Gender differences in quality of life and psychiatric comorbidities among persons with juvenile myoclonic epilepsy: A single-center cross-sectional study.

Objectives: Juvenile myoclonic epilepsy (JME) is the most common idiopathic generalized/genetic epilepsy syndrome. Gender differences are known in clinical presentation, with a well-identified female predilection. We aimed to study gender-based differences in quality of life (QoL) and psychiatric comorbidities among persons with JME. Materials and Methods: This was a cross-sectional study conducted at a teaching hospital in Delhi, India. Persons above 11 years of age with JME diagnosed according to the International League Against Epilepsy criteria established in 2001 were enrolled. QoL assessment was made using Quality of Life in Epilepsy Inventory-Adolescents-48 (QOLIE-AD-48) and Patient-Weighted Quality of Life in Epilepsy Inventory 31 (QOLIE-31-P) for adolescent and adult patients, respectively. For the assessment of psychiatric comorbidities, participants were administered the Mini-International Neuropsychiatric Interview (M.I.N.I). Participants who tested positive for psychiatric comorbidities on M.I.N.I subsequently underwent the Diagnostic and Statistical Manual-5 categorization. Results: We enrolled 50 patients with JME. Eighteen (36%) were male and 32 (64%) were female patients. The median age of males at study enrollment was 23.5 (range 15-38) years. The median age of females was 22 (16-48) years. The median QOLIE-31-P score among males was 68.31 (37.13-91.82) and for females was 66.9 (31.7-99.1). The median overall QoL score for males was 65 (25-87.5), which qualified as "fair" QoL. For females, the median overall QoL score was 62.5 (10-87.5) which also qualified as "fair" QoL. No significant difference was noted between genders in QoL (P = 0.723). Among males, 55.5% had psychiatric comorbidity. Of these, two had mild depression and eight had anxiety. Among female patients, 34.4% had comorbid psychiatric issues; 6 had anxiety and 5 had depression. No significant difference was noted between genders (P = 0.9136). Conclusion: Persons with JME do not have gender-stratified differences in terms of psychiatric comorbidities and QoL despite differences in exposure to antiseizure medications and other gender-related factors. All persons with JME should be screened for psychiatric comorbidities, specifically anxiety, and depression.

Effect of aerobic exercise training on EEG: event-related potential and neuropsychological functions in depressed elderly with mild cognitive impairment.

Increased depressive severity has been linked to neurocognitive impairment. Aerobic exercise (AE) is an efficient technique for improving cognitive function. However, studies indicating the importance of AE to neurophysiological and neuropsychological functions in the depressed elderly using event-related potentials (ERPs) are scarce. Objectives: This study aimed to identify the potential benefits of AE on neurophysiological and neuropsychological functions. Methods: A total of 30 depressed older adults (AE group: n=15; control group (CG): n=14) were recruited based on the inclusion and exclusion criteria. The AE group was subjected to an 8-week-period AE program (3 times/week for 30 min per session) at moderate intensity, determined using heart rate maximum (HRmax). The training intensity was set at 50% HRmax and increased by 5% in subsequent weeks. Pre- and post-training measures for neurophysiological function were tested using ERP-P300 (amplitude-µV and latency-ms) and also for neuropsychological functions using the trail making test (TMT), mini mental status examination (MMSE), and everyday cognition questionnaire (ECog). Results: In the experimental group, statistically significant improvements were observed when analyzed for all 3 (group-by-time interaction effect, main effect of time, and main effect of group), in both neurophysiological functions (*p<0.001) and neuropsychological functions (*p<0.001), except for ECog scores, where the results were insignificant for the main effect of a group. Correlation analysis demonstrated no association between neurophysiological and neuropsychological functions (*p>0.05). Conclusion: Findings showed that 8 weeks of AE training may be a promising approach to improve cognitive functions in depressed older adults. However, considering relatively small number of patients, the question arises for effectiveness in other populations.

