Unmasking bipolarity in recurrent depressive disorder following herpes simplex virus triggered n-methyl-D-aspartate encephalitis.

OBJECTIVE: Herpes simplex virus (HSV) infection triggered n-methyl-D-aspartate (NMDA) encephalitis can lead to varied neuropsychiatric manifestations, including movement disorders and manic symptoms. HSV is known to affect the same brain regions as in secondary mania. METHOD: We present a 35-year-old female diagnosed with recurrent depressive disorder (RDD) who developed NMDA encephalitis triggered by HSV infection. RESULT: HSV-triggered NMDA encephalitis led to a manic switch in a woman with RDD on antidepressants, along with the new onset of dyskinetic movements. CONCLUSION: A neurological insult predisposed our patient to the variable effects of antidepressant drugs.

Intracranial lipoma: an incidental finding and a rare cause of secondary headache.

AIM OF THE STUDY: Expand the differential diagnosis of secondary headache to include rare intracranial tumours, detected incidentally on brain imaging. Intracranial lipomas are rare congenital malformations, and are usually pericallosal asymptomatic midline lesions. However, some cases present with headache and seizures. Symptomatic intracranial lipomas are very rare and often detected incidentally on brain imaging. METHODS: We present a 52-year-old woman referred to our tertiary centre with a history of hypothyroidism presented with headache for 2 years. She had generalised body pains for six months. Her headache was persistent despite being on medications. Physical and neurological examination was unremarkable. Her visual acuity and fundus examination were normal. RESULTS: Her brain imaging revealed a lesion over the corpus callosum and in the interhemispheric fissure with signal attenuation on the fat suppression sequence, features suggestive of curvilinear pericallosal lipoma. Symptomatic treatment with analgesics and anti-inflammatory agents were slightly effective. It is debatable whether tumour removal is required, as the risks of surgical intervention far outweigh the potential benefits. CONCLUSION: Corpus callosal lipoma is a rare and unrecognised cause of secondary headache. It should be suspected in patients with an atypical headache without papilledema and who are unresponsive to analgesics. This may be the only presenting feature of intracranial lipomas rendering it even more difficult to suspect and diagnose, thus emphasising the importance of evaluating secondary headaches. Diagnosis is important because long-term follow-up may be required if patients develop new focal deficits, which may necessitate surgical intervention.

An Illustrative Review of the Pathomechanisms of Symptomatic Developmental Venous Anomalies.

OBJECTIVE: Symptomatic developmental venous anomalies (DVAs) are rare. Here, we illustrate the varied clinicoradiologic profiles of symptomatic DVAs and contemplate the mechanisms that render these (allegedly) benign entities symptomatic supported by a review of literature. METHODS: Institutional databases were searched to identify cases of symptomatic DVAs. Clinical and imaging (angiographic and cross-sectional) data of 9 cases with 11 neurovascular symptoms consequent to inflow/outflow perturbations and mechanical obstruction that manifested because of the strategic topography of underlying DVAs were analyzed. A review of the existing literature on DVAs in agreement with our case series was performed on publications retrieved from the PubMed database. RESULTS: Symptoms secondary to venous hypertension arising from flow-related perturbations were broadly divided into those arising from restricted outflow and increased inflow. Restricted outflow occurred because of collector vein stenosis (n = 2) and collector vein/DVA thrombosis (n = 3), whereas the latter pathomechanism was initiated by arterialized/transitional DVAs (n = 2). A mechanical/obstructive pathomechanism culminating in moderate supratentorial ventriculomegaly was noted in 1 case. One patient was given a diagnosis of hemorrhage associated with a cavernoma. CONCLUSIONS: Awareness and contextualization of potential flow-related perturbations and mechanical insults that render DVAs symptomatic aid in accurate diagnosis, management, and prognostication.

