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• Vol.42 No.10
• CLINICA NEUROL, 42: 963−965, 2002
• Vol.42 No.10 contents

Brief Clinical Note

A case of motor neuron disease with dementia and apraxia of the upper limbs

Bungo Okuda, M.D.¹⁾²⁾, Yasumichi Iwamoto, M.D.¹⁾, Nobuyuki Oka, M.D.¹⁾ and Hisao Tachibana, M.D.¹⁾

¹⁾Division of Neurology, Department of Internal Medicine, Hyogo College of Medicine,
²⁾Department of Neurology, Ehime Prefectural Central Hospital

We report a 61-year-old, right-handed woman with motor neuron disease, dementia, and apraxia of the upper limbs. The patient developed clumsiness of the right hand and dysarthria two years and a half prior to admission. Neurological examination showed limb-kinetic apraxia and ideomotor apraxia, predominantly on the right side, in addition to dementia and anarthria. There was mild muscle wasting in the neck and hands. A muscle biopsy from the biceps muscle of arm as well as needle EMG revealed neurogenic changes compatible with motor neuron disease. Brain MRI indicated pyramidal tract degeneration. Three-dimensional brain perfusion imaging generated from SPECT demonstrated an asymmetric decrease in cerebral blood flow in the fronto-temporo-parietal regions, predominantly on the left side. This case suggests that asymmetric limb apraxia can be associated with motor neuron disease.

(CLINICA NEUROL, 42: 963−965, 2002)
key words: dementia, limb apraxia, motor neuron disease, cerebral blood flow

(Received: 14-Mar-02)