Acquired hyperexcitable peripheral nerve disorders: Clinical and laboratory features, therapeutic responses, and long-term follow-up.

INTRODUCTION/AIMS: Hyperexcitable peripheral nerve disorders (HPNDs) are rare. Although their clinical and laboratory features have been well studied, information on treatment and follow-up is limited. The aim of this study is to explore the long-term clinical, investigative, and therapeutic profile of patients with acquired HPNDs. METHODS: This study retrospectively analyzed patients from a single tertiary care center with HPND (January 2012 to January 2022). Patients were recruited according to published inclusion and exclusion criteria. Details of clinical features, diagnostic tests, therapeutic interventions, and follow-up were recorded. This study included patients with follow-up of 2 or more years. RESULTS: A total of 32 patients (M = 26, F = 6) were studied. The common clinical features included myokymia, neuropathic or shock-like pain, cramps, sleep disturbances, encephalopathy, cerebellar ataxia, and seizures. A total of 81.25% of patients responded favorably to corticosteroids and membrane stabilizers. Among the nonresponders, five received intravenous immunoglobulin (IVIG), and one received plasma exchange (PLEX). Two patients required rituximab due to poor responses to the above treatments. The mean duration of response was 6 weeks (4-24 weeks) from the initiation of treatment. All patients had favorable outcomes, reaching clinical remission within 1-5 years from the initiation of treatment. Only two patients had relapses. Immunotherapy could be stopped in 78% of patients within 3 years and 100% by 5 years. DISCUSSION: Chronic immunosuppression starting with corticosteroids is required for satisfactory outcomes of HPNDs. These disorders usually run a monophasic course, and relapses are uncommon.

Diagnostic Tests in Neoplastic Meningitis: Lessons Learnt from Three Patients.

Neoplastic meningitis (NM) poses diagnostic challenges and investigations need to be chosen carefully. We present three cases of NM with distinct learning points. In case 1, MRI was diagnostic of melanosis; in case 2, ventricular CSF showed malignant cells when lumbar CSF repeatedly failed to show them; and in the third, whole body PET scan diagnosed the tumour when other tests were negative. A comparative evaluation of various diagnostic modalities used in suspected NM is provided.

Methodological difficulties in the conduct of neuroepidemiological studies in low- and middle-income countries.

BACKGROUND: The majority of people with epilepsy (PWE) live in low- and middle-income countries (LMICs). However, they remain largely untreated and the bulk of resources are used to treat patients in the developed world. This disparity constitutes a challenge for neuroepidemiological studies on a global scale. In the past, several studies have focused on diverse populations in disparate countries at various periods of time and for particular purposes. The specificity of different contexts and circumstances makes it difficult to analyse PWE as a group either qualitatively or quantitatively. Such methodological limitations are further complicated by a lack of logistical support. There is a lack of interest in conducting studies, which results in inadequate funding and, in addition, there is the considerable challenge of publishing research reports from LMICs in peer-reviewed international journals. METHODS: This paper focuses on methodological problems related to studies in LMICs and attempts to give the reasons for their limitations using epilepsy as an example. RESULTS: Regional conditions and environmental factors must be given careful consideration in the research design because of the importance of understanding the challenges of living in these environments. There are further limitations to the successful implementation of studies. Existing information on epilepsy is often not readily accessible; there is a lack of census data, and migratory patterns into cities make enumeration and sampling even more challenging. As there is usually no well-developed healthcare system a door-to-door screening process is often the only way to identify those with convulsive epilepsy. The questionnaire and study design should preferably be adapted from standardized protocols, and pre-tested and validated in local conditions. CONCLUSIONS: Systematic reviews and meta-analyses of studies in LMICs can provide data on the burden, risk factors, treatment and outcome of epilepsy only if the primary studies used are properly conducted using uniform and comparable methodology. The use of consistent replicable neuroepidemiological methods in primary studies and systematic reviews enable reduction of the treatment gap and better epilepsy care.

An interesting case of headache.

A 35-year-old businessman with a history of migraine with aura developed new neck pain while lying on a sofa in his home. He was given neck massage and physical therapy for a day, and subsequently after two days developed severe generalized headache when sitting or standing. He was hospitalized in his hometown. Investigations revealed venous sinus thrombosis and bilateral thin subdural collections for which he was anticoagulated. Headache improved and then worsened and became severe in all positions. He was then admitted under our care. MRI scan of the brain at our hospital showed left subdural hematoma with midline shift. It required urgent evacuation. His previous first brain MRI was re-evaluated. It showed characteristic features of spontaneous intracranial hypotension (SIH). If not recognized early, SIH results in various complications, some of which require immediate intervention. Any change in the pattern of headache in SIH one must alert the clinician due to the possibility of one of its complications.

Hypertension in the Parsi community of Bombay: a study on prevalence, awareness and compliance to treatment.

BACKGROUND: Uncontrolled hypertension (HT) is an established risk factor for the development of vascular diseases. Prevalence varies in different communities and no such study has been conducted in the Parsi community living in Bombay, India. The objectives of this study were to determine the prevalence, awareness, compliance to medication and control of HT in this community. METHOD: We used a 1 in 4 random selection of subjects who were > or = 20 years of age. A questionnaire was administered and the blood pressure (BP) was measured by a doctor. HT was defined as diastolic blood pressure (DBP) > or = 90 mm Hg +/- systolic pressure (SBP) > or = 140 mm Hg. Isolated systolic hypertension (ISH) was defined as SBP > or = 160 mm Hg with DBP < 90 mm Hg. Subsequently, we reanalysed the data using current definition of ISH as SBP > or = 140 mm Hg with DBP < 90 mm Hg. RESULTS: 2879 subjects > or = 20 years of age were randomly selected of which 2415 (84%) participated in the study. The overall prevalence of HT in the community was 36.4%, of whom 48.5% were unaware of their hypertensive status. Of those aware of having HT, 36.4% were non-compliant with their anti-hypertensive drugs and only 13.6% had optimally controlled HT. Prevalence of ISH using the present criteria was 19.5% and 73% of hypertensives > or = 60 years had ISH. CONCLUSION: This study shows that prevalence of HT in the Parsi community is high and nearly half are unaware of their hypertensive status. ISH is the dominant form of HT in the elderly. Compliance to treatment is poor and optimal BP control is achieved in only a small minority. The study highlights the need for regular screening coupled with educational programs to detect and optimally treat HT in the community.