Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Letter to the Editor Eur Neurol 2005;54:235–237 DOI: 10.1159/000090719 Fasciculations, Autonomic Symptoms and Limbic Encephalitis: A Thymoma-Associated Morvan’s-Like Syndrome Feza Deymeer a Sukriye Akca a Gulsen Kocaman a Yesim Parman a Piraye Serdaroglu a Oget Oktem-Tanor a Oguzhan Coban a Angela Vincent b a Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey; b Neurosciences Group, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK ties, tremor in the hands, excessive sweating in the head and hands, impotence, urinary urgency, and insomnia. Physical examina- tion revealed tachycardia and hyperhidrosis. Neurological examination was normal ex- cept for fasciculations in the lower extremi- ties and a moderately diminished vibration sense in the toes. There was no postural hypotension. Needle electromyography showed fasciculations in the gastrocnemius muscle. Nerve conduction studies were nor- mal. Autonomic tests revealed decreased RR interval variability and absent sympa- thetic skin responses. A detailed cardiolog- ical evaluation was normal except for sinus tachycardia with a mean heart rate of 110/min during 24 h Holter monitoring. One month after the onset of symptoms, there was a sudden onset of memory deficit. Neuropsychological evaluation (Wechsler Memory Scale Digit Span, Logical Memory and Visual Reproduction subtests, back- ward counting, category fluency test, Wechsler Adult Intelligence Scale-Revised similarities subtest, Stroop test, verbal memory processes test, Luria’s alternating sequences) revealed a defect in primary vi- sual memory registration with an attention deficit, but intact primary verbal memory. Contrast-enhanced MRI of the brain (fig. 1a, b) showed high signal and mass effect predominantly in the right hippocampus on fluid-attenuated inversion recovery and T 2 - weighted images. Computed tomography of the thorax did not show tumor recurrence. Dear Sir, Morvan’s syndrome or ‘chorée fibril- laire de Morvan’ consists of peripheral nerve hyperexcitability (PNH), as well as autonomic and central nervous system (CNS) symptoms. With only a limited num- ber of reported cases, the complete spec- trum of the CNS symptomatology in Mor- van’s syndrome has not been well estab- lished. These symptoms have been noted to bear a striking similarity to limbic encepha- litis (LE) [1–3]; however, there has not been convincing evidence of LE with demonstra- ble involvement of limbic structures associ- ated with a PNH syndrome except for 2 cases [2, 4]. We present a patient with thymoma and myasthenia gravis who had an episode with fasciculations, autonomic symptoms and memory impairment while in pharmaco- logical remission 9 years after the removal of the thymoma. LE was well defined both by neuropsychological tests and magnetic resonance imaging (MRI) studies. Case Report A 45-year-old man was diagnosed with myasthenia gravis 9 years ago. A malignant lymphoepithelial thymoma was found for which he underwent thymomectomy fol- lowed by radiotherapy. He was in pharma- cological remission for the last 4 years. He presented with a 20-day history of tingling and burning sensations under the feet, fol- lowed by severe pain in the thighs and legs, profuse fasciculations in the lower extremi- Received: June 1, 2005 Accepted: October 25, 2005 Published online: January 6, 2006 Feza Deymeer Department of Neurology, Istanbul University Istanbul Faculty of Medicine, Çapa TR–34390 Istanbul (Turkey) Tel. +90 212 41420 000, Fax +90 212 533 8575, E-Mail deymeer@tnn.net © 2005 S. Karger AG, Basel 0014–3022/05/0544–0235$22.00/0 Accessible online at: www.karger.com/ene Prednisolone was increased to 70 mg/ day, 1 week after the onset of the CNS symptoms. At the same time, the patient was given 1 g methylprednisolone intrave- nously twice a week for a total dose of 5 g. After the onset of treatment, all symptoms gradually resolved within about 6 weeks. As the patient improved, there was a short pe- riod of a few days when he complained of brief déjà vu episodes. The electroencepha- logram was normal. Cerebrospinal fluid (CSF) was acellular. CSF protein and IgG index were normal, oligoclonal bands were negative. Pretreatment samples were not available for testing, but voltage-gated po- tassium channel (VGKC) antibodies were not detected in serum and CSF at this time. The serum was tested on a commercial blot that detects antibodies against Hu, Ma, Yo, Ri, collapsing response mediator protein 5 and amphiphysin; all were negative. Sym- pathetic skin responses returned to normal although the pathological findings in RR in- terval variability continued. Twenty-four- hour Holter monitoring showed the mean heart rate to be decreased to 90/min. A sec- ond neuropsychological evaluation showed complete disappearance of the visual mem- ory registration deficit. A repeat MRI showed marked resolution of the signal ab- normality and mass effect (fig. 1c, d). There was further improvement in the third neuropsychological evaluation. The patient made a complete recovery within 5 months from the onset of the disease at