Extrapyramidal involvement in amyotrophic lateral sclerosis: backward falls and retropulsion
- Professor Michael Swash, Department of Neurology, The Royal London Hospital, Whitechapel, London E1 1BB, UK. Telephone 0044 171 377 7472; fax 0044 171 377 7318; email mswash{at}mds.qmw.ac.uk andmswash{at}btinternet.com
- Received 9 November 1998
- Revised 20 January 1999
- Accepted 24 January 1999
Abstract
Three patients with sporadic amyotrophic lateral sclerosis (ALS) presented with a history of backward falls. Impaired postural reflexes and retropulsion accompanied clinical features of ALS. Hypokinesia, decreased arm swing, and a positive glabellar tap were noted in two of these three patients. Cognitive impairment, tremor, axial rigidity, sphincter dysfunction, nuchal dystonia, dysautonomia, and oculomotor dysfunction were absent. Brain MRI disclosed bilateral T2 weighted hyperintensities in the internal capsule and globus pallidus in one patient. Necropsy studies performed late in the course of ALS have shown degeneration in extrapyramidal sites—for example, the globus pallidus, thalamus, and substantia nigra. Clinically, backward falls and retropulsion may occur early in ALS. This may reflect extrapyramidal involvement.
