DILATED EPISCLERAL ARTERIES/Cow/»ee et al. 45carotid artery occlusion. Dilated episcleral arteries, particularly in the absence of other stigmata of ocular ischemia, strongly suggest that the external carotid artery is the major source of blood supply to the homolateral cerebral hemisphere. Consequently, maintaining patency of the external carotid artery in these situations is important. OVER 50 YEARS AGO, Sydensticker 1 established that sickle cell anemia (SCA) could have prominent neurologic manifestations. A recent review of 89 cases of SCA, proven by electrophoresis to be homozygous for hemoglobin (SS), showed that 29% of the patients had developed neurological complications, and that 17% had had strokes. References2 The patient with SCA we studied arrived in coma as the result of bilateral carotid artery occlusion. Angiography revealed a pattern which is consistent with Moyamoya disease. An autopsy showed thrombosis of major intracranial vessels while intimal hyperplasia, unusual in SCA, was also present. Clinical FeaturesA 7-year-old black boy was admitted to Jackson Memorial Hospital with an acute alteration of consciousness. The patient was originally diagnosed as having sickle cell anemia at the age of 7 months. Originally he had edema of both hands and feet, was anemic, and had a positive sickle cell preparation. Subsequently, he had several hemolytic crises. Seven months before this last admission, the child developed a right hemiparesis with hemianesthesia and Broca's aphasia. This was accompanied by focal myoclonic seizures involving his right arm and the right side of his face. He was given phenytoin and improved, but 4 months later he suffered a similar episode, which left him with moderately severe expressive aphasia. A Tc99 (Technetium) brain scan was diagnostic for occlusion of the left middle cerebral artery. His hemoglobin ranged between 6-8 gm/dl, and his reticulocyte count between 10-40%. Prior to his last admission he had been irritable and was later found comatose. In the emergency room, he was stuporous, and had right spastic hemiparesis, a right gaze preference and right-beating jerk nystagmus. His hemoglobin was 6.9 gm/dl, with a white cell count of 17,400 per mm 3 and 16% reticulocytes. The cerebrospinal fluid contained 11 white blood cells per mm, 8 (45% granulocytes). He was treated with phenobarbital and diazepam and became more alert. Subsequently, an EEG revealed bilateral slowing, more marked on the left. Three days later, he became febrile (102.4°F) and more lethargic. Examination revealed nuchal rigidity and a positive Brudzinski sign. A second spinal tap showed 6850 red cells and 340 white cells per mm 3 with 55% granulocytes and a protein of 322 mg/dl; CSF glucose levels were normal. Cultures of spinal fluid, blood and urine were all negative. A brain scan (Tc99) showed another area of increased uptake in the posterior right hemisphere. The child was treated with penicillin and a transfusion of 1000 ml packed cells after which he improved slightly. Hemoglobin electrophoresis...
BACKGROUND Immediate Release (IR) amantadine has been used for treatment of levodopa induced dyskinesia (LID). The immediate-release/extended-release (IR/ER) amantadine formulation OS320 (OSMOLEX ER ® ) contains an IR outer layer and ER core for once-daily dosing. OBJECTIVEReport individual and pooled results for the similarly designed double-blind, placebocontrolled ALLAY-LID I and II trials, assessing IR/ER-amantadine for LID. METHODS PD patients with LID were randomized to IR/ER-amantadine 193mg, 258mg, or placebo. Primary endpoint was Unified Dyskinesia Rating Scale (UDysRS) score change from baseline to Day 98. Secondary outcome was ON time without troublesome dyskinesia based on diaries. Exploratory outcomes were other diary states (including OFF), MDS-UPDRS Parts II+III and Fatigue Severity Scale. RESULTSOverall, 222 individuals enrolled (N=87 ALLAY-LID I, N=135 ALLAY-LID II); both trials terminated early for sponsor's decision. While ALLAY-LID I did not meet its primary endpoint, a significant reduction in UDysRS scores versus placebo was observed in ALLAY-LID II for both 193mg and 258mg doses. In the pooled analysis, placebo-adjusted UDysRS score differences were −5.5[−9.8,−1.2], p=0.012 and −5.2[-9.5,-0.9], p=0.017, respectively. IR/ER-amantadine 258mg significantly increased time spent ON without troublesome dyskinesia in ALLAY-LID II and pooled analysis. Reductions in ON time with dyskinesia supported the primary outcome. There was no effect on OFF time or other outcomes. Overall, 13.3% (193mg), 18.7% (258mg) and 11.1% (placebo) discontinued for adverse events, most commonly hallucinations (4.0%, 10.7%, and 1.4%, respectively).CONCLUSIONS IR/ER-amantadine significantly reduced LID in ALLAY-LID II but not in ALLAY-LID I; post-hoc pooled data also indicated a positive treatment effect on LID.
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