Dataset Information

Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: case report.

ABSTRACT:

Background

We report a rare case of an 18-year-old male with unilateral hand tremor who was finally diagnosed with Hirayama disease (HD).

Case presentation

An 18-year-old male presented with unilateral polymyoclonus that aggravated with neck flexion. The patient did not complain of muscle weakness or muscle atrophy. The needle electromyography showed giant motor unit potentials in right cervical 7 and 8 innervated muscles. The cervical magnetic resonance imaging on supine and flexion state showed prominent forward effacement of posterior dural sac that was compatible with HD.

Conclusions

HD usually presents with unilateral distal hand weakness, muscle atrophy and tremor. Although it is a benign and self-limiting disease, early diagnosis may lead to less clinical deterioration. Moreover, electromyography should be completed in the differentiation of young male patients who present with polymyoclonus without hand weakness or atrophy.

SUBMITTER: Kim JY

PROVIDER: S-EPMC7460763 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Publications

Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: case report.

Kim Jun-Young JY Hwang Su-Keong SK Kwon Soonhak S Park Jin-Sung JS

BMC neurology 20200901 1

<h4>Background</h4>We report a rare case of an 18-year-old male with unilateral hand tremor who was finally diagnosed with Hirayama disease (HD).<h4>Case presentation</h4>An 18-year-old male presented with unilateral polymyoclonus that aggravated with neck flexion. The patient did not complain of muscle weakness or muscle atrophy. The needle electromyography showed giant motor unit potentials in right cervical 7 and 8 innervated muscles. The cervical magnetic resonance imaging on supine and flex ...[more]

PMID: 32873232

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Project description:Introduction and importanceEchinococcus granulosus causes hydatid disease. The most affected organ is the liver which is followed by the lungs. The pleural cavity being the primary location of hydatid cysts is rare and should be discussed further. This paper documents a rare case of primary pleural hydatidosis which can present with a merely isolated cough followed by dyspnea. The diagnosis and surgical treatment along with post-operative medications are vital in this case.Case presentationWe present a case of a 45-year-old who suffered from a cough for more than one week which did not subside after taking medications. This symptom was followed by dyspnoea for which an X-ray was done which showed left-sided pleural effusion, a complication of pleural hydatidosis. Computed tomography showed multiple cysts in the pleural cavity which confirmed the diagnosis of primary pleural hydatidosis as the cysts were not present in any other sites. Blood work revealed eosinophilia which is significant in parasitic diseases. A left posterolateral thoracotomy was performed, and the cysts were surgically removed. Additionally, empyemectomy and pleurectomy were done. The patient was then treated with anti-parasitic therapy and was advised to get X-rays during the follow-up visits. The X-rays were normal and indicated that there was no disease recurrence.Clinical discussionEchinococcus granulosus is a parasitic worm that causes hydatid disease. The primary location is the liver. A diagnosis of intrathoracic but extrapulmonary disease, which involves the presence of hydatid cysts in the pleura, heart, pericardium, mediastinum, chest wall, and diaphragm, is difficult in individuals lacking a primary cyst in a common location (Isitmangil et al., 2003; Saeedan et al., 2020).ConclusionThis case implies the significance of a cough of more than a week that is not relieved by medications. This should be carefully evaluated and followed in cases that have a rare diagnosis requiring surgery. A diagnosis of primary pleural hydatidosis with left-sided pleural effusion and atelectasis with mediastinal shift to the right side was made which was treated with a surgical procedure.

Dataset Information

Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: case report.

Background

Case presentation

Conclusions

Publications

Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: case report.

Similar Datasets

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