Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration.
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.
Grimes
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DA
The Morton and Gloria Shulman Movement Disorder Centre, Toronto Western Hospital, Ontario, Canada.
Bergeron
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CB
Lang
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AE
D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D001828:Body Image; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D003704:Dementia / Q000145:classification / Q000175:diagnosis / Q000209:etiology*; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D009069:Movement Disorders / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D014836:Volition / Q000502:physiology