Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report
An autopsy case of sporadic amyotrophic lateral sclerosis (ALS) with lower urinary tract (LUT) and bowel dysfunction is reported. The dysfunction occurred simultaneously with motor neuron symptoms in the early stages of the illness. A 75-year-old man developed exertional dyspnea and constipation fol...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-09-01
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| Series: | eNeurologicalSci |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2405650222000223 |
| _version_ | 1817999659238424576 |
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| author | Masataka Nakamura Kentaro Nakayama Aya Murakami Satoshi Morise Satoshi Kaneko Hirofumi Kusaka Yusuke Yakushiji |
| author_facet | Masataka Nakamura Kentaro Nakayama Aya Murakami Satoshi Morise Satoshi Kaneko Hirofumi Kusaka Yusuke Yakushiji |
| author_sort | Masataka Nakamura |
| collection | DOAJ |
| description | An autopsy case of sporadic amyotrophic lateral sclerosis (ALS) with lower urinary tract (LUT) and bowel dysfunction is reported. The dysfunction occurred simultaneously with motor neuron symptoms in the early stages of the illness. A 75-year-old man developed exertional dyspnea and constipation following weight loss. Subsequently, he developed swallowing disturbance, fecal incontinence, and urinary retention. Neurological examination showed dysphagia, muscle weakness of the upper limbs, and prominent fasciculation affecting all four limbs and the tongue. All deep tendon reflexes were diminished, but the left plantar response was extensor. Orthostatic hypotension (OH) and the anal reflex were absent. Neuropathological findings did not show neuronal loss and gliosis in the thoracic and sacral intermediolateral nucleus (IML) and in Onuf's nucleus, whereas gliosis was observed in the periaqueductal gray (PAG) and striatum. Therefore, urinary retention may have resulted from involvement of the PAG. Phosphorylated TAR DNA binding protein 43 kDa (p-TDP-43)-positive inclusions were present in the peripheral nerves within the thoracic sympathetic ganglia, as well as the IML of the thoracic spinal cord. However, considering the lack of OH, the IML and peripheral sympathetic nerves unlikely played major roles. Furthermore, neuronal loss or p-TDP-43-immunoreactive deposits were absent in the Auerbach and Meissner plexuses of the rectum, suggesting that the responsible anatomical sites for fecal incontinence could not be found. Although it is difficult to elucidate the precise neuropathological lesions corresponding to LUT and bowel dysfunction, physicians need to recognize that neurogenic bladder and bowel dysfunction can occur in patients with ALS. |
| first_indexed | 2024-04-14T03:11:39Z |
| format | Article |
| id | doaj.art-73ff5df20d1549cd96d8c0e0c45cf3fc |
| institution | Directory Open Access Journal |
| issn | 2405-6502 |
| language | English |
| last_indexed | 2024-04-14T03:11:39Z |
| publishDate | 2022-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | eNeurologicalSci |
| spelling | doaj.art-73ff5df20d1549cd96d8c0e0c45cf3fc2022-12-22T02:15:35ZengElseviereNeurologicalSci2405-65022022-09-0128100413Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case reportMasataka Nakamura0Kentaro Nakayama1Aya Murakami2Satoshi Morise3Satoshi Kaneko4Hirofumi Kusaka5Yusuke Yakushiji6Corresponding author.; Department of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanDepartment of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanDepartment of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanDepartment of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanDepartment of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanDepartment of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanDepartment of Neurology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka 573-1010, JapanAn autopsy case of sporadic amyotrophic lateral sclerosis (ALS) with lower urinary tract (LUT) and bowel dysfunction is reported. The dysfunction occurred simultaneously with motor neuron symptoms in the early stages of the illness. A 75-year-old man developed exertional dyspnea and constipation following weight loss. Subsequently, he developed swallowing disturbance, fecal incontinence, and urinary retention. Neurological examination showed dysphagia, muscle weakness of the upper limbs, and prominent fasciculation affecting all four limbs and the tongue. All deep tendon reflexes were diminished, but the left plantar response was extensor. Orthostatic hypotension (OH) and the anal reflex were absent. Neuropathological findings did not show neuronal loss and gliosis in the thoracic and sacral intermediolateral nucleus (IML) and in Onuf's nucleus, whereas gliosis was observed in the periaqueductal gray (PAG) and striatum. Therefore, urinary retention may have resulted from involvement of the PAG. Phosphorylated TAR DNA binding protein 43 kDa (p-TDP-43)-positive inclusions were present in the peripheral nerves within the thoracic sympathetic ganglia, as well as the IML of the thoracic spinal cord. However, considering the lack of OH, the IML and peripheral sympathetic nerves unlikely played major roles. Furthermore, neuronal loss or p-TDP-43-immunoreactive deposits were absent in the Auerbach and Meissner plexuses of the rectum, suggesting that the responsible anatomical sites for fecal incontinence could not be found. Although it is difficult to elucidate the precise neuropathological lesions corresponding to LUT and bowel dysfunction, physicians need to recognize that neurogenic bladder and bowel dysfunction can occur in patients with ALS.http://www.sciencedirect.com/science/article/pii/S2405650222000223ALSUrinary retentionFecal incontinenceTDP-43Lower urinary tract dysfunctionAnd bowel dysfunction |
| spellingShingle | Masataka Nakamura Kentaro Nakayama Aya Murakami Satoshi Morise Satoshi Kaneko Hirofumi Kusaka Yusuke Yakushiji Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report eNeurologicalSci ALS Urinary retention Fecal incontinence TDP-43 Lower urinary tract dysfunction And bowel dysfunction |
| title | Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report |
| title_full | Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report |
| title_fullStr | Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report |
| title_full_unstemmed | Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report |
| title_short | Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report |
| title_sort | early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis a case report |
| topic | ALS Urinary retention Fecal incontinence TDP-43 Lower urinary tract dysfunction And bowel dysfunction |
| url | http://www.sciencedirect.com/science/article/pii/S2405650222000223 |
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