Inflammatory myofibroblastic tumors: A short series with an emphasis on the diagnostic and therapeutic challenges.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are a distinct entity of mesenchymal tumors. We present the challenges in their diagnosis and management. MATERIALS AND METHODS: A retrospective study with detailed clinical, radiological, and histopathological (HPE) features along with management and outcome of 10 biopsy-proven patients with IMT, between 2001 and 2020. RESULTS: The location included intracranial (5), orbital (4), and spinal (1) with M : F = 7 : 3. The mean age of onset was in the third decade. The commonest symptom was headache, while proptosis and blurred vision occurred in orbital IMTs. HPE revealed diffuse infiltration of mixed inflammatory cells over proliferating myofibroblasts. Smooth muscle antigen immunoreactivity was noted in fibroblastic spindle cells of all IMTs. However, we did not find anaplastic lymphoma kinase expression in any of our cases, as this is only found in ~ 50% of all IMTs. Tumor infiltration into adjacent tissue was noted in 4 patients. Surgical excision was limited to orbital IMTs, as most central nervous system (CNS) tumors were not amenable for resection. Steroid administration showed moderate improvement in the IMT-CNS patients but also required additional immunomodulation. Four patients had a median long-term follow-up of 7 years. Two patients had recurrent lesions demonstrated by imaging after 2 years of initial presentation. CONCLUSION: IMTs are rare and ambiguous tumors of unknown etiology that can occur anywhere in the body. Clinical and radiological features may not be specific to determine the diagnosis, but it should be considered as a differential diagnosis. Extensive thorough workup with histopathology along with the help of immunohistochemistry is conducive to better clinical outcomes. Surgical biopsy with extensive and total resection of these tumors along with steroid and radiotherapy may enhance the survival outcomes.

Utility of 3D T1-weighted turbo spin echo black blood sequence for the diagnosis of cerebral venous thrombosis.

PURPOSE: Accurate assessment of dural sinus, deep and cortical venous thrombosis on MR imaging is challenging. The aim of this study is to evaluate the accuracy of 3D-T1 turbo spin echo (T1S), sequences in detecting venous thrombosis and comparing it with susceptibility-weighted imaging (SWI), magnetic resonance venography (MRV) and post contrast T1 magnetization-prepared rapid acquisition gradient echo (T1C). METHODS: A blinded retrospective observational analysis of 71 consecutive patients evaluated for cerebral venous thrombosis (CVT) and 30 control patients was performed. Multimodality reference standard adopted included T1C, SWI with MRV. Sub-analyses in superficial, deep and cortical venous segments were performed in addition to correlation of signal intensity of thrombus with the clinical stage. RESULTS: A total of 2222 segments in 101 complete MRI examinations were evaluated. Sensitivity/specificity/positive predictive value/negative predictive value/accuracy and precision of T1S for detection of cortical vein thrombosis was 0.994/1/1/0.967/0.995/1, 1/0.874/0.949/1/0.963/0.950 for detection of superficial venous sinus thrombosis and 1/1/1/1/1/1 for deep venous thrombosis. The AUC yield for T1S was 0.997 for cortical, 1 for deep and 0.988 for superficial venous segments. CONCLUSION: T1S paralleled the accuracy of conventional sequences in the overall detection of CVT but showed superior accuracy in the detection of cortical venous thrombosis. It makes a fitting addition to the CVT MRI protocol in scenarios demanding negation of gadolinium administration.

Efficacy of pulse intravenous methylprednisolone in epileptic encephalopathy: a randomised controlled trial.

BACKGROUND: High-level evidence for using steroids in epileptic encephalopathy (EE), other than West syndrome (WS), is lacking. This study investigated the efficacy and safety of pulse intravenous methylprednisolone (IVMP) in EE other than WS. METHODS: This is an open-label evaluator-blinded randomised controlled study. Children aged 6 months or more with EE other than WS were included. Eighty children were randomised into intervention and non-intervention groups with 40 in each group. At the first visit (T1) seizure frequency, electroencephalographic (EEG) and Vineland Social Maturity Scale (VSMS) were obtained, and antiseizure medication (ASM) were optimised. After 1 month (T2), subjects were randomised to intervention (ASM+3 months IVMP pulse) or non-intervention group (only ASM) with 40 subjects in each group. They were followed up for 4 months (T3) and assessed. RESULTS: After 4 months of follow-up, 75% of patients receiving IVMP had >50% seizure reduction versus 15.4% in control group (χ2=28.29, p<0.001) (RR 4.88, 95% CI 2.29 to 10.40), median percentage change in seizure frequency (91.41% vs 10%, p<0.001), improvement in EEG (45.5% vs 9.4%, χ2=10.866, p=0.001) and social age domain of VSMS scores (Z=-3.62, p<0.001) compared with baseline. None of the patients in the intervention group had any serious side-effects. DISCUSSION: Three-month pulse IVMP therapy showed significant improvement in seizure frequency, EEG parameters and VSMS scores, with no steroid-related serious adverse effects. It can be considered as a safe and effective add on treatment in children with EE other than WS. TRIAL REGISTRATION NUMBER: CTRI/2019/02/017807.

