Primary lateral sclerosis presenting parkinsonian symptoms without nigrostriatal involvement.
We encountered three patients with primary lateral sclerosis (PLS) showing bradykinesia, frozen gait, and severe postural instability, as well as slowly progressive spinobulbar spasticity. Cranial magnetic resonance (MR) imaging showed precentral gyrus atrophy. Central motor conduction was markedly prolonged or failed to evoke a response. Positron emission tomography (PET) showed significant reduction of [18F]fluoro-2-deoxy-D-glucose uptake in the area of the precentral gyrus extending to the prefrontal, medial frontal, and cingulate areas. No abnormalities were seen in the nigrostriatal system with PET using [18F]fluorodopa or [11C]raclopride or with proton MR spectroscopy. Thus, widespread prefrontal, medial, and cingulate frontal lobe involvement can be associated with the parkinsonian symptoms in PLS.
Mabuchi
N
N
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Watanabe
H
H
Atsuta
N
N
Hirayama
M
M
Ito
H
H
Fukatsu
H
H
Kato
T
T
Ito
K
K
Sobue
G
G
D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology; D019788:Fluorodeoxyglucose F18; D020233:Gait Disorders, Neurologic / Q000209:etiology; D006801:Humans; D018476:Hypokinesia / Q000209:etiology; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D020734:Parkinsonian Disorders / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D049268:Positron-Emission Tomography; D011187:Posture; D019275:Radiopharmaceuticals
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18