Symptomatic hyperekplexia in a patient with multiple sclerosis
| Autor: | Walter Waespe, Klemens Ruprecht, Ralf Gold, Monika Warmuth-Metz |
|---|---|
| Rok vydání: | 2002 |
| Předmět: |
Adult
Reflex Startle Startle response medicine.medical_specialty Multiple Sclerosis Gabapentin Audiology Central nervous system disease Moro reflex medicine Humans Hyperekplexia Family history Glycine receptor Reflex Abnormal medicine.diagnostic_test business.industry Multiple sclerosis medicine.disease Magnetic Resonance Imaging Acoustic Stimulation Female Neurology (clinical) medicine.symptom business Neuroscience medicine.drug |
| Zdroj: | Neurology. 58:503-504 |
| ISSN: | 1526-632X 0028-3878 |
| Popis: | The startle reflex is a basic alerting reaction to sudden sensory stimuli in mammals. Hyperekplexia, or startle disease, is characterized by an exaggerated, abnormal startle response and occurs as a rare hereditary disorder, typically caused by a dominant mutation in the α 1-subunit of the glycine receptor gene.1 However, nonhereditary cases of hyperekplexia, particularly in association with brainstem lesions, have been described.2-4⇓⇓ We report on a patient with MS with severe symptomatic hyperekplexia that completely disappeared after treatment with gabapentin. A 28-year-old woman presented with a 1-week history of excessive startle responses to sudden acoustic stimuli such as a telephone ringing or a closing door. Unattended visual stimuli, e.g., awareness of a person standing previously unnoticed behind her, could also elicit the reaction. On one occasion, she was startled and fell to the ground when somebody unexpectedly spoke to her on the street. The patient had previously never experienced such symptoms and there was no family history of exaggerated startle. She first had paresthesias in … |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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