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Hirano's syndrome
Also known as:
Guam Parkinsonism-dementia
Guamian ALS-parkinsonism-dementia complex
Guamian amyotrophic lateral sclerosis
Associated persons:
Asao Hirano
Description:
A chronic neurodegenerative disorder highly prevalent in the native Chamorro population of Guam island in the Western Pacific. Age of onset 32 to 64 years. Insidious onset. Featueres include masklike expressionless face, stooped posture, slow posture, slow, shuffeling gait, poor coordination of alternative movements and skilled actions. There is generalized slowness of movements, mental deterioration, apathetic and indifferent depression. In some patients, symptoms and sign of amyotrophic lateral sclerosis. Cerebellar dysfunction not found.
Hirano et al in 1961 described an unusual pattern of neurofibrillary degeneration affecting the mesial temporal cortex, basal ganglia and brainstem, and designated this syndrome the parkinsonism–dementia complex. They described an identical topographical pathology in Guamanian patients with ALS, and concluded that the two clinical syndromes were different manifestations of a single disease and represented two ends of a unitary clinical spectrum.
Bibliography:
- A. Hirano, L. T. Kurland, R. S. Krooth, S. Lessel:
Parkinsonism-dementia complex, an endemic disease on Island of Guam. I. Clinical features. Brain, Oxford, 1961, 84: 642-661.
- A. Hirano, N. Malamud, L. T. Kurland:
Parkinsonism-dementia complex, an endemic disease on Island of Guam. II Pathological features. Brain, Oxford, 1961, 84: 662-679.
- N. Malamud, A. Hirano, L. T. Kurland:
Pathoanatomic changes in amyotrophic lateral sclerosis on Guam.
Archives of Neurology, Chicago, 1961, 5: 401-415.
- T. S. Elizan, A. Hirano, and B.M. Abrams:
Amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam: Neurological reevaluation. Archives of Neurology, 1966, 14:356.
- S. Matsumoto, A. Hirano, S. Goto:
Spinal cord neurofibrillary tangles of Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia complex: an immunohistochemical study.
Neurology, 1990, 40: 975–979
- S. Matsumoto, A. Hirano, S. Goto:
Ubiquitin-immunoreactive filamentous inclusions in anterior horn cells of Guamanian and non-Guamanian amyotrophic lateral sclerosis.
Acta neuropathologica, Berlin, 1990, 80: 233–238.
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