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Behçet's syndrome may present with partial seizures
  1. SIMON MEAD
  1. MRC Prion Unit, Imperial College
  2. St Mary's Hospital, Norfolk Place
  3. London W2 1PG, UK
  4. Royal Free Hospital, Hampstead, London, UK
  5. Royal Free Hospital, Queen Square, London WC1N 3BG, UK
  1. Dr Gordon Plant, Royal Free Hospital, Queen Square, London WC1N 3BG, UK
  1. DESMOND KIDD
  1. MRC Prion Unit, Imperial College
  2. St Mary's Hospital, Norfolk Place
  3. London W2 1PG, UK
  4. Royal Free Hospital, Hampstead, London, UK
  5. Royal Free Hospital, Queen Square, London WC1N 3BG, UK
  1. Dr Gordon Plant, Royal Free Hospital, Queen Square, London WC1N 3BG, UK
  1. CATRIONA GOOD,
  2. GORDON PLANT
  1. MRC Prion Unit, Imperial College
  2. St Mary's Hospital, Norfolk Place
  3. London W2 1PG, UK
  4. Royal Free Hospital, Hampstead, London, UK
  5. Royal Free Hospital, Queen Square, London WC1N 3BG, UK
  1. Dr Gordon Plant, Royal Free Hospital, Queen Square, London WC1N 3BG, UK

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A 25 year old right handed male shop assistant presented with seizures, visual problems, and malaise. The first symptoms were arthralgia and fatigue shortly followed by a bifrontal headache. A few days later he developed a visual disturbance that he described as peripheral blurred patches in both visual fields similar to the effect of staring into a bright light. About 2 weeks from the onset of symptoms he was driving when he had numerous episodes of deja vu and three episodes of a pungent sickly smell. He then lost consciousness and crashed his car into a public house without serious injury. An off duty nurse witnessed a generalised tonic-clonic seizure at the time. He was admitted to hospital and investigated but no diagnosis was made. The headache stopped completely in a month; the visual defects improved slightly but persisted. Six months later he had a relapse with recurrent headaches, pyrexia, and enlargement of the scotoma in the right eye and he was readmitted. He had had recurrent oral ulceration for 3 years and psoriasis since childhood, but no genital ulceration, red eyes, or venous thrombosis.

On examination he had a low grade pyrexia. General examination was otherwise normal with no evidence of pathergy at sites of needle pricks, genital ulceration, arthritis, or venous thrombosis. He was oriented with no meningism. Acuity was 6/5, N4.5 bilaterally. Colour vision was normal. In the left eye there was a partial superior scotoma, in the right eye a superionasal scotoma. Fundoscopy showed specific features of the uveitis of Behçet's syndrome. This consisted of multiple pale yellow patches of retinal infiltration lying deep to retinal vessels. Eye movements were normal. Pupils were equal and reactive with no afferent pupillary defect. The rest of the cranial nerve examination, gait, and limb examination was normal.

Biochemistry, liver function, thyroid function, coagulation studies, serum electrophoresis, serum ACE, B12, folate, and plasma amino acids were all normal. Haematology showed a slight lymphopenia of 1.0 (1.5–4.0) and slightly increased erythrocyte sedimentation rate at 19. Autoantibody profile, RF titre, and syphilis serology were negative; CSF pressure was normal, but analysis was abnormal with 20 white cells (93% lymphocytes, not reactive) and a slightly increased protein of 0.88 g/l, glucose was 3.0 mmol/l (serum 4.2 mmol/l). The CSF had no oligoclonal bands; CSF ACE and cytology were normal. Chest radiography, ECG, transthoracic ECHO, and extracranial magnetic resonance angiography (MRA) were normal. An EEG showed a mild asymmetry of α-rhythm being lower amplitude and less well formed on the left but no epileptiform features. Brain MRI was performed on two occasions. The MRI at presentation showed two small focal T2 hyperintense lesions in the head of the right caudate nucleus and more diffuse signal change in the right mesial temporal lobe within the head and body of the right hippocampus (figure). There was no evidence of venous sinus thrombosis.

T2 weighed MRI showing a mesial temporal lobe lesion with resolution a year later.

A diagnosis of Behçet's syndrome with neurological complications was made on the basis of typical retinal lesions, multiple focal CNS lesions, recurrent mouth ulceration and a constitutional disturbance. Prednisolone was started at a dose of 40 mg daily, his symptoms rapidly improved and so combination immunosuppression was not used. A second MRI, a year later, showed that the lesions previously seen in the caudate had disappeared and that in the mesial temporal region had undergone a marked reduction in size (figure). There have been no more seizures and he has remained off antiepileptic medication.

Behçet's syndrome is a multisystem inflammatory disorder of unknown aetiology.1 It is a disorder of young adults with a male preponderance. There is a striking geographical variation in prevalence. The triad of oral and genital ulceration with hypopyon iritis is classic but neurological involvement is the most serious manifestation. There is no specific laboratory test and so diagnosis is made on clinical features. The International Study Group for Behçet's syndrome diagnostic criteria are recurrent oral ulceration plus two from recurrent genital ulceration, eye lesions, skin lesions, or positive pathergy test. Strict use of these criteria leads to underdiagnosis and it is accepted, as in this case, that experienced clinicians may make the diagnosis on the more unusual features of the syndrome. In the British series neuroBehçet's syndrome usually manifested as a subacute brainstem meningoencephalitis, occasionally with involvement of hemispheres or spinal cord. Brain MRI demonstrates lesions in about three quarters of patients with neuroBehcet's disease.1

To our knowledge this is the first report of Behçet's syndrome presenting with seizures. The phenomenology of the seizure cluster at presentation suggests that the focus was the lesion in the medial temporal lobe identified on the first MRI. As this lesion has regressed the prognosis for further seizures should be good and to date there has been no recurrence. There are occasional reports in the literature of seizures associated with Behçet's syndrome. A 35 year old man developed frank seizures coincident with a myocardial infarction and ventricular tachycardia after 2 years of Behçet's syndrome.2 A 38 year old woman also developed generalised seizures and recurrent status epilepticus 3 years before a diagnosis of Behçet's syndrome.3 4 A patient in a Turkish series was reported to have myoclonic jerks.4 In these reports the phenomenology of epilepsy was not presented and no clear relation could be made to the disease process. There have been a few a reports of EEG abnormalities in some severely affected cases consisting of periodic lateralising epileptiform discharges (here herpes simplex encephalitis was the main differential diagnosis),5 but mostly of non-specific EEG changes without prognostic value, as seen in the present case.

In summary, this case illustrates an unusual neurological complication of Behçet's syndrome. Diagnosis was made on the basis of a typical posterior uveitis, recurrent mouth ulceration, multiple focal CNS lesions on MRI, and constitutional upset. He presented with complex partial and secondary generalised seizures with a medial temporal lobe lesion on MRI that disappeared 6 months later.

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