Psychosocial Basis of Human Sufferings and Poverty in Patients with Neurological and Psychiatric Disorders.

Neurological disorders and psychiatric ailments often lead to cognitive disabilities and low attainment of education, pivoting misconceptions, myths, and misbeliefs. Poverty and low educational attainment are intriguingly associated with poor awareness and perception of these diseases that add to the suffering. Poverty goes parallel with a low level of education and is intricately associated with neuropsychiatric ailments, which have the potential to spread transgenerationally. Robust education policies, proper government rules and regulations against the spread of disease-related myths and misconceptions, uplifting medical education in its true sense, voices against consanguinity, and programs to raise scientific perception about diseases can help to throw light at the end of this dark tunnel. In this article, the authors intend to 1) decipher the potential psychosocial basis of human suffering and poverty in patients with neurological and psychiatric disorders, and 2) discuss the apropos way-outs that would potentially mitigate suffering, and alleviate the economic burden and cognitive disabilities of families with neuropsychiatric diseases.

Spectrum of Anti-NMDA Receptor Antibody Encephalitis: Clinical Profile, Management and Outcomes.

BACKGROUND: Anti-N-methyl D-aspartate receptor (anti NMDAR) antibody encephalitis is an immune-mediated entity characterised by a constellation of neuro-psychiatric symptoms. OBJECTIVE: To describe clinical profile and treatment outcomes of patients with anti NMDAR antibody encephalitis. SETTINGS AND DESIGN: Subjects were selected by screening for all patients satisfying Graus et al.'s criteria for probable anti NMDAR antibody encephalitis, admitted in neurology department of a tertiary care centre in Eastern India. MATERIALS AND METHODS: A prospective, longitudinal study was conducted by identifying 25 patients with anti NMDAR antibodies in CSF and or serum, between September 2018 to February 2020. STATISTICAL ANALYSIS: Chi square test was used to compare variables. RESULTS: Out of 98 patients screened, 25 subjects (14 females: 11 male) were positive for anti NMDAR autoantibodies, with a mean age of 17 years. 13 subjects belonged to paediatric age group. Most common presenting feature was memory/learning deficit (88%) followed by behavioural abnormalities (84%) and seizures (68%). 11 patients (44%) patients needed escalation to second line therapy, rituximab. Seven (28%) and twelve (48%) patients underwent complete (mRS 0-1) and partial recovery (mRS 2-3) respectively, while 4 (16%) became disabled (mRS 4-5). Mortality was 8%. Paediatric population had a better outcome in terms of disability (p = 0.043). CONCLUSION: Anti NMDAR-Ab encephalitis is the most common cause of antibody positive autoimmune encephalitis worldwide. There are important clinical markers and investigational profiles which carry prognostic significance.

Status epilepticus and bilateral middle cerebral artery infarction: A rare presentation after viper bite.

Owing to the antihemostatic property of viper venom, hemorrhagic complications including intracerebral hemorrhage are the most commonly encountered after viper bite. Ischemic strokes have been rarely reported after viper envenomation, and its occurrence has been attributed to multiple mechanisms. Postsnakebite seizures are known to occur after neurotoxic bite. Here, we report the case of a viper bite victim who developed status epilepticus within 3 h after viper bite. He had only mild signs of local envenomation, and prolonged whole blood clotting time was the only manifestation of systemic envenomation. Subsequently, he was found to have developed right hemiparesis and global aphasia. Brain imaging revealed large infarcts in bilateral middle cerebral artery (MCA) territories. We report this as a unique case of viper bite which presented to the emergency room with status epilepticus. Moreover, bilateral MCA infarct, as was found in this case, is genuinely rare in scientific literature. Finally, the absence of overt features of envenomation makes this case stand out from other similar reported occurrences.

Résumé En raison de la propriété antihémostatique du venin de vipère, les complications hémorragiques, y compris l'hémorragie intracérébrale, sont les plus courantes. rencontré après morsure de vipère. Des AVC ischémiques ont rarement été signalés après une envenimation par vipère, et son apparition a été attribuée à mécanismes multiples Les crises d'épilepsie postnakebite se produisent après une piqûre neurotoxique. Ici, nous rapportons le cas d'une victime de morsure de vipère qui état de mal épileptique dans les trois heures suivant la piqûre des vipères. Il ne présentait que de légers signes d'envenimation locale et un temps de coagulation du sang total prolongé était la seule manifestation de l'envenimation systémique. Par la suite, il s'est avéré avoir développé une hémiparésie droite et une aphasie globale. L'imagerie cérébrale a révélé de grands infarctus dans les territoires bilatéraux de l'artère cérébrale moyenne (ACM). Nous rapportons cela comme un cas unique de morsure de vipère présenté à la salle d'urgence avec le statut épileptique. De plus, l'infarctus bilatéral à MCA, comme on l'a constaté dans ce cas, est vraiment rare dans littérature scientifique. Enfin, l'absence de caractéristiques évidentes d'envenimation fait que ce cas se distingue des autres cas similaires.

