Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration

The frontotemporal dementias are a group of relatively new and evolving cli nical and pathologic entities. The predominant frontal-temporal atrophy cau ses a variety of clinical syndromes, usually dominated by disturbances in b ehavior, mood, and speech. The motor neuron disease-inclusion dementia (MND ID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immuno reactivity. We present a patient with the clinical and neuroimaging charact eristics of a highly asymmetric neurodegenerative condition distinguished b y limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cort ical sensory findings, and limb apraxia. His premorbid diagnosis was cortic al-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.