O aumento da gravidade de quadros de depressão tem sido associado a comprometimento neurocognitivo. O exercício aeróbico (EA) é uma técnica eficiente para melhorar a função cognitiva. No entanto, estudos sugerindo a importância de EA para funções neurofisiológicas e neuropsicológicas usando potenciais relacionados a eventos (PRE) em idosos deprimidos são escassos. Objetivo: Identificar os potenciais benefícios do EA nas funções neurofisiológicas e neuropsicológicas. Métodos: Vinte e nove idosos deprimidos (grupo EA: n=15; grupo controle: GC: n=14) foram recrutados segundo critérios de inclusão e exclusão. O grupo EA foi submetido a um programa de EA de oito semanas (3 vezes/semana em sessões de 30 minutos cada) em intensidade moderada, determinada pela frequência cardíaca máxima (FCmáx). A intensidade do treinamento foi fixada em 50% da FCmax e aumentada em 5% nas semanas subsequentes. As medidas pré- e pós-treinamento para a função neurofisiológica foram testadas pelo ERP-P300 (amplitude-µV e latência-ms) e também para as funções neuropsicológicas usando o Trail Making Test (TMT), o Mini Mental Status Examination (MMSE) e o Everyday Cognition Questionnaire (ECog). Resultados: No grupo experimental, melhorias estatisticamente significativas foram observadas quando analisadas para todos os 3 (efeito de interação grupo por tempo, efeito principal do tempo e efeito principal do grupo), em ambas as funções neurofisiológicas (*p<0,001) e funções neuropsicológicas (*p<0,001), exceto para escores ECog, onde os resultados foram insignificantes para o efeito principal de um grupo. A análise de correlação não demonstrou nenhuma associação entre funções neurofisiológicas e neuropsicológicas (*p>0,05). Conclusão: Os achados mostraram que 8 semanas de treinamento de EA podem ser uma abordagem promissora para melhorar as funções cognitivas em idosos deprimidos. No entanto, considerando um número relativamente pequeno de pacientes, surge a questão da eficácia em outra população.

Perfusion CT imaging as a diagnostic and prognostic tool for dementia: prospective case-control study.

BACKGROUND: As functional changes precede structural changes in dementia, we aimed to elucidate changes on cerebral perfusion CT (PCT) for early diagnosis of dementia; and to differentiate Alzheimer's disease (AD) from vascular dementia (VaD). We also aimed to study correlation between Montreal Cognitive Assessment (MOCA) score and PCT parameters. METHODS: We conducted a prospective case-control study enrolling 25 dementia patients (15 cases of VaD, 10 cases of AD) and 25 age-matched controls. PCT was performed on a 256-slice CT scanner. Using perfusion software, colour maps were generated for cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time and time-to-peak. These colour maps were first visually inspected for any abnormalities. Subsequently, quantitative assessment of perfusion parameters was done using symmetrical freehand region of interests drawn in bilateral frontal, temporal, parietal regions, basal ganglia and hippocampi. RESULTS: Strategic infarcts were present in 93.3% cases and white matter ischaemic changes in 100% cases of VaD. A global reduction in CBF and CBV was also observed in cases of VaD; whereas these parameters were significantly lower mainly in temporoparietal regions and hippocampi of patients with AD. There was significant positive correlation between MOCA score and various perfusion parameters in both forms of dementia. CONCLUSION: PCT is a reliable imaging modality for early diagnosis of dementia and in differentiating VaD from AD. As perfusion parameters show positive correlation with MOCA score, they could be used as a surrogate marker of cognitive status in the follow-up of patients with dementia.