Utility of multiparametric pre-operative magnetic resonance imaging in differentiation of chordoid meningioma from the other histopathological subtypes of meningioma-a retrospective study.

PURPOSE: To determine the magnetic resonance imaging (MRI) features which could pre-operatively differentiate chordoid meningioma (CM) from other histopathological subtypes of meningioma. METHODS: Retrospective analysis of pre-operative MRI of cases with histopathologically confirmed diagnosis of meningioma during the last 5 years at our institute was done. T1W, T2W, FLAIR sequences, and post-contrast enhancement were evaluated on a qualitative scale. Normalized ADC ratios (nADCR) and normalized fractional anisotropy ratios (nFAR) were derived. The intratumoral susceptibility score (ITSS), presence of sunburst pattern of vasculature, bone changes, tumour-parenchyma interface, and oedema-to-tumour ratio were also determined. RESULTS: A total of 81 lesions were analyzed out of which 15 were CM. CM showed a higher relative contrast enhancement as compared to all other subtypes except for angiomatous and microcystic meningioma. Relative signal intensity on FLAIR could differentiate CM from transitional meningioma. nFAR was found to be significantly higher in fibroblastic meningioma and significantly lower in microcystic meningiomas as compared to CM. Anaplastic meningiomas were remarkable for bone changes and an ill-defined tumour-brain interface in significantly higher proportion of cases as compared to CM. nADCR > 1.5 was found to be an independent predictor of CM with a sensitivity of 84.6%, specificity of 89.8%, positive predictive value of 64.7%, and negative predictive value of 96.4%. CONCLUSION: Routine pre-operative MRI may be able to differentiate CM from other meningioma subtypes and a cut-off value of greater than 1.5 for nADCR could be predictive of > 50% chordoid histology of meningioma with a high sensitivity, specificity, and negative predictive value.

"Trapped labelled spins"-related signal on arterial spin labelling in the assessment of flow-diverted aneurysms: preliminary experience.

PURPOSE: To investigate ASL-MRI features of flow-diverted aneurysms, review their haemodynamic surrogates, and discuss their pertinent clinical implications. METHODS: Retrospective single institutional analysis was performed on the clinical and imaging data of patients who underwent digital subtraction angiography (DSA) and ASL-MRI after endovascular flow diversion for cerebral aneurysms. Pseudo-continuous ASL-MRI was performed with post-label delays of 1525-1800 ms. Intra-aneurysmal "trapped labelled spins" (TLS)-related hypersignal, as seen on cerebral blood flow (CBF)-weighted maps of ASL-MRI, was investigated. Intermodality equivalence with DSA [O'Kelly-Marotta (OKM) grading for occlusion], 3D-TOF-MRA, and 3D spin-echo T1-weighted ("black-blood") images was assessed. RESULTS: Ten cases were included. "TLS" signal was demonstrable in 7/8 (87.5%) of the DSA-visible flow-diverted aneurysms (OKM grade B3, n = 6; OKM grade A3, n = 2). No TLS was seen in both OKM-D (excluded) aneurysms. TLS was not visualised in an OKM-B3 aneurysm with < 3 mm opacifying remnant. 3D-TOF-MRA and ASL-MRI were discordant at 5 instances (45.4%; TOF-MRA false negative, n = 4; false positive, n = 1). Loss of flow void on black-blood images corresponded to the absence of TLS and vice versa in all cases but one. CONCLUSION: "Trapped labelled spins"-related signal on ASL-MRI occurs in patent large aneurysms that have undergone successful endovascular flow diversion. This phenomenon likely represents an interplay of a multitude of haemodynamic factors including decelerated intra-aneurysmal inflow and outflow restriction. Serial intra-saccular TLS signal changes may hold diagnostic value, including contexts where 3D-TOF-MRA interpretation becomes dubious. "Trapped labelled spins"-related signal as a non-invasive proxy marker of aneurysm patency can possibly obviate unnecessary DSA.

Diffusion and perfusion imaging biomarkers of H3 K27M mutation status in diffuse midline gliomas.

