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Early onset epileptic auditory and visual agnosia with spontaneous recovery associated with Tourette's syndrome
  1. B G R NEVILLE
  1. Neurosciences Unit, Institute of Child Health (UCL), The Wolfson Centre, Mecklenburgh Square, London WC1N 2AP, UK
  2. General Hospital, Gloucester Street, St Helier, Jersey HE2 3QS, UK
  3. Principal Educational Psychologist, States of Jersey Education, PO Box 142, Jersey JE4 8QJ, UK
  1. Professor B G R Neville
  1. H C SPRATT
  1. Neurosciences Unit, Institute of Child Health (UCL), The Wolfson Centre, Mecklenburgh Square, London WC1N 2AP, UK
  2. General Hospital, Gloucester Street, St Helier, Jersey HE2 3QS, UK
  3. Principal Educational Psychologist, States of Jersey Education, PO Box 142, Jersey JE4 8QJ, UK
  1. Professor B G R Neville
  1. J BIRTWISTLE
  1. Neurosciences Unit, Institute of Child Health (UCL), The Wolfson Centre, Mecklenburgh Square, London WC1N 2AP, UK
  2. General Hospital, Gloucester Street, St Helier, Jersey HE2 3QS, UK
  3. Principal Educational Psychologist, States of Jersey Education, PO Box 142, Jersey JE4 8QJ, UK
  1. Professor B G R Neville

https://doi.org/10.1136/jnnp.71.4.560

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    Potentially recoverable impairments of cognition, behaviour, and movement are integral to early onset epilepsies.1 The classic epilepsy syndrome presenting as developmental regression is Landau-Kleffner syndrome, in which receptive aphasia and behavioural, cognitive, and motor impairments occur with centrotemporal discharges enhanced in sleep.2 We report a novel biography of domain specific impairments and recovery in infantile spasms.

    At 12 years of age the patient presented with Tourette's syndrome, with an extraordinary developmental history of epilepsy, regression, and recovery. He was normal until 6 months, being socially responsive, visually alert, reaching and transferring objects. Development slowed from 7 months. There was no relevant family history.

    At 8 months runs of typical symmetric flexion spasms at intervals of 5–10 seconds, 3–4 times/day began. The EEG was disorganised, with bilateral very high amplitude (450 μV) activity and more left temporal area multifocal spikes and polyspikes, approaching classic hypsarrhythmia. ACTH (10 units daily and 40 units daily from 10–12 months) stopped the spasms after 2 weeks. Electroencephalography, CT, metabolic investigations, electroretinography, and visually evoked potentials were normal. CMV antibodies were present in blood, and virus in the urine.

    Physical examination was normal. At 1 year an EEG showed excess of …

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