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Psychosis is a common and often debilitating non-motor symptom of Parkinson’s disease (PD). It typically manifests in the form of well-formed visual hallucinations and minor hallucinations, and, at times, can present with delusions and nonvisual hallucinations. Psychosis is associated with many adverse outcomes in PD, and for that reason, it is essential to recognize and treat the symptoms early. The objective of this review article is to highlight the phenomenology, diagnosis, and pathophysiology of PD-associated psychosis (PD-P) and discuss a step-by-step approach to its management. One of the critical steps in managing PD-P is the identification of potential non-PD causes of psychosis, which often require conservative measures. If no secondary causes are identified, pharmacotherapy should be considered. Role of several drugs including pimavanserin (the only FDA-approved agent for the treatment of PD-P), atypical antipsychotics such as quetiapine and clozapine, and cholinesterase inhibitors such as rivastigmine and donepezil are discussed in this review. In addition, we also highlight the potential role of noninvasive brain stimulation (electroconvulsive therapy and transcranial magnetic stimulation) for the treatment of medication-refractory psychosis.

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Type III GM1 gangliosidosis is the adult or chronic variant of a lysosomal storage disorder, which occurs secondary to deficiency of β-galactosidase. The wishbone pattern of iron deposition which involves the medial and latter parts of the globus pallidus is pathognomonic of this disease. The diagnosis of type III GM1 gangliosidosis should be considered in patient with young onset, progressive generalized dystonia with prominent facial dystonia, and a wishbone pattern of iron deposition.

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BACKGROUND: Non-motor symptoms (NMSs) were a real burden in Parkinson’s disease (PD) and contributed to severe disability, impaired quality of life (QoL), and shortened life expectancy. AIM: The aim of this study was to investigate the prevalence of NMSs in PD and their correlation of NMS with disease duration, severity, and Unified Parkinson's Disease Rating Scale (UPDRS) motor score and their impact on patient’s QoL. MATERIALS AND METHODS: This was a prospective cross-sectional study. Sixty-four patients who were diagnosed by United Kingdom Parkinson's Disease Brain Bank criteria were studied. Non-motor symptom scale (NMSS) analyzed NMS, and motor dysfunction was assessed by the UPDRS II and III during OFF condition and QoL by Parkinson’s Disease Questionnaire-39 (PDQ-39) questionnaire. RESULTS: The prevalence of NMS was 93.75% (n = 60). Most frequent NMS was difficulty in falling asleep (54.7%), urinary urgency (39%), and memory impairment (37.5%). Most disabling symptoms are difficulty in falling asleep (3.34 ± 4.1) and fatigue (2.48 ± 4.2). The total NMSS scores were correlated with Hoehn and Yahr stage, and Movement Disorder Society UPDRS and PDQ-39 scores, but not with duration of disease. Correlation between NMSS and PDQ-39 scores was stronger as compared to the relationship between UPDRS and PDQ-39 scores (r = 0.71 and 0.58, respectively, P = 0.00). CONCLUSION: This study showed the high prevalence of NMSs and value of NMS as predictors of QoL in patients with PD. Therefore, understanding the pathophysiology of these NMSs should be placed at the forefront to develop new therapeutic approaches by improving the QoL of patients with PD.

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AIM: Premotor phase in Parkinson’s disease (PD) begins many years before the onset of motor symptoms. Despite advancement in the understanding of this common neurodegenerative disease, clinical diagnosis is still the gold standard. Various fluid- and tissue-based biomarkers have been postulated to play a role in the pathogenesis of PD. We aimed to study the role of serum DJ-1 and apolipoprotein A1 (Apo A1) in PD. SETTINGS AND DESIGN: A cross-sectional observational study was conducted in the Department of Neurology, in collaboration with the Department of Pharmacology. Cases of PD and healthy controls were recruited over 1 year with written informed consent from all the participants. The study was approved by the institutional ethics committee. SUBJECTS AND METHODS: All cases underwent detailed neurological examination, Hoehn and Yahr staging, and MDS-UPDRS (MDS-UPDRS (Movement Disorders Society–Unified Parkinson Disease Rating Scale) scoring. Serum of all the cases and controls were collected for the estimation of DJ-1 and Apo A1 by enzyme-linked immunosorbent assay. STATISTICAL ANALYSIS: Serum DJ-1 and Apo A1 levels were compared between cases and controls using Mann–Whitney test. Correlation of characteristics was carried out using Spearman correlation coefficient. RESULTS: The study cohort included 77 cases of PD and 69 healthy controls. Serum DJ-1 and Apo A1 levels were found to be significantly lower in cases compared to controls (P < 0.001). However, no correlation was found with age of onset, duration of disease, and severity of illness. A negative correlation was observed between DJ-1 levels and cognitive impairment and bladder dysfunction. CONCLUSION: Low levels of DJ-1 and Apo A1 are found in patients with PD of Indian origin. However, correlation of these biomarkers with progression and severity is needed to be explored in future randomized controlled trials with a large sample size.

