P1: SBT
0521779407-03 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 19:6
116 Amyotrophic Lateral Sclerosis (ALS)■Diffuse weakness, wasting & fasciculations of at least 3 limbs or 2
limbs & bulbar muscles
■Mixed upper & lower motor neuron deficit
■Babinski signs
■Bulbar palsy
■No sensory deficit
tests
■EMG: chronic denervation & reinnervation in at least 3 spinal regions
(cervical, thoracic, lumbosacral) or 2 spinal regions & bulbar muscles
■NCS: motor conduction normal or slightly slow; no conduction block
■Sensory conduction normal
■CSF normal
■Muscle biopsy: signs of denervation
differential diagnosis
■Compressive lesion excluded by imaging studies
■Familial form excluded by genetic studies
■Multifocal motor neuropathy excluded by NCS
■Infective diseases (poliovirus or West Nile virus infection) distin-
guished by acute onset & monophasic illness
management
■Ensure ventilation & nutrition
■Anticholinergics for drooling
■Baclofen or diazepam for spasticity
■Palliative care in terminal stages
specific therapy
■Riluzole may slow progression (monitor liver enzymes, blood count,
electrolytes)follow-up
■Regularly assess ventilatory function & nutritional status
➣May require semiliquid diet, nasogastric feeding, gastrotomy or
cricopharyngomyotomy
➣May require ventilator support
complications and prognosis
Complications
■Dysphagia is common
■Dysarthia or dysphonia
■Aspiration pneumonia
■Respiratory insufficiency