Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report
<i>Background and Objectives</i>: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-08-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/59/8/1495 |
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| author | Ieva Navickaitė Miglė Ališauskienė Sandra Petrauskienė Gintarė Žemgulytė |
| author_facet | Ieva Navickaitė Miglė Ališauskienė Sandra Petrauskienė Gintarė Žemgulytė |
| author_sort | Ieva Navickaitė |
| collection | DOAJ |
| description | <i>Background and Objectives</i>: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. <i>Case presentation</i>: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. <i>Conclusions</i>: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions. |
| first_indexed | 2024-03-10T23:45:11Z |
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| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-10T23:45:11Z |
| publishDate | 2023-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-0819cfbca3f94c2186e90329751e91312023-11-19T02:07:13ZengMDPI AGMedicina1010-660X1648-91442023-08-01598149510.3390/medicina59081495Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case ReportIeva Navickaitė0Miglė Ališauskienė1Sandra Petrauskienė2Gintarė Žemgulytė3Department of Neurology, Medical Academy, Lithuanian University of Health Sciences, A. Mickeviciaus Str. 9, LT-44307 Kaunas, LithuaniaDepartment of Neurology, Medical Academy, Lithuanian University of Health Sciences, A. Mickeviciaus Str. 9, LT-44307 Kaunas, LithuaniaDepartment of Preventive and Paediatric Dentistry, Lithuanian University of Health Sciences, Luksos-Daumanto Str. 6, LT-50106 Kaunas, LithuaniaDepartment of Neurology, Medical Academy, Lithuanian University of Health Sciences, A. Mickeviciaus Str. 9, LT-44307 Kaunas, Lithuania<i>Background and Objectives</i>: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. <i>Case presentation</i>: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. <i>Conclusions</i>: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions.https://www.mdpi.com/1648-9144/59/8/1495ganglionopathysmall-fiber neuropathyHarlequin syndromesarcoidosis |
| spellingShingle | Ieva Navickaitė Miglė Ališauskienė Sandra Petrauskienė Gintarė Žemgulytė Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report Medicina ganglionopathy small-fiber neuropathy Harlequin syndrome sarcoidosis |
| title | Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report |
| title_full | Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report |
| title_fullStr | Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report |
| title_full_unstemmed | Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report |
| title_short | Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report |
| title_sort | sarcoidosis associated sensory ganglionopathy and harlequin syndrome a case report |
| topic | ganglionopathy small-fiber neuropathy Harlequin syndrome sarcoidosis |
| url | https://www.mdpi.com/1648-9144/59/8/1495 |
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