Long-term sequelae of herpes simplex virus encephalitis-related white matter injury: correlation of neuropsychological outcome and diffusion tensor imaging.

The pathophysiology of the memory impairment following Herpes Simplex virus encephalitis is not yet established and understood. This study attempts to elucidate the role of white matter injury and its impact on neuropsychological outcome in patients with history of Herpes Simplex virus encephalitis. This is a single-institution prospective study assessing 9 patients and 15 matched controls utilizing a combination of MRI with diffusion tensor imaging and neuropsychological testing. Tract-based spatial statistics analysis was performed and correlated with neuropsychological outcomes. Significantly decreased fractional anisotropy (FA) values were noted in corpus callosum, corona radiata, left posterior thalamic radiation, cingulum, superior longitudinal fasciculus, fornix, inferior longitudinal fasciculus, inferior fronto-occipital fasciculus, and uncinated fasciculus. Impaired facial recognition significantly correlated with reduction in FA of right uncinate fasciculus, right inferior longitudinal fasciculus, and splenium genu of corpus callosum. FA value of left cingulum significantly correlated with logical memory, auditory verbal learning. FA value of fornix correlated with visual recognition; FA value of left uncinate fasciculus with auditory verbal learning and delayed recall. In conclusion, this study demonstrates microstructural abnormalities involving several white matter tracts corresponding to neuropsychological deficits.

Non-motor symptoms in patients with autosomal dominant spinocerebellar ataxia.

OBJECTIVE: The non-motor manifestations of motor predominant disorders have been an area of active interest in recent times. The objective of the study was to determine the prevalence of non-motor symptoms in patients with genetically confirmed spinocerebellar ataxia (SCA). MATERIALS AND METHODS: Forty-one patients of SCA and 48 age-, gender-, and education-matched controls were included. The severity of ataxia was evaluated using the International Cooperative Ataxia Rating Scale (ICARS) and cognitive impairment using a neuropsychological battery. Non-motor features were assessed using standardized scales (HAM-A, HAM-D, Modified Fatigue Severity Scale, RLS questionnaire, ESS, PSQI, WHOQOL, RBDSQ, and BPI). The data were compared with controls and correlated with the severity of ataxia. RESULTS: There were 17 SCA1, 14 SCA2, and 10 SCA3 patients. The mean age of presentation was 35.7 ± 7.9 years for SCA1, 31.1 ± 7.9 years for SCA2, and 30.5 ± 9.5 years for SCA3 patients. The neuropsychological evaluation showed severe impairment of attention, executive functions, visuospatial function, motor speed, response speed, and memory. The severity of ataxia was more for SCA2 patients (ICARS of 39.5 ± 24.4). Ataxia severity was correlated with MMSE, fatigue scale, depression scale, and REM sleep behavior disorder in SCA1 individuals and global cognition, fatigue, anxiety, and depression scales, and RLS in SCA3 patients. All patients reported quality of life as dissatisfied. These patients also had sleep disturbances in the form of RBD, RLS, and EDS. CONCLUSIONS: In addition to the motor symptoms, patients with SCA have several non-motor symptoms that impair the quality of life.

Neuronal Intranuclear Inclusion Disease: A Rare Etiology for Rapidly Progressive Dementia.

INTRODUCTION: Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder pathologically characterized by localized neuronal loss, and presence of eosinophilic intranuclear inclusions in neurons and glial cells. CASE REPORT: A 50-year-old man presented with rapidly progressive dementia, behavioral changes, gait disturbances, and incontinence of 3 months duration. His brain magnetic resonance imaging showed diffuse T2/FLAIR hyperintensity of basal ganglia, thalami, cerebral peduncles, ventral pons, and supratentorial white matter with a frontal predominance. Hyperintensity was noted along the corticosubcortical junction on diffusion-weighted images. NIID was suspected and the patient underwent triple biopsy of the sural nerve with adjacent skin and biceps biopsy. Biopsy revealed ubiquitin-positive intranuclear inclusions surrounding the myofibers, and vascular smooth muscles suggestive of NIID. CONCLUSIONS: NIID is a rare neurodegenerative disorder usually diagnosed postmortem. The rectal and skin biopsy had proved helpful in antemortem diagnosis. We have increased the diagnostic armamentarium by showing the presence of intranuclear inclusions in smooth muscle cells of the muscle. Hence, a high degree of suspicion, magnetic resonance imaging features, with nerve/muscle/skin biopsy can help in diagnosis of NIID.

Therapeutic role of plasma exchange in the management of stiff person syndrome: experience from a tertiary care centre.

INTRODUCTION: The stiff person syndrome (SPS) is a rare and disabling neurological disorder characterized by muscle stiffness, painful spasms and rigidity involving the proximal and axial limb muscles, with an estimated incidence of 1 case per million per year. The first line of treatment for symptomatic management includes gamma-aminobutyric acid (GABA)ergic agonists, benzodiazepines and baclofen. The therapeutic plasma exchange (TPE), alone or as an adjuvant to other forms of immunomodulation, has been used as a therapeutic option, particularly in refractory cases. METHODS: An observational study was performed to review SPS patient symptoms, comorbidities, electromyography (EMG) studies and treatment, identifying autoantibodies, therapeutic plasma exchange (TPE) procedural details and clinical response. MAIN RESULTS: Five patients (4 male and one female) were treated with TPE during the study period as adjuvant therapy. The average age was 47 years (range 34 - 61 years), and anti-glutamic acid decarboxylase 65-kilodalton isoform (anti-GAD65) antibodies were positive in 80 % (4/5) of the patient population. All patients received immunosuppressive drugs along with TPE. Four patients received TPE during the first admission and one received it during the third hospital admission. All patients showed good improvement immediately after TPE, but it was not a sustainable effect. CONCLUSION: TPE may be helpful as adjuvant therapy for SPS patients to provide relief from clinical symptoms.

