| CASE REPORT |
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| Year : 2017 | Volume
: 12
| Issue : 1 | Page : 36-39 |
Sporadic hyperekplexia plus syndrome
Sadanandavalli Retnaswami Chandra1, Chetan Vekhande1, Lakshminarayanapuram Gopal Viswanathan1, Pooja Mailankody2, Karru Venkata Ravi Teja3
1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
3 Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
Correspondence Address:
Sadanandavalli Retnaswami Chandra
Department of Neurology, Faculty Block, Neurocentre, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jpn.JPN_170_16
A disorder of infants and children with pathological startle response, features of other system involvement, falls, and stiffness with retained consciousness. It should be differentiated from conditions such as myoclonic epilepsy, psychogenic movement disorder, Isaac syndrome, Schwartz–Jampel syndrome, Gilles de la Tourette, and culture-specific startle syndromes such as jumping Frenchman of Maine. A 5-year-old child symptomatic with repeated falls spontaneously as well as by sound and activities since neonatal period. He was having hyperalert facies, intelligent, cooperative with mild dysmorphism. His investigations were noncontributory except giant somatosensory evoked potentials and skeletal abnormalities. He showed excellent response to clonazepam and no complications on withdrawing the antiepileptic drugs. Proper diagnosis is of great therapeutic relevance and is based on high degree of suspicion.
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