Parkinsonian syndromes presenting with circadian rhythm sleep disorder–advanced sleep-phase type.

Background. Circadian rhythm sleep disorder–advanced sleep-phase type is a relatively uncommon disorder, mostly seen among the elderly population. Impaired circadian rhythms have been reported in neurodegenerative conditions; however, there are no reports of any circadian rhythm sleep disorder among patients with Parkinsonian syndromes. We report two patients who presented with this circadian rhythm disorder, and were then diagnosed with a Parkinsonian syndrome. The cases. A 65-year-old retired man presented with history of abrupt change in sleep schedules, sleeping around 6.30–7 p.m. and waking up around 3–4 a.m. for the last 2 months. On detailed examination, the patient was observed to have symmetrical bradykinesia and cogwheel rigidity of limbs. A diagnosis of multiple system atrophy was made, supported by MRI findings and evidence of autonomic dysfunction. Symptoms of change in sleep–wake cycles resolved over the next 1 year, while the patient was treated with dopaminergic therapy. A 47-year-old man, who was being evaluated for presurgical investigation for refractory temporal lobe epilepsy, presented with complaints suggestive of dysarthria, bradykinesia of limbs and frequent falls for 5 months. Simultaneously, he began to sleep around 7 p.m. and wake up at about 2–3 a.m. Examination revealed severe axial rigidity, restricted vertical gaze and bradykinesia of limbs. A diagnosis of progressive supranuclear palsy was made. Conclusion. This is the first report of Parkinson’s plus syndromes presenting with a circadian rhythm sleep disorder– advanced sleep-phase type. More prospective assessment for circadian sleep disorders may introduce useful insights into similar associations. Natl Med J India 2015;28:233–5

Consensus & Evidence-based INOSA Guidelines 2014 (First edition).

Obstructive sleep apnoea (OSA) and obstructive sleep apnoea syndrome (OSAS) are subsets of sleep-disordered breathing. Awareness about OSA and its consequences amongst the general public as well as the majority of primary care physcians across India is poor. This necessiated the development of the INdian initiative on Obstructive Sleep Apnoea (INOSA) guidelines under the auspices of Department of Health Research, Ministry of Health & Family Welfare, Government of India. OSA is the occurrence of an average five or more episodes of obstructive respiratory events per hour of sleep with either sleep related symptoms or comorbidities or >15 such episodes without any sleep related symptoms or comorbidities. OSAS is defined as OSA associated with daytime symptoms, most often excessive sleepiness. Patients undergoing routine health check-up with snoring, daytime sleepiness, obesity, hypertension, motor vehicular accidents and high risk cases should undergo a comprehensive sleep evaluation. Medical examiners evaluating drivers, air pilots, railway drivers and heavy machinery workers should be educated about OSA and should comprehensively evaluate applicants for OSA. Those suspected to have OSA on comprehensive sleep evaluation should be referred for a sleep study. Supervised overnight polysomnography (PSG) is the “gold standard” for evaluation of OSA. Positive airway pressure (PAP) therapy is the mainstay of treatment of OSA. Oral appliances are indicated for use in patients with mild to moderate OSA who prefer oral appliances to PAP, or who do not respond to PAP or who fail treatment attempts with PAP or behavioural measures. Surgical treatment is recommended in patients who have failed or are intolerant to PAP therapy.

Saccadic eye movements in Parkinson's disease.

This review focuses on saccadic eye movement research in Parkinson’s disease (PD) patients. Results from various studies related to Parkinson disease and saccades have been discussed in terms of various saccadic parameters like latency, amplitude, velocity and gain. Neural circuitry of saccadic eye movements and cognitive processes and it’s relation with altered saccadic performance in Parkinson disease has been discussed here. This article also covers various research paradigms commonly used to study saccades. Eff ects of medication on saccadic parameters in PD patients have also been discussed along with the eff ects of deep brain stimulation of subthalamic nucleus on saccadic performance in PD patients. Literature review was done using online Pubmed search engine and National Medical Library.

Surgical outcome of cortical dysplasias presenting with chronic intractable epilepsy: A 10-year experience.

