The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques · 2007
Multimodal evoked potentials of Kennedy's disease.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Lai Tsu-Hsien, Soong Bing-Wen, Chen Jen-Tse, Chen Yen-Yu, Lai Kuan-Lin, Wu Zin-An, Liao Kwong-Kum
Аннотация
BACKGROUND: Kennedy's disease (KD) is an X-linked recessive polyglutamine disease. Traditionally, it is a lower motor neuron syndrome with additional features such as gynecomastia and tremor. Sensory symptoms are minimal if ever present. We used multimodal evoked potential (EPs) tests to study the distribution of the involvement of the disease.
METHODS: Visual, brainstem auditory, somatosensory and motor EPs were studied in six KD patients. All of them had typical presentations and had been proved genetically.
RESULTS: Abnormal findings were noted as follows: prolonged peak latencies of visual EPs, increased hearing threshold level, inconsistent brainstem auditory EPs, decreased amplitudes of cortical potentials of somatosensory EPs, and increased motor threshold to transcranial magnetic stimulation.
CONCLUSIONS: Our multimodal EP studies showed that KD involved multiple levels of the nervous system. It implies the widespread effects of the mutant androgen receptors.
METHODS: Visual, brainstem auditory, somatosensory and motor EPs were studied in six KD patients. All of them had typical presentations and had been proved genetically.
RESULTS: Abnormal findings were noted as follows: prolonged peak latencies of visual EPs, increased hearing threshold level, inconsistent brainstem auditory EPs, decreased amplitudes of cortical potentials of somatosensory EPs, and increased motor threshold to transcranial magnetic stimulation.
CONCLUSIONS: Our multimodal EP studies showed that KD involved multiple levels of the nervous system. It implies the widespread effects of the mutant androgen receptors.
Предметные рубрики MeSH
Acoustic StimulationAdultAgedBrain StemElectric StimulationEvoked PotentialsEvoked Potentials, AuditoryEvoked Potentials, MotorEvoked Potentials, SomatosensoryEvoked Potentials, VisualHumansMaleMiddle AgedMuscular Atrophy, SpinalMutationNervous SystemNeural ConductionNeural PathwaysNeurologic ExaminationPhotic StimulationPredictive Value of TestsReceptors, AndrogenSpinal CordTranscranial Magnetic Stimulation