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X-linked adrenoleukodystrophy (X-ALD) is a pan-ethnic disorder and affects approximately 1:20,000 males (Moser HW, Mahmood A, Raymond GV. X-linked adrenoleukodystrophy. Nat Rev Clin Pract Neurol 2007;3:140-51; Natarajan A, Christopher R, Netravathi M, Bhat M, Chandra SR. Liquid chromatography-tandem mass spectrometry method for estimation of a panel of lysophosphatidylcholines in dried blood spots for screening of X-linked adrenoleukodystrophy. Clin Chim Acta 2018;485:305-10). Phenotypes include the childhood cerebral form, which affects children who present with rapid cognitive decline. Adolescents and adults present with spastic paraparesis or adrenomyeloneuropathies and also as pure Addison's disease. The less common presentations are psychosis and cortical blindness. A 9-year-old boy presented with progressive cognitive decline, behavioral disturbance, extrapyramidal symptoms, and opisthotonic posturing. Magnetic resonance imaging of brain and blood levels of very long-chain fatty acids lysophosphatidylcholine confirmed X-ALD.

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Episodes of hemifacial spasm (HFS) along with ipsilateral trigeminal neuralgia (TN) is known as painful tic convulsif (PTC). Neurovascular conflict due to vascular compression of involved cranial nerve roots is the most commonly reported pathology resulting in PTC, with ectatic course of vertebrobasilar artery being the most frequent cause. HFS and TN commonly occur in elderly population and atypical causes such as arteriovenous malformation (AVM) may likely affect young patients. To date only three cases of PTC resulting from AVM have been reported. We report a young male presenting with PTC resulting from posterior fossa AVM.

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Drugs, such as dopamine receptor blockers or dopamine depleters, produce a functional dopamine-deficient state mimicking parkinsonism, but presentation with a progressive supranuclear palsy (PSP) is a rare manifestation. We report three patients with a PSP-like presentation, with symmetrical parkinsonism, postural instability, and gaze palsy due to drugs, such as metoclopramide, risperidone, and olanzapine, which reversed after drug withdrawal.

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AIM: The aim of this study was to detect the diagnostic accuracy of a novel app (Neurology Dx) vis-à-vis neurology residents. METHODS: A multicenter cross-sectional study involving seven leading teaching neurology institutes in India was conducted by recruiting 100 neurology residents. Primary outcome was proportion of correctly identified high likely gold standard differential diagnoses. Secondary outcomes were proportions of correctly identified first high likely, first three high likely, first five high likely, and combined moderate plus high likely gold standard differentials. RESULTS: Four sets comprising 15 movement disorder vignettes each (total 60) were tested on 100 neurology residents (one set for each resident) and also on the app (60 vignettes). Residents correctly identified the gold standard “high likely” differentials with a frequency of 13.6% as against 41.5% by the app (95% confidence interval [CI]: 21.9–34.1). On combining “high” and “moderate likely” differentials, residents could accurately identify gold standard differentials with a frequency of 10.8% as against 37.9% by the app (95% CI: 22.6–31.9). The residents correctly identified first five high likely gold standard differentials with a frequency of 13.5% versus 23.7% by the app (95% CI: 5.3–15.9). The residents correctly identified first three high likely gold standard differentials with a frequency of 13.0% versus 15.8% by the app (95% CI: -1.2–7.9). Residents correctly identified the first “high likely” gold standard differential in 32.3% as against 35% by the app (95% CI: -8.4–15.6). CONCLUSIONS AND RELEVANCE: This study suggests that an app (Neurology Dx) is capable of generating differential diagnoses to complement clinical reasoning of neurology residents (CTRI/2017/06/008838).

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Neurogenic orthostatic hypotension is a common disorder often seen in elderly patients affected with neurodegenerative disorders and peripheral neuropathies. Defective baroreflex-mediated sympathetic activation in response to standing results in neurogenic orthostatic hypotension. Lightheadedness, dizziness, blurring of vision, palpitations, and/or recurrent syncope in response to sudden postural change or prolonged standing are the characteristic symptoms presented by patients with orthostatic hypotension. Orthostatic symptoms may be further aggravated by certain medications, fluid depletion, food intake, increased temperature, or physical deconditioning. Bedside orthostatic test, head-up tilt table test, and 24-h ambulatory blood pressure monitoring are the important tests to diagnose orthostatic hypotension. Discontinuation of medications that cause or exacerbate orthostatic hypotension and non-pharmacological approaches should be initially tried to treat neurogenic orthostatic hypotension. If the patient does not have significant improvement, add-on pharmacotherapy should be administered. Midodrine, droxidopa, and fludrocortisone are the commonly administered medications for treating neurogenic orthostatic hypotension. Systematic reviews have shown that there is moderate level of evidence that droxidopa and midodrine improve orthostatic hypotension in the short term. Fludrocortisone is included in the treatment guidelines based on expert opinion, despite having low level of evidence. However, there is no much evidence for the long-term efficacy of any pharmacological agent used to treat orthostatic hypotension. Treatment of neurogenic orthostatic hypotension should be periodically assessed. Predominantly, it involves measuring symptomatic benefit including impact on activities of daily living as well as blood pressure monitoring.

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