMI, and weight. Enteric feeding can be considered with more than ten percent weight loss. Swallow studies, assessment of choking, food texture, drooling, meal duration , fatigue, video studies to assess silent aspiration. Use thickeners, lip seal, tongue exercises, chin tuck flexing neck forward when swallowing) with eating smaller more frequent meals with enough calories.

Authors emphasize role of hospice, grief couselling for families.

new seizure drugs coming up

Drugs. 2008;68(14):1925-39.

Related Articles, Links

Pharmacological management of epilepsy : recent advances and future prospects.

Johannessen Landmark C, Johannessen SI.

Department of Pharmacy, Faculty of Health Sciences, Oslo University College, Oslo, Norway.

There is still a need for new antiepileptic drugs (AEDs) as the clinical efficacy, tolerability, toxicity or pharmacokinetic properties of existing AEDs may not be satisfactory. One new AED has recently been approved (rufinamide in 2007) and six others are in late-stage development (phase III and onwards) [brivaracetam, carisbamate, eslicarbazepine, lacosamide, retigabine and stiripentol]. The purpose of this review is to provide updated data on proposed mechanisms of action, efficacy and tolerability on these new AEDs, and to discuss the rationale for their development and possible advantages compared with existing treatment, based on recent publications and MEDLINE searches.Rufinamide, brivaracetam and stiripentol have been given the status of orphan drugs. Rufinamide was approved in Europe in 2007 for the use in Lennox-Gastaut syndrome. Brivaracetam has gained orphan status for development in progressive and symptomatic myoclonic seizures in Europe and the US, respectively. Stiripentol has gained orphan status in children with Dravet's syndrome and pharmaco-resistant epilepsy. All of these drugs demonstrate efficacy as adjunctive therapy in partial seizures. Three of the drugs are derivatives of existing AEDs: brivaracetam is a derivative of levetiracetam with improved affinity for the target molecule; carisbamate is a derivative of felbamate with improved tolerability; and eslicarbazepine is a derivative of carbamazepine with less interaction potential and no auto-induction. Lacosamide, retigabine, rufinamide and stiripentol are new compounds, unrelated to other AEDs.Further investigation and development of new broad-spectrum drugs is important for improved treatment of patients with epilepsy and other neurological and psychiatric disorders.

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