A TYPICAL FORMS OF ALS: REVIEW AND OBSERVATION FROM PRACTICE

Abstract

The combination of parkinsonism with other neurological disorders, such as
autonomic failure, dementia, cerebellar ataxia, visual disturbances, pyramidal syndrome,
is characteristic of some neurodegenerative diseases, for example, multisystem atrophy,
dementia with Lewy bodies, progressive supranuclear palsy, corticobasal degeneration.
These diseases are often found in the practice of a neurologist, have a detailed description
and clear diagnostic criteria.
The isolated combination of parkinsonism and ALS (without other neurological disorders)
is extremely rare and is called Bright-Fan-Schwarz disease in honor of the scientists who
first reported this cross-syndrome.
We did not find descriptions of cases of familial neurodegenerative disease with
parkinsonism and ALS in the literature. This article presents the authors' own observation
of three siblings, one of which had parkinsonism with ALS syndrome, and the other two
had Parkinson's disease.
The combination of amyotrophic lateral sclerosis (ALS) with Parkinson's syndrome and
dementia is described as Guam-type ALS, in which up to 70% of patients have a positive
family history.