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[An autopsied case of Parkinson's disease manifesting Shy-Drager syndrome].
Rinsho Shinkeigaku. 1992 Nov; 32(11):1238-44.RS

Abstract

We report an autopsied case of Parkinson's disease manifesting Shy-Drager syndrome. At the age of 63 years, the patient noticed an onset of progressive orthostatic dizziness, which was followed by constipation, dysuria, and sexual impotence. When he was 66 years old, syncopal attack for a few minutes, tremor in the bilateral hands, and memory disturbance developed. On admission, his blood pressure was 142/72 mmHg in supine position, which fell to 58/42 mmHg on standing with appropriate increase of heart rate. Neurological examination revealed hallucination, memory disturbance, masked face, muscular rigidity, bradykinesia, mild postural tremor, and autonomic dysfunction including severe orthostatic hypotension, hypohydrosis, constipation, dysuria, and sexual impotence. Electroencephalogram showed diffuse slowing. Brain CT demonstrated absence of severe atrophy of the cerebellum, and brain stem. Pharmacological study revealed denervation hypersensitivity to the intravenously administrated noradrenaline. A diagnosis of Shy-Drager syndrome was made, and he was treated with anti parkinsonian drugs. However, no improvement was observed in his clinical symptoms. Seven months later, he died of pneumonia. Neuropathological examination revealed marked neuronal cell loss and gliosis in the substantia nigra and locus ceruleus. Lewy bodies were seen in those pigmented nuclei, dorsal vagal nucleus, hypothalamus and nucleus basalis of Meynert. No abnormality was found in the intermediolateral nucleus of the spinal cord. This is the first report on a Japanese patient who presented clinically Shy-Drager syndrome and pathologically typical Parkinson's disease. In this patient, from the pharmacological and pathological findings, sympathetic ganglia were supposed to be the responsible lesion for orthostatic hypotension.

Authors+Show Affiliations

Department of Neurology, Tsuchiura Kyodou Hospital.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

1301325

Citation

Saito, F, et al. "[An Autopsied Case of Parkinson's Disease Manifesting Shy-Drager Syndrome]." Rinsho Shinkeigaku = Clinical Neurology, vol. 32, no. 11, 1992, pp. 1238-44.
Saito F, Tsuchiya K, Kotera M. [An autopsied case of Parkinson's disease manifesting Shy-Drager syndrome]. Rinsho Shinkeigaku. 1992;32(11):1238-44.
Saito, F., Tsuchiya, K., & Kotera, M. (1992). [An autopsied case of Parkinson's disease manifesting Shy-Drager syndrome]. Rinsho Shinkeigaku = Clinical Neurology, 32(11), 1238-44.
Saito F, Tsuchiya K, Kotera M. [An Autopsied Case of Parkinson's Disease Manifesting Shy-Drager Syndrome]. Rinsho Shinkeigaku. 1992;32(11):1238-44. PubMed PMID: 1301325.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [An autopsied case of Parkinson's disease manifesting Shy-Drager syndrome]. AU - Saito,F, AU - Tsuchiya,K, AU - Kotera,M, PY - 1992/11/1/pubmed PY - 1992/11/1/medline PY - 1992/11/1/entrez SP - 1238 EP - 44 JF - Rinsho shinkeigaku = Clinical neurology JO - Rinsho Shinkeigaku VL - 32 IS - 11 N2 - We report an autopsied case of Parkinson's disease manifesting Shy-Drager syndrome. At the age of 63 years, the patient noticed an onset of progressive orthostatic dizziness, which was followed by constipation, dysuria, and sexual impotence. When he was 66 years old, syncopal attack for a few minutes, tremor in the bilateral hands, and memory disturbance developed. On admission, his blood pressure was 142/72 mmHg in supine position, which fell to 58/42 mmHg on standing with appropriate increase of heart rate. Neurological examination revealed hallucination, memory disturbance, masked face, muscular rigidity, bradykinesia, mild postural tremor, and autonomic dysfunction including severe orthostatic hypotension, hypohydrosis, constipation, dysuria, and sexual impotence. Electroencephalogram showed diffuse slowing. Brain CT demonstrated absence of severe atrophy of the cerebellum, and brain stem. Pharmacological study revealed denervation hypersensitivity to the intravenously administrated noradrenaline. A diagnosis of Shy-Drager syndrome was made, and he was treated with anti parkinsonian drugs. However, no improvement was observed in his clinical symptoms. Seven months later, he died of pneumonia. Neuropathological examination revealed marked neuronal cell loss and gliosis in the substantia nigra and locus ceruleus. Lewy bodies were seen in those pigmented nuclei, dorsal vagal nucleus, hypothalamus and nucleus basalis of Meynert. No abnormality was found in the intermediolateral nucleus of the spinal cord. This is the first report on a Japanese patient who presented clinically Shy-Drager syndrome and pathologically typical Parkinson's disease. In this patient, from the pharmacological and pathological findings, sympathetic ganglia were supposed to be the responsible lesion for orthostatic hypotension. SN - 0009-918X UR - https://www.unboundmedicine.com/medline/citation/1301325/[An_autopsied_case_of_Parkinson's_disease_manifesting_Shy_Drager_syndrome]_ L2 - https://www.diseaseinfosearch.org/result/6584 DB - PRIME DP - Unbound Medicine ER -
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