Effect of aerobic exercise training on EEG: event-related potential and neuropsychological functions in depressed elderly with mild cognitive impairment / Efeito do treinamento de exercício aeróbio no EEG: potencial relacionado a eventos e funções neuropsicológicas em idosos deprimidos com comprometimento cognitivo leve

ABSTRACT. Increased depressive severity has been linked to neurocognitive impairment. Aerobic exercise (AE) is an efficient technique for improving cognitive function. However, studies indicating the importance of AE to neurophysiological and neuropsychological functions in the depressed elderly using event-related potentials (ERPs) are scarce. Objectives: This study aimed to identify the potential benefits of AE on neurophysiological and neuropsychological functions. Methods: A total of 30 depressed older adults (AE group: n=15; control group (CG): n=14) were recruited based on the inclusion and exclusion criteria. The AE group was subjected to an 8-week-period AE program (3 times/week for 30 min per session) at moderate intensity, determined using heart rate maximum (HRmax). The training intensity was set at 50% HRmax and increased by 5% in subsequent weeks. Pre- and post-training measures for neurophysiological function were tested using ERP-P300 (amplitude-μV and latency-ms) and also for neuropsychological functions using the trail making test (TMT), mini mental status examination (MMSE), and everyday cognition questionnaire (ECog). Results: In the experimental group, statistically significant improvements were observed when analyzed for all 3 (group-by-time interaction effect, main effect of time, and main effect of group), in both neurophysiological functions (*p<0.001) and neuropsychological functions (*p<0.001), except for ECog scores, where the results were insignificant for the main effect of a group. Correlation analysis demonstrated no association between neurophysiological and neuropsychological functions (*p>0.05). Conclusion: Findings showed that 8 weeks of AE training may be a promising approach to improve cognitive functions in depressed older adults. However, considering relatively small number of patients, the question arises for effectiveness in other populations.

RESUMO. O aumento da gravidade de quadros de depressão tem sido associado a comprometimento neurocognitivo. O exercício aeróbico (EA) é uma técnica eficiente para melhorar a função cognitiva. No entanto, estudos sugerindo a importância de EA para funções neurofisiológicas e neuropsicológicas usando potenciais relacionados a eventos (PRE) em idosos deprimidos são escassos. Objetivo: Identificar os potenciais benefícios do EA nas funções neurofisiológicas e neuropsicológicas. Métodos: Vinte e nove idosos deprimidos (grupo EA: n=15; grupo controle: GC: n=14) foram recrutados segundo critérios de inclusão e exclusão. O grupo EA foi submetido a um programa de EA de oito semanas (3 vezes/semana em sessões de 30 minutos cada) em intensidade moderada, determinada pela frequência cardíaca máxima (FCmáx). A intensidade do treinamento foi fixada em 50% da FCmax e aumentada em 5% nas semanas subsequentes. As medidas pré- e pós-treinamento para a função neurofisiológica foram testadas pelo ERP-P300 (amplitude-μV e latência-ms) e também para as funções neuropsicológicas usando o Trail Making Test (TMT), o Mini Mental Status Examination (MMSE) e o Everyday Cognition Questionnaire (ECog). Resultados: No grupo experimental, melhorias estatisticamente significativas foram observadas quando analisadas para todos os 3 (efeito de interação grupo por tempo, efeito principal do tempo e efeito principal do grupo), em ambas as funções neurofisiológicas (*p<0,001) e funções neuropsicológicas (*p<0,001), exceto para escores ECog, onde os resultados foram insignificantes para o efeito principal de um grupo. A análise de correlação não demonstrou nenhuma associação entre funções neurofisiológicas e neuropsicológicas (*p>0,05). Conclusão: Os achados mostraram que 8 semanas de treinamento de EA podem ser uma abordagem promissora para melhorar as funções cognitivas em idosos deprimidos. No entanto, considerando um número relativamente pequeno de pacientes, surge a questão da eficácia em outra população.

Association of impaired cognitive function with balance confidence, static balance, dynamic balance, functional mobility, and risk of falls in older adults with depression.

