Fasciculations, Autonomic Symptoms and Limbic Encephalitis: A Thymoma-Associated Morvan’s-Like Syndrome
| Autor: | Piraye Serdaroglu, Oguzhan Coban, Sukriye Akca, Oget Oktem-Tanor, Angela Vincent, Feza Deymeer, Yesim Parman, Gulsen Kocaman |
|---|---|
| Rok vydání: | 2005 |
| Předmět: |
Pathology
medicine.medical_specialty Wechsler Memory Scale Thymoma medicine.diagnostic_test business.industry Limbic encephalitis Neurological examination medicine.disease Fasciculation Neurology Visual memory Internal medicine Memory span medicine Cardiology Neurology (clinical) Verbal memory medicine.symptom business |
| Zdroj: | European Neurology. 54:235-237 |
| ISSN: | 1421-9913 0014-3022 |
| DOI: | 10.1159/000090719 |
| Popis: | ties, tremor in the hands, excessive sweating in the head and hands, impotence, urinary urgency, and insomnia. Physical examination revealed tachycardia and hyperhidrosis. Neurological examination was normal except for fasciculations in the lower extremities and a moderately diminished vibration sense in the toes. There was no postural hypotension. Needle electromyog raphy showed fasciculations in the gastrocnemius muscle. Nerve conduction studies were normal. Autonomic tests revealed decreased RR interval variability and absent sympathetic skin responses. A detailed cardiological 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 defi cit. Neuropsychological evaluation (Wechsler Memory Scale Digit Span, Logical Memory and Visual Reproduction subtests, backward counting, category fl uency test, Wechsler Adult Intelligence Scale-Revised similarities subtest, Stroop test, verbal memory processes test, Luria’s alternating sequences) revealed a defect in primary visual memory registration with an attention defi cit, but intact primary verbal memory. Contrast-enhanced MRI of the brain ( fi g. 1 a, b) showed high signal and mass effect predominantly in the right hippocampus on fl uid-attenuated inversion recovery and T 2 weighted images. Computed tomography of the thorax did not show tumor recurrence. Dear Sir, Morvan’s syndrome or ‘choree fi brillaire de Morvan’ consists of peripheral nerve hyperexcitability (PNH), as well as autonomic and central nervous system (CNS) symptoms. With only a limited number of reported cases, the complete spectrum of the CNS symptomatology in Morvan’s syndrome has not been well established. These symptoms have been noted to bear a striking similarity to limbic encephalitis (LE) [1–3] ; however, there has not been convincing evidence of LE with demonstrable involvement of limbic structures associated 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 pharmacological remission 9 years after the removal of the thymoma. LE was well defi ned both by neuropsychological tests and magnetic resonance imaging (MRI) studies. |
| Databáze: | OpenAIRE |
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