Young-onset sporadic Creutzfeldt-Jakob disease with atypical phenotypic features: a case report.

BACKGROUND: Sporadic Creutzfeldt-Jakob disease, with a mean survival of 6 months, is duly considered among the most fatal neurological disorders. Rapidly progressive dementia with multi-axial involvement of the nervous system is the known presentation. Although, the peak age at onset is between sixth and eighth decades, cases of young-onset sporadic Creutzfeldt-Jakob disease have also been reported in the literature. Interestingly, these young-onset cases were reported to have some features distinct from their older age group counterparts, such as slower progression as well as longer duration of illness, dominance of psychiatric manifestations at the onset, and relatively less prevalence of radiological and electroencephalographic abnormalities. CASE PRESENTATION: We describe here the case of a 42-year-old Asian woman from India who presented with cerebellar ataxia, pyramidal and extrapyramidal involvement, followed by rapidly progressive dementia along with myoclonus, all within a span of 1 month. Probable infective, metabolic, autoimmune, and paraneoplastic etiologies were ruled out. Magnetic resonance imaging of her brain revealed bilateral caudate nucleus hyperintensity in T2/fluid-attenuated inversion recovery sequence. Diffusion-weighted imaging revealed bilateral caudate and putaminal diffusion restriction plus ribbon pattern in bilateral parieto-occipital and insular cortex. Serial electroencephalography revealed diffuse slowing of background activity along with triphasic waves in short periodic interval. Cerebrospinal fluid was tested positive for 14-3-3 protein. Based on these findings, a diagnosis of sporadic Creutzfeldt-Jakob disease was made. CONCLUSION: Our patient represents an atypical clinical situation as she is much younger than the usual presentation of Creutzfeldt-Jakob disease and it progressed far too rapidly. Cognitive decline came late in the temporal sequence of clinical events; rather, the onset was dominated by features consistent with cerebellar ataxia and basal ganglia involvement. The presence of magnetic resonance imaging abnormality and electroencephalography changes are other rare findings in young-onset sporadic Creutzfeldt-Jakob disease.

Chorea-ballism as a dominant clinical manifestation in heteroplasmic mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome with A3251G mutation in mitochondrial genome: a case report.

BACKGROUND: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes, the most common maternally inherited mitochondrial disease, can present with a wide range of neurological manifestations including both central and peripheral nervous system involvement. The most frequent genetic mutation reported in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome is A3243G in MT-TL1 gene. Stroke-like episodes, dementia, epilepsy, lactic acidemia, myopathy, recurrent headaches, hearing impairment, diabetes, and short stature constitute the known presentations in this syndrome. Among the abnormal involuntary movements in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome, myoclonus is the commonest. Other movement disorders, including chorea, are rarely reported in this disorder. CASE PRESENTATION: A 14-year-old South Asian boy from rural Bengal (India), born of a second degree consanguineous marriage, with normal birth and development history, presented with abnormal brief jerky movements involving his trunk and limbs, with recurrent falls for 10 months. We present here a case of heteroplasmic mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome with A3251G mutation, in which the clinical picture was dominated by a host of involuntary abnormal movements including chorea-ballism, myoclonus, and oromandibular dystonia in a backdrop of cognitive decline, seizure, and stroke-like episode. A final diagnosis was established by muscle biopsy and genetic study. Haloperidol was administered to control the involuntary movements along with introduction of co-enzyme Q, besides symptomatic management for his focal seizures. Six months into follow-up his seizures and abnormal movements were controlled significantly with slight improvement of cognitive abilities. CONCLUSION: The dominance of hyperkinetic movements in the clinical scenario and the finding of a point mutation A3251G in MT-TL1 gene make this a rare presentation.

Hemispheric venous dysgenesis in a woman presenting with seizures, weakness and parkinsonism.

This report describes an unusual case of a woman who developed progressive hemiparesis, seizures and hemiparkinsonism associated with MRI and angiographic evidence of chronic venous hypertension in the contralateral cerebrum and cerebellum. In the absence of inflammatory or veno-occlusive disorders, the patient's clinical and neuroradiological findings point to a developmental disorder, such as a hemispheric venous dysgenesis, as the underlying lesion.