Resolution of dopamine dysregulation syndrome following cessation of dopamine agonist therapy in Parkinson's disease.

Repetitive and compulsive behaviours can occur in association with the use of dopaminergic medications for the treatment of Parkinson's disease (PD). This syndrome has been referred to as the 'dopamine dysregulation syndrome'. The prognosis for patients with this syndrome is unclear. We report five PD patients in whom the abnormal behaviours resolved completely after withdrawal of dopamine agonist therapy. We alert clinicians to the apparent role of dopamine agonists in this syndrome. In addition, we highlight the potential reversibility of the syndrome, its varied phenomenology and its potential pathophysiology.

Idiopathic generalized epilepsy presenting with hemiconvulsive seizures.

PURPOSE: Unilateral seizures, or hemiconvulsive attacks, are motor seizures with tonic and/or clonic phenomena that involve only one side of the body. METHODS: We describe three adolescents who presented with hemiconvulsive seizures and were found to have 3-cps generalized spike-and-wave discharges on ictal and/or interictal EEG. All had normal neuroimaging studies. Two patients had been previously treated with carbamazepine, which led to a partial response in one patient. RESULTS: All three patients, however, are now seizure free on either sodium valproate or a combination of sodium valproate and lamotrigine. We believe the electroclinical diagnosis is that of idiopathic generalized epilepsy. CONCLUSIONS: Idiopathic generalized epilepsy presenting with hemiconvulsive seizures has not, to our knowledge, been previously described. However, the correct diagnosis of an idiopathic generalized seizure disorder, as opposed to a partial seizure disorder, has important treatment implications. The possible mechanism of hemiconvulsive seizures in idiopathic generalized epilepsy is discussed.

Central pontine myelinolysis.

Central pontine myelinolysis (CPM) was initially associated with alcoholism. Subsequently other factors, including rapid reversal of hyponatraemia and extreme serum hypoosmolality associated with severe burns, have been identified as other important factors in its pathogenesis. Extra-pontine lesions have also been described. CPM may be found at autopsy, either having been overlooked during life or as an incidental finding. Its precise incidence is not known but the ability to diagnose it during life has been helped by modern neuroimaging, particularly magnetic resonance imaging (MRI) of the brain stem. In the past the prognosis for CPM was thought to be invariably fatal. It is clear now that with the greater general awareness of the disorder and the ability to diagnose it during life that some degree of recovery is possible. However, the number who do recover and the degree of recovery is not known. We report a 40-year-old man who developed CPM presenting with quadriparesis and inability to speak and swallow. There were risk factors for CPM and the diagnosis was confirmed by MRI scanning. He made a complete recovery although he remains ataxic. We are reporting this case as we believe it is important to make clinicians aware of the potential for recovery of CPM. While no specific treatment has been shown to influence the degree and rate of recovery of the demyelination, the fact that the quadriplegia and bulbar paralysis can recover fully is of considerable importance. In particular, it means that when the diagnosis is made, complete and vigorous nursing and medical care is warranted.