| Article Access Statistics | | | Viewed | 3510 | | | Printed | 59 | | | Emailed | 0 | | | PDF Downloaded | 86 | | | Comments | [Add] | | | Cited by others | 5 | | |
|
Click on image for details.
|
|
 |
| CASE REPORT |
|
|
|
| Year : 2013 | Volume
: 61
| Issue : 3 | Page : 300-302 |
Morvan syndrome: After scrotal sac drainage and chemical instillation in hydrocele
Sanjay Sharma, Pranita Sharma
Department of Neurology, Ramkrishna Care Hospital, Raipur, Chhattisgarh, India
| Date of Submission | 05-Feb-2013 |
| Date of Decision | 11-Feb-2013 |
| Date of Acceptance | 07-Jun-2013 |
| Date of Web Publication | 16-Jul-2013 |
Correspondence Address:
Sanjay Sharma
Department of Neurology, Ramkrishna Care Hospital, Raipur, Chhattisgarh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.115072
We have described the occurrence of Morvan syndrome (MoS) after scrotal tap and injection of sclerosing agent for the treatment of hydrocele in 5 male. The mean age was 43.2 years, and the gap between the procedure and development of clinical features suggestive of MoS was 1.5-3 months. The neurophysiology studies demonstrated hyper excitability of peripheral nerves. In addition, autonomic dysfunction, severe insomnia, and neuropsychiatric features were observed. Significant VGKC-complex/CASPR-2 antibodies titer were present in all cases. Symptoms resolved spontaneously within 6 months of onset.
Keywords: CASPR-2 antibodies, hydrocele, Morvan syndrome, sclerosing agent, VGKC-complex antibody
How to cite this article:
Sharma S, Sharma P. Morvan syndrome: After scrotal sac drainage and chemical instillation in hydrocele. Neurol India 2013;61:300-2 |
| » Introduction | |  |
Morvan syndrome (MoS) is characterized by the presence of neuromyotonia, dysautonomia, and encephalopathy. [1] The syndrome is associated with the formation of voltage-gated potassium channel complex antibodies (VGKC-Complex Abs), most commonly directed against contactin associated protein-2 (CASPR-2). [2],[3] The neurophysiology studies demonstrate hyperexcitability of peripheral nerves, autonomic dysfunction, and severe insomnia in sleep study. Neuroimaging is usually normal. [4] The male preponderance is striking in MoS, and thymoma was seen in 37.9-41.0%. [5],[6] We have described 5 male patients who developed MoS after scrotal drainage and instillation of chemicals in to the scrotal sac.
| » Case Report | | |
Five male underwent scrotal drainage and sclerotherapy for treatment of hydrocele. All of them developed clinical features of scrotal inflammation after the procedure. After a gap of 1.5-3 months, they developed pain involving back and lower extremities, excessive sweating, and insomnia. Needle electromyography was performed in all patients and polysomnography done in 1 patient. Scrotal sonography was performed to search for evidence of inflammation in all cases. Sera of 3 patients were tested for VGKC-complex antibodies using the immune-precipitation assay. A titer above 100 pM was considered significant. CASPR2 antibodies were tested in 2 cases and were positive in both. One patient (KB) who underwent serial serum evaluation to judge sequential titer showed gradual fall in VGKC-complex antibodies titer. The symptoms gradually resolved over a period of 6 months. Clinical presentation and investigation are detailed in [Table 1].
| » Discussion | | |
MoS are rarely reported in India, mostly as a case report. [7] A series of 20 patients who acquired neuromyotonia, as described by Panagariya et al., from northwest India included 19 male, and 11 of these patients received indigenous Ayurvedic drugs. VGKC antibody estimation in a single patient revealed a high titer of 1028 pM. None of them were classified as MOS, although sleep disorders, restlessness, perspiration, tachycardia, and raised temperature were noted in many of the patients. [8] Earlier case reports of MoS involved gold and inorganic mercury. These heavy metals either directly harm neuron or indirectly via stimulation of the immune system. [9],[10]
Irani et al., reported clinical and serological observation on 29 cases of MoS. The reported series also included two of our present cases. [5] We reported occurrence of MoS after scrotal tap and instillation of lignocaine and denatured spirit into the scrotal sac in 9 patients; however, they were not tested for VGKC antibody. The pathogenesis of syndrome was unclear and the course of illness was self-limiting. [11] In the present series, all patients had evidence of persistent scrotal inflammation and had higher titer of VGKC complex antibodies. Two of them were positive against CASPR-2 antigen. Declining level of antibodies noted on sequential estimation was done in 1 patient. This may explain self-limiting course of illness. Low serum sodium level is noted in two cases and serum hyponatremia is reported in 25% cases with MoS. [5] Sleep study performed in only one patient was normal, but it was done late in the course of illness.
