OBJECTIVE: To describe the rate of suicide and explore its possible related factors among patients with X-linked Dystonia Parkinsonism. Specifically, this paper aimed to describe the rate of suicide among patients with XDP based on the Philippine XDP registry and to describe these patients in terms of severity of XDP and psychosocial factors.
BACKGROUND: Chronic progressive neurologic conditions have been associated with serious psychosocial stresses. Suicide among patients with X-linked Dystonia Parkinsonism has been previously reported to be high.
METHODS: A retrospective chart review was done on XDP patients with deaths attributable to suicide. XDP related variables and available psychosocial factors were noted.
RESULTS: The prevalence of suicide among all XDP patients registered is 4.16%. There are 194 deaths in the Philippine XDP registry, 21 of which were attributable to suicide, a proportion of 10.8%. Their mean age at suicide was 44, around 7.76 mean years from the onset of illness. All of the patients were either in generalized dystonia or parkinsonian stage when they had suicide. Psychosocial variables noted were marital and family conflict, and loss of employment. None of the patients had a prior documented psychiatric illness but several of them showed symptoms of depression prior to suicide.
CONCLUSIONS: There is a high rate of suicide among patients with XDP which is comparable to other disabling neurodegenerative diseases. It occurs relatively late in the course where the patient is already in the stage of generalized dystonia or parkinsonism. Possible psychosocial risks include poor family support, marital conflict, loss of employment and symptoms suggestive of depression. The present understanding is that depression and suicide in XDP is more likely reactive to the disease rather than part of its clinical feature. This study supports this view.

Human ; Male ; Female ; Depression ; Dystonia ; Dystonic Disorders ; Family Conflict ; Genetic Diseases, X-linked ; Neurodegenerative Diseases ; Parkinsonian Disorders ; Suicide ;

BACKGROUND: XDP is an X-linked recessive disorder characterized by parkinsonism and dystonia described among Filipinos. Oral medications are frequently ineffective. Lately, DBS have been promising. However these are not generally available or affordable for the vast majority of patients. We then decided to evaluate the effectiveness of levodopa-carbidopa for XDP.
OBJECTIVE: To compare the efficacy, safety and tolerability of levodopa-carbidopa vs. placebo in XDP patients.
METHODS: After informed consent and randomization, the BFM and the UPDRS parts III and IV were performed at baseline and monthly up to 6 months. Patients were randomized to receive either levodopa-carbidopa at a starting dose of 125 mg levodopa/ day in 2 divided doses or corresponding placebo. Gradual uptitration was done to a maximum of 1000 mg levodopa/ day or until side effects appeared.
Homogeneity of the characteristics of patients in the 2 groups was determined using Independent t-test and Chi-square test. To determine the significance of changes in the efficacy parameters within each group, Wilcoxon Matched Pairs Signed Ranks Test was used. To compare the scores of the different efficacy parameters of the 2 groups, Mann Whitney U Test was applied to the data. A p?0.050 was considered significant.
RESULTS: A total of 107 patients were recruited. There were 13 screen failures, and 94 were subsequently enrolled. The baseline characteristics (age, duration of illness, baseline BFM and UPDRS (motor) scores were not significant between levodopa and placebo (age in years: 47 + 9.35 vs. 50 + 9.51; duration of illness in years 6.3 + 7 vs. 6.2 + 5.2; BFM score: 32.8 + 24.5 vs. 28.4 + 26.5; UPDRS score 29.9 + 20.7 vs. 34.8 + 26.8).
There was a decrease in BFM scores from baseline to all follow-up periods in patients given levodopa but were statistically significant only on visit 2 and visit 9. In the placebo group, decrease in scores was also observed in some observation periods but no statistical significance was shown. A comparison of the 2 groups showed that the magnitude of decrease in the levodopa group was statistically greater than the placebo group on the second visit. There were no significant differences observed in all other follow-up periods. Both groups showed a decrease in UPDRS scores but significant decrease was observed in visits 2, 5, 6, 7, 9 of the levodopa group. While in the placebo group, a significant decrease was observed only on visit 2. Comparison of the 2 groups did not show any significant differences.
There were 17 patients from the levodopa group who reported adverse events (most common: increased involuntary movements, nausea/ vomiting/ dizziness, headache, and generalized weakness. In the placebo group, there were 11 patients (most common: increased involuntary movements, abdominal pain). There were 9 patients who dropped out (levodopa: 4, placebo: 5).
CONCLUSION: There was a significant decrease in the BFM and UPDRS scores in XDP patients given levodopa compared to placebo. Levodopa is a safe and effective drug that may be considered in patients with XDP.
NOTE: This study was supported by an unrestricted grant by Torrent Pharma Philippines, Inc.

Human ; Abdominal Pain ; Carbidopa ; Dyskinesias ; Dystonia ; Dystonic Disorders ; Headache ; Levodopa ; Nausea ; Parkinsonian Disorders ; Statistics, Nonparametric ; Vomiting

