Reference values for nerve conduction studies in healthy newborns, infants and children in Philippine Children's Medical Center

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Nerve conduction studies play a diagnostic role in the clinical evaluation of neuromuscular disorders in children. Reference ranges define the expected parameter values in disease-free children.</p><p style="text-align: justify;"><strong>OBJECTIVE:</strong> To propose reference values for sensory and motor nerve conduction and late responses in upper and lower limb peripheral nerves in Filipino children 5 years and below.</p><p style="text-align: justify;"><strong>METHODS</strong>: Sensory nerve conduction studies on median, ulnar, superficial peroneal, and sural nerves and motor nerve conduction and late response studies on median, ulnar, peroneal and posterior tribal nerves were done using standardized techniques among 100 healthy Filipino children.</p><p style="text-align: justify;"><strong>RESULTS:</strong> Subjects were stratified according to age groups. Reference values for the following parameters: (1) sensory conduction velocity and amplitude; (2) motor conduction velocity, amplitude and latency at distal sites;  (3) F-wave latency; and (4) H-reflex latency were summarized. These were expressed as mean ±standard deviation or median (range) for values that follow Gaussian and non-Gaussian distributions. The 5th and 95th percentile values were likewise reported. Age had direct correlation with various nerve conduction parameters. Height was directly correlated with F-wave parameters of median, ulnar and peroneal nerves but not posterior tribal nerve.</p><p style="text-align: justify;"><strong>CONCLUSIONS</strong>: Reference standards for nerve conduction studies of commonly tested nerves of Filipino children are presented. Values are comparable to reference ranges elsewhere except for the H-reflex latency which is higher in this study.</p>

Levodopa+carbidopa in x-linked dystonia parkinsonism (XDP/DYT3/Lubag): A randomized, double-blind, placebo-controlled trial

@#<p><strong>OBJECTIVE:</strong> X-linked dystonia parkinsonism (XDP) is an adult-onset, progressive and debilitating movement disorder described among Filipino males from Panay Island. The available oral medications have been ineffective. While chemodenervation with botulinum toxin A works and deep brain stimulation surgery is promising, these are not affordable for the vast majority of patients. Thus, we decided to look into the efficacy, safety and tolerability of levodopa+carbidopa (levodopa) versus placebo among patients with XDP.</p><p><strong>METHODS:</strong> This was a double blind, randomized, placebo-controlled clinical trial. Patients were randomized to receive levodopa or placebo for 6 months. The dose was increased gradually until 1000 mg levodopa/day is reached or until side effects appear.</p><p><strong>RESULTS:</strong> A total of 86 out of 94 randomized patients (91.5%) were included in the intention-to-treat cohort for the primary efficacy analysis. Nineteen patients (9 in levodopa, 10 in placebo) dropped out or were lost to follow up. There was no significant difference in the baseline and last visit Burke Fahn Marsden Dystonia Rating Scale and the part III of the Unified Parkinson's Disease Rating Scale scores between levodopa and placebo. The most common adverse events in the levodopa group were increased movements, pain and nausea/ vomiting.</p><p><strong>CONCLUSION:</strong> While levodopa is safe and well-tolerated, it does not have any effect in alleviating the dystonia or parkinsonism in XDP.</p>

Suicidality among patients with X-linked dystonia-parkinsonism

BACKGROUND: Sex-linked dystonia-parkinsonism (XDP) is an adult-onset, progressive, debilitating movement disorder that manifests with features of dystonia and parkinsonism. It is endemic among Filipino males from Panay Island. Many of these patients would have peculiar postures and bizarre movements forcing them to retreat to the confines of their home or probably in solitude. OBJECTIVE: To describe the rate of suicide among XDP patients. METHODS: We identified the patients from the Philippine XDP registry maintained by the XDP Study Group. A retrospective chart review was then done. RESULTS: There were 21 deaths attributable to suicide from the 194 deaths from the XDP registry (10.8%). The mean age at suicide was 44 + 11.38 years, around 7.76 + 4.65 years from onset of illness and 5.81 + 4.32 years from the diagnosis. Most patients (17/21) were in the generalized dystonia stage at the time of suicide. Five of the suicide cases belonged to two families. The most common method of suicide were hanging (10, 47.6%), refusal to eat (5, 23.8%), non-accidental organophosphate poisoning and self-mutilation (1 each, 4.8%). CONCLUSION: There was a high rate of suicide (10.8%) among XDP patients compared to the national suicide rate of 3.59/100,000. Hanging was the most common method used to commit suicide.