Abstract
A case of late onset sporadic startle syndrome in a patient with a right posterior fossa brain tumour is reported. The exaggerated startle response did not respond to treatment with clonazepam. In addition to anxiety and depression, the patient developed obsessive- compulsive symptoms which responded to behavioural therapy. The possible mechanisms for this unique pattern of symptoms are discussed.
Highlights
The startle response is a normal primitive reaction which consists of a stereotyped pattern of movements including blinking, facial grimacing, flexion of the head, shoulders, elbows, trunk and knees
Major startle disease is characterised by hypertonia in infancy, falling attacks without loss of consciousness, episodic generalised shaking, hyperreflexia and gait disturbance [10]
The physical and mental problems which K developed over a 10 year period may be summarised as follows: epilepsy and/or non-epileptic seizures, exaggerated startle response, dysphonia and stuttering, tremor, anxiety and depression, compulsive behaviour and mild cognitive impairment
Summary
The startle response is a normal primitive reaction which consists of a stereotyped pattern of movements including blinking, facial grimacing, flexion of the head, shoulders, elbows, trunk and knees. It may be elicited by any unexpected stimuli and will usually decrease in magnitude on repeated stimulation [2]. Startle ‘disease’ or hyperekplexia is an autosomal dominant condition in which the exaggerated startle response is a primary feature [2]. Minor startle disease consists only of an inconstant excessive startle response. The patient we describe here, who came into our care at the age of 77 y, developed an exaggerated star-.
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