The striking presentation of MoS after scrotal drainage and intra-scrotal chemical injection is intriguing. CASPR2 is localized and associates with K + channels at the juxta paranodal region along myelinated axons. [12] Although CASPR2 is predominantly expressed in the nervous system, low level of CASPR2 mRNA has been demonstrated in the human prostate. The male reproductive system may be the source of antigen. [5],[13] One patient with MoS and prostate adenoma reported in literature, who also had in situ carcinoma in sigmoid polyp. [14] Introduction of chemical in to sac and scrotal inflammation in our cases may be crucial factor to break immune tolerance. The present case further strengthens our observations on MoS syndrome associated with high VGKC antibodies after hydrocele tapping and intra-scrotal injection of a variety of chemical substances. It is often difficult to obtain information regarding the chemical used by treating person. This syndrome is not frequently recognized as hydrocele tapping by quack is kept secret by the patient, and it is very unlikely to be revealed without direct questioning.
| » Acknowledgement | | |
We thank Dr. Sarosh Irani and Dr. Angela Vincent, John Radcliffe Hospital UK for performing VGKC antibody estimation in 3 cases and suggestion on manuscript. We also thank Dr. Ajay Parashar for uro-surgical evaluation of patients, Drs. Rajesh Gupta and Abbas Naqvi for their contribution of cases.
| » References | | |
| 1. | Newsom-Davis J, Buckley C, Clover L, Hart I, Maddison P, Tuzum E, et al. Autoimmune disorders of neuronal potassium channels. Ann N Y Acad Sci 2003;998:202-10. 
|
| 2. | Vincent A. Antibodies to contacting-associated protein-2 (CASPR2) in thymoma and Morvan's syndrome. Ann Neurol 2009;66(Suppl 13):S3.
|
| 3. | Irani SR, Waters P, Kleopa K, Nicosia C, Lang B, Vincent A. Antibodies to components of the voltage-gated potassium channel-associated complex: LGI1 and CASPR2 as antigenic targets in limbic encephalitis, Morvan's and neuromyotonia. Neurology 2010;75:379.
|
| 4. | Keith JA, Michael SH, Robert FD, Todd NB, Raymond AG, Steven V. Neurophysiologic studies in Morvan syndrome. J Clin Neurophysiol 2004;21:440-5.
|
| 5. | Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C, et al. Morvan syndrome: Clinical and serological observations in 29 cases. Ann Neurol 2012;72:241-55.
[PUBMED] |
| 6. | Merchut MP. Management of voltage-gated potassium channel antibody disorders. Neurol Clin 2010;28:941-59.
[PUBMED] |
| 7. | Bajaj BK, Shrestha S. An interesting case report of Morvan's syndrome from the Indian subcontinent. Neurol India 2007;55:67-9.
[PUBMED]  |
| 8. | Panagariya A, Kumar H, Mathew V, Sharma B. Neuromyotonia: Clinical profile of twenty cases from North West India. Neurol India 2006;54:382-6.
[PUBMED] |
| 9. | Mitsumoto H, Wilbourn AJ. Generalized myokymia and gold therapy. Arch Neurol 1982;39:449-50.
|
| 10. | Gil R, Lefevre JP, Neau JP. Morvan's fibrillary chorea and acrodynic syndrome following mercury treatment. Rev Neurol (Paris) 1984;140:728-33.
|
| 11. | Sharma S, Bhoi KK, Sharma P, Anant S, Parashar A. Morvan's syndrome after intra-scrotal injection of lignocaine and denatured spirit for hydrocoele. Neurol Asia 2010;15:133-5.
|
| 12. | Poliak S, Gollan L, Martinez R, Custer A, Einheber S, Salzer JL, et al. CASPR2, a new member of the neurexin superfamily is localized at the juxtaparanodes of myelinated axons and associates with K + channels. Neuron 1999;24:1037-47.
|
| 13. | Poliak S, Gollan L, Salomon D, Berglund EO, Ohara R, Ranscht B, et al. Localization of CASPR2 in myelinated nerves depends on axon-glia interactions and the generation of barriers along the axon. J Neurosci 2001;21:7568-75.
[PUBMED] |
| 14. | Madrid A, Gil-Peralta A, Gil-Neciga E, Gonzalez JR, Jarrin S. Morvan's fibrillary chorea: Remission after plasmapheresis. J Neurol 1996;243:350-3.
|
[Table 1]
| This article has been cited by | | 1 |
Paraneoplastic Morvanæs syndrome following surgical treatment of recurrent thymoma: A case report |
|
| Edvina Galié,Rosaria Renna,Domenico Plantone,Andrea Pace,Mirella Marino,Bruno Jandolo,Tatiana Koudriavtseva | | Oncology Letters. 2016; 12(4): 2716 | | [Pubmed] | [DOI] | | | 2 |
A Case of Morvan Syndrome Mimicking Amyotrophic Lateral Sclerosis With Frontotemporal Dementia |
|
| Brin Freund,Manoj Maddali,Thomas E. Lloyd | | Journal of Clinical Neuromuscular Disease. 2016; 17(4): 207 | | [Pubmed] | [DOI] | | | 3 |
Clinical and experimental studies of potentially pathogenic brain-directed autoantibodies: current knowledge and future directions |
|
| James Varley,Angela Vincent,Sarosh R. Irani | | Journal of Neurology. 2015; 262(4): 1081 | | [Pubmed] | [DOI] | | | 4 |
Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma |
|
| Chih-Hong Lee,Jainn-Jim Lin,Kun-Ju Lin,Bao-Luen Chang,Hsiang-Yao Hsieh,Wei-Hsun Chen,Kuang-Lin Lin,Hon-Chung Fung,Tony Wu | | Journal of the Neurological Sciences. 2014; 341(1-2): 36 | | [Pubmed] | [DOI] | | | 5 |
Ceftriaxone/lidocaine |
|
| | | Reactions Weekly. 2013; 1475(1): 12 | | [Pubmed] | [DOI] | |
|
|
|
|
|
|
|
|