Objectives: Increased depression severity has been linked to cognitive impairment (CI). Importantly, CI is a known risk factor for impaired balance and falls. Therefore, this study aims to explore the relationship between CI and neuromuscular functions and secondarily it aims to find out if CI is a potential predictor for neuromuscular functions deficits in depressed elderly. Methods: Eighty-four depressed elderly participated in the study. Assessment for CI symptoms were done using Mini Mental Status Examination (MMSE) in subjects having confirmed depression. Neuromuscular functions such as balance confidence, static and dynamic balance, functional mobility, and fall risk were subjectively assessed using Activities-specific Balance Confidence (ABC) Scale, Berg Balance Scale (BBS), Timed Up and Go (TUG) Test, and Performance Oriented Mobility Assessment (POMA), respectively. Results: Pearson's analysis revealed that there was moderate positive linear-correlation between MMSE and BBS (R = 0.382, p = <0.001) and between MMSE and ABC (R = 0.229, p = 0.036*). Further, regression analysis (R 2) revealed that MMSE significantly predicted the neuromuscular functions using BBS [F(1, 82) = 14.013, p < 0.001, with an R 2 of 0.146] and ABC [F(1, 82) = 4.545, p= 0.036*, with an R 2 of 0.053]. Conclusion: Results of this study points to an impaired CI as a possible factor in development of neuromuscular function impairment in depressed elderly.

Event-related potential and neuropsychological function in depressed older adults with cognitive impairment: A correlational study.

Background: Increased depression severity has been linked to cognitive functioning impairment, such as deficits in episodic memory and executive function, causing difficulties in planning strategies, which ultimately lead to impaired decision-making functions. There are number of ways to assess cognitive functions, two most important and routinely done tests are neuropsychological test battery (NBT) and event-related potentials (ERPs). Objective: This study examines the relationship between conventional neuropsychological tests assessing various cognitive domains and an ERP-P300 in depressed older adults. Methods: Forty-six depressed elderly subjects participated in the study. NBT (Pennsylvania's Penn Computerized Neurocognitive Battery [Penn CNP]) assessing attention, episodic memory, working memory, social cognition, complex cognition, and sensorimotor speed and ERP-P300 (amplitude µV and latency ms) was recorded using an auditory oddball paradigm. Results: Correlation test was run and Pearson's analysis and revealed that there was a negative statistically significant linear correlation between working memory on NBT and P300 wave amplitude on ERP-P300 (r = -0.34, P = 0.021) and between complex cognition on NBT and P300 wave latency on ERP-P300 (r = -0.47, P < 0.001). No correlation was found between other tests on NBT and ERP-P300 wave characteristics. Further, the regression analysis (R 2) revealed that P300 amplitude was found to significantly predict the working memory (R 2 = 0.116) and P300 latency was found to significantly predict the complex cognition (R 2 = 0.224). Conclusion: Therefore, we conclude that neurophysiological measurements cannot be substituted by neuropsychological tests or vice versa; rather, higher brain functions should be estimated by both of the methods.

Cranial electrostimulation improves slow wave sleep in collegiate population: a polysomnographic study.

Objective: Sleep disturbance is quite prevalent among students, which leads to deleterious consequences on health. Cranial electrostimulation (CES) has been speculated to entrain cortical slow waves; therefore, we investigated the efficacy of cranial electrostimulation to improve slow wave sleep in collegiates. Methods: Twenty-eight students with Pittsburgh sleep quality index (PSQI) score >5 were randomly assigned into two groups: CES and control. Participants in CES group completed 60 minutes of CES intervention for 12 weeks with 100 µA microcurrent and 0.5 Hz frequency parameters during night. Pre- and post-intervention measures were taken for sleep architecture using over-night polysomnography (PSG) and sleep quality using PSQI. Participants were instructed to report to the laboratory at 10:00 p.m. and PSG was performed with electroencephalograms (EEG), chin electromyography (EMG) and bilateral electrooculogram (EOG) in place. Sleep stages were scored manually in accordance with the new AASM guidelines. PSG variables reported in the present study are sleep latency (SL), total sleep time (TST), percentage of N1, N2, N3, NREM (non-rapid eye movement), REM (rapid eye movement) and sleep efficiency (SE%). Results: After ascertaining the comparability of demographic and sleep variables at baseline for both the groups, a 2X2 mixed model ANOVA was employed. Significant between-group differences were obtained for N1% and N3% such that N1% decreased and N3% increased post CES. However, other PSG variables, along with PSQI score did not demonstrate statistically significant between-group difference. Discussion: The present study demonstrated that 12-weeks of CES improved N3% and reduced N1%. Future researches should be undertaken to build upon the findings of present study.

