Abstract
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.
Publication types
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Case Reports
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Clinical Conference
MeSH terms
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Adult
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Age of Onset
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Aged
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Atrophy
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Dementia / etiology*
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Dementia / pathology
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Disease Progression
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Fatal Outcome
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Female
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Frontal Lobe / diagnostic imaging
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Frontal Lobe / pathology*
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Frontal Lobe / physiopathology
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Gliosis / etiology
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Humans
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Magnetic Resonance Imaging
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Memory Disorders / etiology
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Memory Disorders / pathology
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Middle Aged
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Mood Disorders / etiology
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Mood Disorders / pathology
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Pyramidal Tracts / pathology
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Severity of Illness Index
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Spastic Paraplegia, Hereditary / complications
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Spastic Paraplegia, Hereditary / diagnostic imaging
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Spastic Paraplegia, Hereditary / pathology*
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Spastic Paraplegia, Hereditary / physiopathology
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Thalamus / diagnostic imaging
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Thalamus / pathology*
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Tomography, Emission-Computed, Single-Photon