INTRODUCTION: Coenzyme Q10, also known as Ubiquinone, is a substance now being used as a dietary supplement in many countries including the Philippines. It has also been the focus of several researches as treatment for several diseases including Parkinson's Disease. Several studies have shown that Coenzyme Q10 inhibits mitochondrial dysfunction in Parkinson's Disease, hence delaying its progression.
OBJECTIVES: The objective of this study is to assess and summarize the available evidence on the efficacy and safety of Coenzyme Q10 administration in the prevention of the progression of early Parkinson's Disease.
METHODS: This is meta-analysis of randomized controlled trials on the use of Coenzyme Q10 in Parkinson's Disease. A literature search in several databases was conducted for relevant studies. Three randomized controlled trials met the inclusion criteria. The efficacy of Coenzyme Q10 were measured using the total and the component scores of the Unified Parkinson Disease Rating Scale on follow-up. On the other hand, safety were measured using the withdrawal rate and the associated adverse reactions during the therapy of CoQ10. The Review Manager Software was utilized for the meta-analysis.
RESULTS: Compared to Placebo, treatment of CoQ10 did not show any significant difference in the mean scores of the UPDRS mental and ADL scores. Interestingly, the UPDRS motor score showed a significant difference between Coenzyme Q10 and placebo, but no significant difference when a subgroup analysis between high-dose (-4.03 [-15.07-7.01], p-value 0.47, I2 67%, P for heterogeneity 0.08) and low-dose Coenzyme Q10 (0.53 [-0.89-1.94], p-value 0.47, I2 34%, P for heterogeneity 0.22) was done. Overall, there was no significant difference in the total UPDRS score (0.68 [-0.61-1.97], p-value 0.30, I2 0%, P for heterogeneity 0.70). The anxiety, back pain, headache, sore throat, nausea, dizziness and constipation.
CONCLUSION: Contrary to some animal and human studies, this meta-analysis showed that the use of CoQ10 results to non-significant improvement in all components of the UPDRS scores as opposed to placebo. However, the use of CoQ10 is tolerated and seems to be safe but further studies are needed to validate this finding.

Neuroprotection ; Parkinson Disease

Objective: Since the release of vaccines against coronavirus disease 2019 (COVID-19), there have been reports of vaccine-related neurologic complications. This study aimed to perform a descriptive systematic review of movement disorders associated with COVID-19 vaccines. Methods: We described the demographics, clinical presentation, management, outcomes, and proposed pathomechanism of the patients. A systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A standardized tool was used to assess the quality of the cases. Results: We identified 8 articles that met our inclusion criteria; these articles included 10 patients who developed movement disorders after vaccination. The majority were males (n = 8), with a median age of 64.5 years. The most common movement disorder was hemichorea. The rest presented with generalized chorea with myoclonus, cervical dystonia, and akathisia. Most patients respond to immunotherapy. The standardized tool used showed that most studies had a low risk of bias. Conclusion The reported incidence of vaccine-related movement disorders was low based on available published cases.

X linked Dystonia Parkinsonism (XDP) is a progressive movement disorder among male inhabitants of the island of Panay in the Philippinesi, it would start as a focal dystonia then become generalised. The disease is believed to be due to a founder mutation of some 50 meiotic generations ago;;_ Although initially seen in male population, current data shows otherwise. Reports of about 11 female cases are detected and listed in the registry. Male more than female predilection is seen with a ratio of 75:1 iii_ XDP is a very disabling degenerative disease causing involuntary torsion of the body, torticolis, blepharospasm and eventually parkinsonian featuresiv. There is no known treatment, and alleviation of symptoms is difficult to achieve, some patients even die due to infection and self harm. This report will present a case of XDP with predominating parkinsonian feature, who has undergone bilateral Globus Pallidus lnterna(GPi} Deep brain Stimulation (DBS}. After the operation, return to functionality and improvements in activities in daily living is seen. The UPDRS and FMDRS scores show improvement noted on regular follow up. However adverse effects such as slurring of speech and minimal blepharospasm are still observed.

Sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag") is an adult-onset, progressive, debilitating movement disorder first described in Filipino males from Panay Island in 1975. XDP manifests predominantly as torsion dystonia, later combined with or sometimes replaced with parkinsonism. Within the Island of Panay, the preva-lence rate is highest in the province of Capiz, where 1:4000 men suffer from the disorder. There is a high degree of penetrance and generalization. While women often serve as carriers, XDP is not limited to men. An updated XDP Philippine registry (as of January 2010) has identified 505 cases, with 500 males and 5 females. While some report that females may carry a milder form of the disorder, in our experience, both sexes generally follow a similar progressive clinical course.

Human ; Male ; Female ; Aged ; Adult ; Dystonia ; Dystonia Musculorum Deformans ; Dystonic Disorders ; Genetic Diseases, X-linked ; Islands ; Parkinsonian Disorders ; Penetrance

The novel coronavirus disease 2019 (COVID-19) has created a global health impact to millions of people. There have been studies of COVID-19 patients manifesting with neurologic symptoms. Although the number of adult COVID-19 infections diagnosed with Guillain-Barré Syndrome (GBS) is increasing, the occurrence of cases in pediatric population remains limited or perhaps underreported. We report a rare case of an asymptomatic COVID-19 infection manifesting as acute progressive ascending polyneuropathy and hyporeflexia in a 16-year-old teen. The diagnosis of COVID-19 infection was confirmed by reverse transcription polymerase chain reaction for SARS-CoV-2 of oropharyngeal and nasopharyngeal swab specimens. Magnetic resonance imaging of the spine revealed abnormal enhancement of the cauda equina, including the dorsal and ventral roots. Electromyography and nerve conduction studies were compatible with an acute inflammatory demyelinating polyneuropathy subtype of GBS. Although lumbar puncture was not done, the clinical findings and electrodiagnostic tests were both consistent with GBS. The patient had improvement of both motor and sensory functions after completing the treatment of intravenous immunoglobulins. Neurologic manifestations of systemic illness especially in children during this time of pandemic warrants scrutiny, as these may mask a potentially dangerous and infectious ongoing COVID infection.
COVID-19 ; Guillain-Barre Syndrome ; Polyneuropathies ; Pediatrics ; SARS-CoV-2