Perfusion CT imaging as a diagnostic and prognostic tool for dementia: prospective case-control study.

BACKGROUND: As functional changes precede structural changes in dementia, we aimed to elucidate changes on cerebral perfusion CT (PCT) for early diagnosis of dementia; and to differentiate Alzheimer's disease (AD) from vascular dementia (VaD). We also aimed to study correlation between Montreal Cognitive Assessment (MOCA) score and PCT parameters. METHODS: We conducted a prospective case-control study enrolling 25 dementia patients (15 cases of VaD, 10 cases of AD) and 25 age-matched controls. PCT was performed on a 256-slice CT scanner. Using perfusion software, colour maps were generated for cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time and time-to-peak. These colour maps were first visually inspected for any abnormalities. Subsequently, quantitative assessment of perfusion parameters was done using symmetrical freehand region of interests drawn in bilateral frontal, temporal, parietal regions, basal ganglia and hippocampi. RESULTS: Strategic infarcts were present in 93.3% cases and white matter ischaemic changes in 100% cases of VaD. A global reduction in CBF and CBV was also observed in cases of VaD; whereas these parameters were significantly lower mainly in temporoparietal regions and hippocampi of patients with AD. There was significant positive correlation between MOCA score and various perfusion parameters in both forms of dementia. CONCLUSION: PCT is a reliable imaging modality for early diagnosis of dementia and in differentiating VaD from AD. As perfusion parameters show positive correlation with MOCA score, they could be used as a surrogate marker of cognitive status in the follow-up of patients with dementia.

Neurological safety and efficacy of darifenacin and mirabegron for the treatment of overactive bladder in patients with history of cerebrovascular accident: A prospective study.

OBJECTIVES: To evaluate the neurological safety and clinical efficacy of darifenacin and mirabegron in patients with a history of cerebrovascular accident (CVA) who had overactive bladder (OAB) symptoms. METHODS: This prospective randomized study, approved by the institute's ethics committee, was carried out at a tertiary care center from December 2018 to June 2020. Treatment naïve adult patients with a past history of CVA with stable neurological status for atleast past 3 months with symptoms of OAB for 3 or more months were included. Eligible patients received either darifenacin or mirabegron for a period of 3 months and various parameters on the 3-day International Consultation on Incontinence Questionnaire (ICIQ) bladder diary, the Montreal Cognitive Assessment-Basic score (MoCA-B), and the adverse events at 3 months posttreatment were compared to that at the baseline. RESULTS: A total of 60 patients were included, 30 in each arm. After 3 months of treatment with darifenacin or mirabegron, the majority of the ICIQ bladder diary parameters improved and there was no deterioration in the cognitive function as noted on the MoCA-B score in either of the arms. On intergroup comparison, the mean change in bladder diary parameters and the MoCA-B scores was similar between the two groups. CONCLUSION: Darifenacin and mirabegron, in the short term, do not adversely affect the cognitive function in patients with a history of CVA with OAB symptoms. Both are safe and effective treatment options in patients with OAB post-CVA.

Cognitive Dysfunction in Juvenile Myoclonic Epilepsy (JME) - A Tertiary Care Center Study.

