Startle seizures are induced by sudden and unexpected stimuli. The startle (unexpected and sudden presentation of the stimulus) is the provoking factor, although, rarely, patients may be specifically sensitive to one sensory modality. Sudden noise is the main triggering stimulus, but somatosensory and less often visual stimuli are also effective in some patients. Habituation to repetitive stimulation occurs.

Prevalence
Very low.

Age at onset
1 to 16 years.

Sex
Males = females.

Clinical manifestations
Most patients have static neurological/intellectual handicaps; hemiplegia predominates.

Startle seizures are usually generalized tonic.

The startle response is brief (<30 sec); axial tonic posturing frequently causes falls, which are often traumatic. It is asymmetrical in 1/4 of patients. In hemiparetic patients, the seizure starts with flexion and abduction of the paretic arm and extension of the ipsilateral leg, which rapidly involve the contralateral side. Marked autonomic manifestations, automatisms, laughter, and jerks may occur concurrently. Atonic or myoclonic startle seizures are less common.

Seizures are frequent, occurring many times a day, and sometimes progress to status epilepticus.

Infrequent spontaneous seizures are common (probably in all patients).

Etiology
Various localized or diffuse static brain pathology, mainly due to pre-, perinatal or early insults. Common in Down syndrome.

Diagnostic procedures
Brain MRI is necessary, even in neurologically normal patients. Abnormalities predominate in the lateral sensory-motor cortex.

Inter-ictal EEG
Diffuse or focal abnormalities reflect the underlying lesions.

Ictal EEG
A vertex discharge followed by diffuse flattening or low-voltage rhythmic (10 Hz) activity, which begins in lesioned motor or pre-motor cortex and spreads to mainly frontal regions. This is often obscured by muscle artefacts.

Differential diagnosis
(1) hyperekplexia (also called 'startle disease'), a non-epileptic disorder, and (2) reflex seizures (by touch, tap, or sudden dousing with hot water) may have a startle component that is not essential for their provocation; reflex seizures are mainly myoclonic, ictal EEG shows generalized discharges, and patients are otherwise normal.

Prognosis
Often poor.

Management options*
Treatment is often unsatisfactory with clonazepam, clobazam#, carbamazepine, or lamotrigine.

*Expert opinion, please check FDA-approved indications and prescribing information
#Not approved by the FDA

This page was adapted from:

The educational kit on epilepsies: The epileptic syndromes By C. P. Panayiotopoulos Originally published by MEDICINAE
21 Cave Street, Oxford OX4 1BA

First published 2006 and reprinted in 2007

Authored by: C. P. Panayiotopoulos MD PhD FRCP on 1/2005
Reviewed by: Steven C. Schachter MD on 6/2008
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