Efficacy and Safety of Entacapone in the Patients with Parkinson's Disease Experiencing Wearing-off Phenomenon: Multicenter Randomized Placebo-controlled Double Blind Study

BACKGROUND: The purpose of this study was to assess the efficacy and safety of entacapone, a catechol-O-methyltransferase (COMT) inhibitor in Parkinson's disease (PD) patients with wearing-off phenomenon. METHODS: A total of 197 PD patients were included in this 2-month multi-center, randomized, placebo-controlled, double blind, parallel-group study. After a 2-week screening period, each patient was randomly allocated to receive either entacapone (n=98) or placebo (n=99) as an adjunct to levodopa. The efficacy was evaluated with the changes of "on" and "off" time percentage while awake, the reduction of the levodopa dose, Unified Parkinson's Disease Rating Scale (UPDRS), and the clinical global impression (CGI) by the examiner. RESULTS: The percentage of "on" time increased by 9.4 +/- 18.0% in the entacapone group, 7.4 +/- 15.6% in the placebo group. The percentage of "off" time was reduced by 8.6 +/- 16.9% in the entacapone group, 6.6 +/- 18.2% in the placebo group. These parameters did not show a statistical significance between the two groups. However, the levodopa dose was significantly reduced in the entacapone group (51.6 +/- 154.5 mg/day) compared with the placebo group (0.7 +/- 130.0 mg/day) (p=0.009). The total and motor scores of the UPDRS were significantly decreased in the entacapone group (p=0.039, p=0.017, respectively). The most common adverse drug reactions in the entacapone group were urine discoloration (22%), dyskinesia (13%), dizziness (7%). CONCLUSIONS: Entacapone was a safe and well-tolerated drug. Although the changes of "on" and "off" time were not significant, entacapone showed an overall significant beneficial effect in the PD patients with wearing-off phenomenon.

A case of Neuro-Behcet's disease with CNS lesions confined to multiple cortical areas

We report a patient diagnosed with neuro-Behcet's disease showing multiple enhancing lesions on a brain MRI that were confined to the cerebral cortex without any involvement of other brain regions. The patient, a 30-year-old man, was admitted because of seizure attacks. He had the symptom triad of recurrent oral ulcer, genital ulcer, and uveitis. Neurologic symptoms included ataxia, dysarthria, and dementia which had progressed over several years. During the hospital course, he underwent a right hemicolectomy due to bowel perforation. A pathological examination showed multiple discrete ulcerations from the cecum to the mid-transverse colon, consistent with Behcet's colitis. A brain MRI revealed multiple small enhancing lesions distributed over wide areas of the cerebral cortex. To our knowledge, this pattern of lesion distribution has not yet been reported in neuro-Behcet's disease.

A Novel Mutation in Exon 6 in a Patient with Fabry's Disease

Fabry's disease, angiokeratoma corporis diffusum, is a rare X-linked inborn error of glycosphingolipid metabolism due to the lack of the lysosomal enzyme, alpha-galactosidase A, resulting in a progressive intracellular deposition of neutral glycosphingolipids in various tissues, including the dorsal root ganglia, autonomic nervous system, vascular endothelial, and smooth muscle cells. Clinical manifestations of Fabry's disease result predominantly from the progressive deposition of globotriaocylceramide in the nervous system or vascular endothelium, and are characterized by acro-paresthesia, angiokeratoma, corneal opacity, TIA or stroke, ischemic heart disease, and renal failure. We report a case of a 19-year-old man presenting with a 12-year history of severe distal pain, acroparesthesia, short stature, and delayed puberty. An enzymatic assay disclosed substantially diminished alpha-galactosidase A activity and an electron microscopy of the peripheral nerve showed lipid inclusions which were composed of concentrically laminated, ovoid osmiophilic bodies in the perineural fibroblast and endothelial cells. These findings are typical of Fabry's disease and additional genetic study revealed deletion mutation(TTAG) at the 6th exon of the alpha-galactosidase A gene, which is a novel mutation that had never been reported in literatures. Symptomatic treatment with carbamazepine and clonazepam was tried with a good response.