BACKGROUND AND AIM: Epilepsy often leads to cognitive impairment. Idiopathic generalized epilepsy as a group is considered to be benign in terms of its effects on cognition. Though, neuropsychological testing reveals subtle frontal impairment in patients with juvenile myoclonic epilepsy (JME). The aim of this study is to evaluate cognitive dysfunction in patients with JME. METHOD: We compared 50 JME patients and 50 age and sex matched healthy controls above 12 years of age on various cognitive tests which included Mini Mental State Examination (MMSE), Frontal Assessment Battery (FAB), Executive Interview (EXIT), PGI Memory Scale (PGIMS), Clock Drawing Test (CDT), Cube copying test (CCT), and Nahor Benson Test (NBT). We correlated the cognitive dysfunction with education level, age of onset, duration of epilepsy, electroencephalogram (EEG) abnormalities, treatment, and seizure control status. RESULTS: JME patients performed significantly worse on MMSE (P = 0.001), PGI MS (P value = 0.001), FAB (P =.001), EXIT (P =.001), CDT (P =.02), and CCT (P =.001) when compared to the controls. JME patients had impaired attention, verbal fluency, design fluency, verbal memory, visual memory, conceptualization, set shifting, mental flexibility, response inhibition, and visuospatial functions. Cognitive dysfunction correlated with education level, duration of epilepsy and EEG abnormality. No correlation was seen with seizure frequency or type of antiepileptic therapy. CONCLUSIONS: JME patients demonstrate both frontal and parietooccipital lobe dysfunction. Hence detailed higher mental function tests supplemented by functional neuroimaging studies should be done in JME patients for their comprehensive management. This would also enhance our knowledge about the pathogenesis of JME.

Association of Dengue with Longitudinally Extensive Transverse Myelitis and Subarachnoid Hemorrhage: An Unusual Presentation.

Association of dengue fever with transverse myelitis is a rare phenomenon; involvement of a long segment is even rarer. We describe a middle-aged female who presented with weakness of bilateral lower limbs and urinary retention 4 days after recovery from dengue fever. She, in addition, had a sensory level up to the level of nipples. Magnetic resonance imaging confirmed the diagnosis of longitudinally extensive transverse myelitis. Besides, the patient had spontaneous subarachnoid hemorrhage (SAH) in the absence of dengue hemorrhagic fever. The patient was started on steroids along with rehabilitation. Our case highlights the extensive involvement of spinal cord in the postinfectious phase of dengue and inclusion of this arboviral disease in the differential diagnoses of myelitis as well as an etiology of SAH.

Modulation of epileptiform EEG discharges in patients with JME.

PURPOSE: To study modulation of epileptiform EEG discharges in patients with JME. METHOD: 50 subjects with JME underwent a sleep deprived EEG recording along with conventional provocative methods and testing with cognitive tasks (CTs). Both categories of tests were evaluated for their effect on occurrence of IEDs. Number of IEDs per unit time was calculated at baseline as well as with each task. Statistical and arbitrary methods were used to assess modulation. By arbitrary method if frequency of IEDs was more than twice that of baseline, it was considered as provocation and if less than half, it was considered as inhibition. To account for spontaneous fluctuation of IEDs, 95% CI was calculated for baseline IEDs in each patient and provocation/inhibition was considered if frequency of IEDs exceeded/remained below limits of CI respectively. RESULTS: There was no significant difference in rates of provocation of IEDs by conventional or CTs. However there was exclusive provocation of IEDs by CTs in 4 patients, 3 of whom were already on AEDs. There was a significant inhibitory effect of CTs as mean baseline discharge frequency was 0.4 ±â€¯1.16 IEDS/min and during CTs was 0.1 ±â€¯0.38 IEDs/min. However when spontaneous fluctuation was accounted for, inhibition was seen in only 22.23% patients by statistical method as compared to 90.91% by arbitrary method. CONCLUSIONS: Inclusion of CTs may assist in provocation of IEDs, thereby increasing yield of routine EEG. Spontaneous fluctuation of IEDs accounts for much observed inhibition by CTs in JME patients.

