Motor neuron disease‐inclusion dementia presenting as cortical‐basal ganglionic degeneration
Identifieur interne : 003736 ( Main/Exploration ); précédent : 003735; suivant : 003737Motor neuron disease‐inclusion dementia presenting as cortical‐basal ganglionic degeneration
Auteurs : David A. Grimes [Canada] ; Catherine B. Bergeron [Canada] ; Anthony E. Lang [Canada]Source :
- Movement Disorders [ 0885-3185 ] ; 1999-07.
English descriptors
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Abstract
The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal‐temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease‐inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin‐immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical‐basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.
Url:
DOI: 10.1002/1531-8257(199907)14:4<674::AID-MDS1019>3.0.CO;2-X
Affiliations:
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The predominant frontal‐temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease‐inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin‐immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. 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