Clobazam-Clonazepam Combination Effective for Stimulus-Induced Falling in Hyperekplexia

McAbee, Gary N.

doi:10.1177/0883073813516677

Cited by 7 publications

(6 citation statements)

References 15 publications

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“…Clobazam as monotherapy has been published in the literature recently with good success in two severe infantile cases 3 . Clobazam was added to clonazepam with good results in our patient, and this combination was also found to be successful in a 7-year-old boy who had significant falls because of exaggerated startle 4 .…”

Section: Discussionmentioning

confidence: 55%

Case 1: A Term Infant with Apnea and Stiffening

Arroyo¹,

Hufnagel

2017

NeoReviews

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Section: Discussionmentioning

confidence: 55%

Case 1: A Term Infant with Apnea and Stiffening

Arroyo¹,

Hufnagel

2017

NeoReviews

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“…If diminished integration of the startle reflex can also involve first postural responses then this could conceivably be a factor in the risk of falls in patients with Parkinson’s disease. If so, then it is worth noting that therapies exist to suppress startle and therefore falls in patients with hyperekplexia (exaggerated startle) (McAbee 2015).…”

Section: Discussionmentioning

confidence: 99%

Neurophysiological analysis of the clinical pull test

Tan

Perera

McGinley

et al. 2018

Journal of Neurophysiology

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Postural reflexes are impaired in conditions such as Parkinson's disease, leading to difficulty walking and falls. In clinical practice, postural responses are assessed using the 'pull test', where an examiner tugs the pre-warned, standing patient backwards at the shoulders and grades the response. However, validity of the pull test is debated with issues including scaling and variability in administration and interpretation. It is unclear whether to assess the first trial or only subsequent repeated trials. The ecological relevance of a forewarned backwards challenge is also debated. We therefore developed an instrumented version of the pull test to characterize responses and clarify how the test should be performed and interpreted. In thirty-three healthy participants, 'pulls' were manually administered and pull force measured. Trunk and step responses were assessed with motion tracking. We probed for the StartReact phenomenon (where pre-prepared responses are released early by a startling stimulus) by delivering concurrent normal or 'startling' auditory stimuli. We found that the first pull triggers a different response, including a larger step size suggesting more destabilization. This is consistent with 'first trial effects', reported by platform translation studies, where movement execution appears confounded by startle reflex-like activity. Thus, first pull test trials have clinical relevance and should not be discarded as practice. Supportive of ecological relevance, responses to repeated pulls exhibited StartReact, as previously reported with a variety of other postural challenges including those delivered with unexpected timing and direction. Examiner pull force significantly affected the postural response particularly the size of stepping.

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“…Stiffness and startle may be difficult to completely treat, and are best approached with clonazepam or clobazam (Stewart et al ., ). In addition to benzodiazepines, epileptic myoclonus is also treated with valproate and levetiracetam (McAbee, ). In our patient, levetiracetam failed, but high‐dose clonazepam was effective in completely suppressing the child's spells.…”

Section: Discussionmentioning

confidence: 99%

A child with hyperekplexia and epileptic myoclonus

Wadi

Medlej

Obeid

2018

Epileptic Disorders

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Hyperekplexia is a rare neurogenetic disorder characterized by startle. Accurate diagnosis of this notorious mimicker of epilepsy is important to prevent life-threatening apnoea. We report a novel case of concomitant GLRA1-related hyperkeplexia and myoclonic epilepsy. A toddler with daily paroxysms of head drops and falls presented with epileptic myoclonus on EEG, however, whole-exome sequencing revealed hyperekplexia-related GLRA1 mutation. The boy eventually developed spells induced by noise and surprise. All his spells remitted upon treatment with clonazepam. Paediatricians and paediatric neurologists should be aware of this possible mixed presentation in order to appropriately tailor medication regimens and treatment goals. [Published with video sequence on www.epilepticdisorders.com].

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Clobazam-Clonazepam Combination Effective for Stimulus-Induced Falling in Hyperekplexia

Cited by 7 publications

References 15 publications

Case 1: A Term Infant with Apnea and Stiffening

Case 1: A Term Infant with Apnea and Stiffening

Neurophysiological analysis of the clinical pull test

A child with hyperekplexia and epileptic myoclonus

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