Hereditary spastic paraplegia with frontal lobe dysfunction: a clinicopathologic study.
The authors report an unusual family with hereditary spastic paraplegia (HSP) with frontal lobe dysfunction having the onset in the sixth decade. All the patients showed hypoperfusion in the frontal lobes and thalami on SPECT. Neuropathologic findings revealed thin corpus callosum and degeneration in the thalamic dorsomedial nuclei as well as degeneration of the corticospinal tracts. This family was likely affected by a novel form of HSP characterized by frontal lobe dysfunction caused by thalamic degeneration.
Yanase
D
D
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1, Takara-machi, Kanazawa 920-8640, Japan. d.yanase@med.kanazawa-u.ac.jp
Komai
K
K
Hamaguchi
T
T
Okino
S
S
Yokoji
H
H
Makifuchi
T
T
Takano
H
H
Yamada
M
M
D000328:Adult; D017668:Age of Onset; D000368:Aged; D001284:Atrophy; D003704:Dementia / Q000209:etiology* / Q000473:pathology; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005625:Frontal Lobe / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D005911:Gliosis / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008569:Memory Disorders / Q000209:etiology / Q000473:pathology; D008875:Middle Aged; D019964:Mood Disorders / Q000209:etiology / Q000473:pathology; D011712:Pyramidal Tracts / Q000473:pathology; D012720:Severity of Illness Index; D015419:Spastic Paraplegia, Hereditary / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D013788:Thalamus / Q000000981:diagnostic imaging / Q000473:pathology*; D015899:Tomography, Emission-Computed, Single-Photon