A 39-year-old lady complained of epigastric pain for last seven months. Due to persistent pain, she underwent a contrast enhanced computed tomography of the abdomen which revealed a tumor in the head of the pancreas. Four months later ,she started having insomnia along with continuous twitching in bilateral calves, angle of the mouth, chin and nostrils. In addition ,she had severe bilateral leg pain which was associated with episodes of hyperhidrosis . Subsequently three months afterwards her tumor was resected. A diagnosis of solid pseudopapillary tumor of the pancreas was made on histopathology. However, her neurological symptoms persisted . On examination, the patient was distressed due to continuous leg pain. General physical examination was normal. Neurological examination revealed continuous rippling in the face, bilateral calves and tongue (Video 1). Rest of the neurological examination was normal.Her routine biochemistry, hematology and CSF examination were normal. HRCT chest did not reveal any thymoma. She underwent contrast enhanced MRI of the Brain with whole spine which was normal. Nerve conduction studies in all the four limbs were normal. Electromyography (EMG) revealed continuous spontaneous activity in the form of doublets, triplets, multiplets and fasciculation potentials in bilateral gastrocnemii and the genioglossus suggestive of peripheral nerve hyperexcitability (video 2). In view of the history, examination and EMG findings, a diagnosis of Morvan’s syndrome was suspected. Anti-CASPR2 antibodies were done and were positive. Treatment with intravenous methylprednisolone along with antiepileptics was instituted. However, her symptoms persisted, following which she received intravenous rituximab and her symptoms improved .
Morvan’s syndrome has been associated with tumors of the thymus, lung, colon and prostate.To the best of our knowledge, this is the first case of Morvan syndrome associated with a pseudopapillary pancreatic tumor to be reported. High clinical suspicion and awareness of this rare potentially treatable is required .