Psychiatric manifestations of neurosyphilis over past two decades: Findings from a tertiary care neuropsychiatric hospital in south India.

OBJECTIVE: To assess presentation of neurosyphilis with a focus on the psychiatric aspects. METHOD: File review of the cases with a positive cerebrospinal fluid venereal disease research laboratory test between 1999 to 2020. RESULTS: Medical records of 143 neurosyphilis patients were analysed. Hallucinations, delusions, and catatonia were the commonest psychiatric symptoms. Brain atrophy was the commonest neuroimaging finding. The number of neurosyphilis patients and the proportion with delirium or catatonia declined during the second decade (2010-2020). CONCLUSION: Atypical presentation of psychiatric symptoms around the fifth decade, with associated neurological symptoms or brain imaging changes, should prompt evaluation for neurosyphilis.

Amide proton transfer imaging for differentiation of tuberculomas from high-grade gliomas: Preliminary experience.

PURPOSE: Tuberculomas can occasionally masquerade as high-grade gliomas (HGG). Evidence from magnetisation transfer (MT) imaging suggests that there is lower protein content in the tuberculoma microenvironment. Building on the principles of chemical exchange saturation transfer and MT, amide proton transfer (APT) imaging generates tissue contrast as a function of the mobile amide protons in tissue's native peptides and intracellular proteins. This study aimed to further the understanding of tuberculomas using APT and to compare it with HGG. METHOD: Twenty-two patients (n = 8 tuberculoma; n = 14 HGG) were included in the study. APT was a 3D turbo spin-echo Dixon sequence with inbuilt B0 correction. A two-second, 2 µT saturation pulse alternating over transmit channels was applied at ±3.5 ppm around water resonance. The APT-weighted image (APTw) was computed as the MT ratio asymmetry (MTRasym) at 3.5 ppm. Mean MTRasym values in regions of interest (areas = 9 mm2; positioned in component with homogeneous enhancement/least apparent diffusion coefficient) were used for the analysis. RESULTS: MTRasym values of tuberculomas (n = 14; 8 cases) ranged from 1.34% to 3.11% (M = 2.32 ± 0.50). HGG (n = 17;14 cases) showed MTRasym ranging from 2.40% to 5.70% (M = 4.32 ± 0.84). The inter-group difference in MTRasym was statistically significant (p < 0.001). APTw images in tuberculomas were notable for high MTRasym values in the perilesional oedematous-appearing parenchyma (compared to contralateral white matter; p < 0.001). CONCLUSION: Tuberculomas demonstrate lower MTRasym ratios compared to HGG, reflective of a relative paucity of mobile amide protons in the ambient microenvironment. Elevated MTRasym values in perilesional parenchyma in tuberculomas are a unique observation that may be a clue to the inflammatory milieu.

Profile of 26 HIV Seropositive individuals with Cerebral Venous Thrombosis.

BACKGROUND: HIV infection has been found to be prothrombotic condition. However, venous thromboembolism associated with HIV is restricted to peripheral vasculature with few reports of cerebral venous thrombosis (CVT). OBJECTIVE: To examine the clinical manifestations of CVT among HIV seropositive individuals and explore the possible etiological factors. METHODS AND RESULTS: It is a prospective study of 26 (M:F-18:8) patients of CVT associated with HIV seropositive status. Their age and duration of illness was 33.8±6.8years and 11.3±8.5days respectively. Headache and vomiting was the most common symptom followed by seizures. Drowsiness with GCS (Glasgow coma score) ranging from 9-14 was present in two-thirds of the patients. Serum homocysteine was elevated in 70% of patients. Vitamin B12 was low in 12.5% and insufficient levels in 25%. 88.5% of the patients recovered completely to GCS 15/15 in 2-7days during follow up; 11.5% patients expired during the acute state. CONCLUSION: This study represents the largest series of CVT in HIV seropositive individuals. There is increased risk of thrombosis due to elevated homocysteine and low Vitamin B12. They have better sensorium inspite of extensive radiological involvement.

Unique clinical and imaging findings in a first ever documented PCR positive rabies survival patient: A case report.

BACKGROUND: Rabies is fatal encephalitis which is a major public health problem in Asian and African countries. Till date, only 12 cases have been reported who have survived after rabies. CASE REPORT: In this communication we report a patient who is unique as the first documented long term rabies survivor with PCR positivity even after 4½ years of illness. Child sustained dog bite following which he received adequate prophylaxis. Within two weeks, child developed encephalopathy requiring evaluation. Child continues to have persistent myoclonic jerks, seizures, is dependent on all activities with severe neurological deficits. Nested reverse transcriptase PCR (RT-PCR) specific for rabies nucleoprotein gene in CSF and nuchal skin biopsy were positive for rabies viral RNA. The nuchal skin biopsy was also positive for rabies nucleoprotein antigen by fluorescent antibody test (FAT). We describe the clinical evolution and sequential MRI brain changes in this child. CONCLUSIONS: Despite the uniformly dismal prognosis of human rabies, these unusual reports of survival of rabies patients may provide an impetus to explore newer therapeutic strategies for this otherwise fatal disease.