Sleep abnormalities in juvenile myoclonic epilepsy-A sleep questionnaire and polysomnography based study.

PURPOSE: To evaluate the quality of sleep, its architecture and occurrence of epileptiform discharges with their distribution across various stages of sleep in patients of Juvenile myoclonic epilepsy (JME), both drug naïve as well as those already on treatment. METHODS: 99 patients of JME [36 drug naïve, 63 on antiepileptic drug(s) (AED)], and 30 healthy controls were recruited. Sleep quality and daytime sleepiness were evaluated with Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS), respectively.Polysomnography (PSG) was done to assess the sleep architecture. The EDI (Epileptiform Discharge Index) per stage of sleep was calculated. RESULTS: JME patients had significantly poor quality of sleep by PSQI (p=0.02).PSG revealed reduced sleep efficiency [p<0.001], increased sleep latency [p=0.02], increased%WASO [p<0.001], increased%N1 [p=0.01] and decreased% REM sleep [p=0.002] in the patients compared to controls. Epileptiform discharges were frequent among drug naïve JME patients [drug naïve, 868 vs. 727, treatment group]. EDI was higher in N1 (p=0.001) and N2 (p=0.007) in drug naïve compared to JME patients on treatment. EDI in valproate treatment group was relatively lower to other AEDs. CONCLUSION: JME is associated with poor sleep quality and altered architecture, irrespective of treatment status. REM sleep is significantly decreased in JME patients. Epileptiform discharges are frequent in lighter NREM sleep and EDI is higher in drug naïve patients. Although AEDs disrupt the NREM sleep, their use is associated with arousal stability in lighter stages of sleep and lower EDI, in particular with valproate.

Writer's cramp in spinocerebellar ataxia Type 1.

Dystonia can be encountered in a small subset of patients with spinocerebellar ataxia (SCA), but task specific dystonia is extremely rare. We report a case of a 48-year-old male with confirmed SCA Type 1 (SCA1) with mild progressive cerebellar ataxia and a prominent and disabling Writer's cramp. This case highlights the ever-expanding phenotypic heterogeneity of the SCA's in general and SCA1 in particular.

Idiopathic CD4 lymphocytopenia with sensorimotor polyneuropathy.

A, 21-years-old, male, presented with acute onset, gradually progressive, predominantly distal, symmetrical weakness of both upper and lower limbs with arreflexia. He had impaired sensations in glove and stocking distribution with distal gradient. He was found to have absolute CD4 + cell count of 188 cells/µL, absolute CD8 cell count, 532 cells/µL and CD4: CD8 ratio of 0.35. Electrophysiology revealed reduced to absent CMAP amplitude as well as SNAPs in various nerves of upper and lower limbs, along with normal conduction velocity and normal F wave latencies. Pattern evoked visual potentials were prolonged, on both sides, P100 being 130 ms, on right and 108 ms, on left side. In the follow up of 2 years, he showed spontaneous but gradual clinical improvement but his electrophysiological parameters as well as CD 4+ cells count did not show any significant improvement.

Reversible cerebral and brain stem dysfunction in n: Hexane neuropathy.

A 18-year-old male, screen printer by profession developed sensory motor polyneuropathy, change in his behavior, bilateral 6(th) and 7(th) cranial nerve palsies, down beat nystagmus and cerebellar dysarthria. He had bilaterally prolonged P100 latency; left: 137 ms; right: 144 ms. P 37 was not recordable on either side while N 20 was normal. The inter latency difference between Ipsilateral R2 and Contralateral R2 was 6.15 ms, on the left side and normal on the right side. In the follow-up, there was normalization of the blink reflex study, improvement in P100 latency [left: 114 ms; right: 120 ms.] but worsening of peripheral nerve conductions. The sequential clinical recovery was of the behavioral dysfunction, down beat nystagmus, 6(th) nerve, 7(th) nerve involvement and ataxia, in that order. Sural nerve biopsy showed loss of large diameter myelinated fibers.