Internal Medicine

0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:16

Motor Neuron Diseases 999

➣Pseudobulbar palsy: upper motor neuron deficit in bulbar mus-

cles

➣Amyotrophic lateral sclerosis: mixed upper & lower motor neu-

ron deficit in limbs

tests

■EMG: chronic partial degeneration & reinnervation

■NCS: normal motor & sensory conduction studies

➣No evidence of motor conduction block

■CSF: normal

■Serum & WBC hexosaminidase A: normal

■Serum protein electrophoresis & immunoelectrophoresis: normal

differential diagnosis

■Hexosaminidase deficiency or monoclonal gammopathy may be

associated w/ similar clinical picture

■Multifocal motor neuropathy excluded by NCS

■Compressive lesion excluded by imaging studies

■Familial forms excluded by genetic studies

management

■Ensure ventilation & nutrition

■Anticholinergics for drooling

■Baclofen or diazepam for spasticity

■Palliative care in terminal stages

specific therapy

■Riluzole may slow progression of ALS (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

■Dysphagia is common

■Dysarthia or dysphonia

■Aspiration pneumonia

■Respiratory insufficiency