Background: There has been sparse description of cortical dysplasias (CDs) causing intractable epilepsy from India. Aim: Clinical retrospective study of CDs causing intractable epilepsy that underwent surgery. Materials and Methods: Fifty-seven cases of CDs reviewed (1995 till July 2006) are presented. All patients had intractable epilepsy, and underwent a complete epilepsy surgery workup (inter ictal electroencephalography (EEG), video EEG, MRI as per epilepsy protocol, SPECT {interictal, ictal with subtraction and co-registration when required}, and PET when necessary). Surgical treatment included a wide exposure of the pathology with a detailed electrocorticography under optimal anesthetic conditions. Mapping of the sensori-motor area was performed where indicated. Procedures included resection either alone or combined with multiple subpial transactions when extending into the eloquent areas. Results: Our study had 28 (49.12%) cases of isolated focal CDs, and 29 (50.67%) with dual pathology. Average age at the time of onset of seizures in our series was 7.04 years (three months to 24 years), and average age at the time of surgery was 10.97 years (eight months to 45 years). Among coexistent pathologies, one had associated MTS, 16 had coexistent gangliogliomas and 12 (dysembryonic neuroepithelial tumor) DNTs. At an average follow-up of 3.035 years (range 5-10 years), three patients were lost to follow-up. Fifty-one per cent (29/57) patients had a good outcome (Engel Grade I) and 26%(15/57) had a Grade II outcome. Conclusion: Cortical dysplasias have a good outcome if evaluated and managed with concordant electrical and imaging modalities.

IgM anti-GM1 antibody titers in patients with monomelic amyotrophy.

BACKGROUND: Monomelic amyotrophy (MMA) is a benign motor neuron disorder, which particularly affects young people and the etiology is still unknown. Gangliosides are located on the outer surface of motor neurons. Anti-GM1 antibodies have been found to be elevated in multi-focal motor neuropathy with conduction block and other neurological diseases, which may have therapeutic implication. AIM: To evaluate IgM anti-GM1 antibody titers in patients of monomelic amyotrophy. SETTING AND DESIGN: prospective controlled study. MATERIALS AND METHODS: Forty-six clinically and electrophysiologically diagnosed cases of MMA were assessed for IgM anti-GM1 antibody titers by enzyme-linked immunosorbent assay (ELISA) method and compared with titers in healthy controls, cases of amyotrophic lateral sclerosis (ALS) and acute inflammatory demyelinating polyneuropathy (AIDP). Titer of 800 units was taken as upper limit of normal (Buhlmann Laboratories AG, Switzerland). STATISTICAL ANALYSIS USED: one-way ANOVA. RESULTS: The mean age of 46 patients with MMA was 24.5 (+/- 7.3) years, with male female ratio of 44:2. The mean age of 19 healthy controls was 24.1 (+/- 3) years with male: female ratio of 18:1. Five (26%) individuals in the healthy control group, 22 (48%) patients of MMA, four (30%) of ALS and five (50%) of AIDP had high titers of IgM anti-GM1 antibody (P> 0.05). CONCLUSIONS: Although larger number of patients with MMA had higher IgM anti-GM1 antibody titers, the difference was not statistically significant from titers of healthy individuals and of patients in the ALS and AIDP group.

A clinical study of non-parkinsonian and non-cerebellar tremor at a specialty movement disorders clinic.

BACKGROUND: No Indian studies have focused on the clinical aspects of tremor. AIMS: To study the distribution of various etiological types of tremor disorders at a Movement Disorders clinic of a large, tertiary care hospital in India and to study the clinical characteristics of essential tremor [ET]. SETTING AND DESIGN: Prospective cross-sectional study at a tertiary care specialty clinic. MATERIAL AND METHODS: Patients presenting with tremor as the chief complaint, with no features suggestive of parkinsonism, cerebellar disorder or acute central nervous system disorder, were included. Patients were classified into different etiological categories from detailed history. All patients diagnosed as ET, were further interrogated for a detailed family history and examined for characteristics of tremor. These patients were then classified into 'definite', 'probable' and 'possible' ET. RESULTS: One hundred and six patients (mean age 44.4 + 15.1 years) were examined during the study period. ET (59.4%) and dystonic tremor (21.7%) were the commonest types. Only 43% patients of ET reported progression; response to alcohol was seen in only a single patient, a positive family history was present in 52.4% and in 36.4% the inheritance was of an autosomal dominant pattern. CONCLUSION: ET and dystonic tremor are the commonest causes of tremor presenting to a specialty Movement Disorders clinic. Most patients with ET have high-frequency tremor, with mild asymmetry in 40% cases. Alcohol responsiveness may not be a useful tool in the diagnosis of ET.