ABSTRACT
Behçet's disease (BD) is a multisystemic, recurrent, inflammatory disorder of unknown aetiology. Neurological involvement is characterised either by primary parenchymal lesions or secondary to major vascular involvement. Seizures are rarely seen in BD and their occurrence can be related to seizure provoking factors or exacerbation of the disease. We experienced a case of neuro-BD presenting with subacutely developing mental and behavioral changes, followed by left dominant tetraparesis with bilateral pyramidal signs, fever and left hand focal motor seizures with elementery clonic motor signs which later evolved into right hand epilepsia partialis continua (EPC) of Kojevnikov. The seizures were very resistant to antiepileptic drugs and 8 months after neurological involvement the patient died. The EPC evolving after neurological involvement is associated with high mortality rate.
Subject(s)
Behcet Syndrome/complications , Epilepsies, Partial/complications , Adult , Anti-Inflammatory Agents/therapeutic use , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Brain/pathology , Brain/physiopathology , Electroencephalography , Epilepsies, Partial/diagnosis , Epilepsies, Partial/physiopathology , Female , Humans , Magnetic Resonance Imaging , Methylprednisolone/therapeutic use , Periodicity , RecurrenceABSTRACT
We aimed to assess the relapse rate of epilepsy, prospectively attributable to antiepileptic drug (AED) withdrawal in seizure-free patients and to determine the risk factors for seizure recurrence. Seventy-nine patients with epilepsy who were seizure-free for at least 4 years were enrolled into the study. The AEDs were tapered by one-sixth every 2 months. The EEG and clinical examination were performed at the beginning; at each visit during discontinuation and 2, 6, 12, 24, and 36 months after the complete drug withdrawal. For each patient, records were obtained of the main demographic and clinical variables. A total of 49 patients completed the discontinuation programme. Twenty-eight patients (57%) relapsed while 21 of those (42.8%) did not suffer a relapse at the end of the study period. In patients discontinuing treatment, the probability of relapse was 21.4% during the tapering period (especially in the last months), 28.6% at 1 month, 14.3% at 3 months, 3.6% at 6 months, 7.1% at 12 months, 17.8% at 24 months, and 7.1% at 36 months. The age at onset of epilepsy and the duration of active disease were found to affect the risk of relapse. Although drug withdrawal could be considered in adult patients free of seizures for 4 years, the final decision should be tailored to the patient's clinical, emotional, and socio-cultural profile.
Subject(s)
Anticonvulsants/administration & dosage , Epilepsy/drug therapy , Remission Induction , Adult , Drug Administration Schedule , Electroencephalography , Female , Humans , Male , Prospective Studies , Recurrence , Risk Factors , Withholding TreatmentABSTRACT
Progressive facial hemiatrophy (PFH), Parry-Romberg syndrome, is a rare disorder frequently associated with epilepsy. We describe a 28-year-old man who had PFH and partial epilepsy that was easily controlled with antiepileptic drugs. In accordance with this patient's benign course of seizures, the cortical silent period was prolonged in the symptomatic hemisphere. This finding may represent compensatory interictal mechanisms in epilepsy.