PURPOSE: H3K27M-mutant diffuse midline gliomas (M-DMGs) exhibit a clinically aggressive course. We studied diffusion-weighted imaging (DWI) and perfusion (PWI) MRI features of DMG with the hypothesis that DWI-PWI metrics can serve as biomarkers for the prediction of the H3K27M mutation status in DMGs. METHODS: A retrospective review of the institutional database (imaging and histopathology) of patients with DMG (July 2016 to July 2020) was performed. Tumoral apparent diffusion coefficient (ADC) and peritumoral ADC (PT ADC) values and their normalized values (nADC and nPT ADC) were computed. Perfusion data were analyzed with manual arterial input function (AIF) and leakage correction (LC) Boxerman-Weiskoff models. Normalized maximum relative CBV (rCBV) was evaluated. Intergroup analysis of the imaging variables was done between M-DMGs and wild-type (WT-DMGs) groups. RESULTS: Ninety-four cases (M-DMGs-n = 48 (51%) and WT-DMGs-n = 46(49%)) were included. Significantly lower PT ADC (mutant-1.1 ± 0.33, WT-1.23 ± 0.34; P = 0.033) and nPT ADC (mutant-1.64 ± 0.48, WT-1.83 ± 0.54; P = 0.040) were noted in the M-DMGs. The rCBV (mutant-25.17 ± 27.76, WT-13.73 ± 14.83; P = 0.018) and nrCBV (mutant-3.44 ± 2.16, WT-2.39 ± 1.25; P = 0.049) were significantly higher in the M-DMGs group. Among thalamic DMGs, the min ADC, PT ADC, and nADC and nPT ADC were lower in M-DMGs while nrCBV (corrected and uncorrected) was significantly higher. Receiver operator characteristic curve analysis demonstrated that PT ADC (cut-off-1.245), nPT ADC (cut-off-1.853), and nrCBV (cut-off-1.83) were significant independent predictors of H3K27M mutational status in DMGs. CONCLUSION: DWI and PWI features hold value in preoperative prediction of H3K27M-mutation status in DMGs.

Exploring immunoreactivity of TTF-1 and AVP in hypothalamic hamartoma.

INTRODUCTION: Hypothalamic hamartoma (HH) is a rare developmental disorder presenting with gelastic seizures or precocious puberty attributed to gonadotrophin-releasing hormone expression by the hamartoma. The histogenesis of HH is uncertain, and diagnosis of HH is difficult in small biopsies due to its close resemblance to normal hypothalamic nuclei. TTF-1 and arginine vasopressin (AVP) are associated with gonadotropin-releasing hormone release. MATERIALS AND METHODS: In this study, we explored the expression pattern of TTF-1 and AVP in HH and its utility, if any, in diagnosis. We reviewed the clinical, radiologic, and histopathological features of 23 HH diagnosed over the past decade at our Institute. RESULTS: The age at presentation ranged from 11 months to 34 years with gelastic seizures (82.6%), precocious puberty (17.4%), and developmental delay (8.7%) as presenting symptoms. On imaging, all the lesions (n = 9) involved the posterior and tuberal group of hypothalamic nuclei, while 5 cases involved the anterior hypothalamus. Anatomically, the lesions involved mammillary body, arcuate and periventricular nuclei. On histopathology, 52% cases revealed nodular arrangement of small neurocytic cells separated by glial stroma. TTF-1 and AVP immunoreactivity was absent in all the cases, whereas in normal hypothalamus, AVP was expressed in periventricular nuclei. CONCLUSION: Our results suggest that immunoexpression of TTF-1 is absent in HH, particularly in those arising from the posterior hypothalamus, and this can be used in small biopsies to distinguish from a normal hypothalamus as well as from posterior pituitary tumors.

Novel CSF1R variant in adult-onset leukoencephalopathy masquerading as frontotemporal dementia: a follow-up study.

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a rare white matter degenerative disease manifesting as progressive cognitive decline, pyramidal, and extrapyramidal features resulting from mutations in the colony-stimulating factor-1 receptor (CSF1R) gene. We describe a sporadic case of a young man who developed five months history of progressive cognitive decline with predominant neuropsychiatric symptoms, suggestive of frontotemporal dementia. Brain magnetic resonance imaging (MRI) showed bilateral frontotemporal atrophy, high signal intensities in frontal and high parietal deep white matter with persistent diffusion restriction on follow-up imaging. Genetics showed a novel heterozygous mutation in CSF1R gene confirming the diagnosis of ALSP. Being a rare disease, and given its particular adult-onset presentation especially presenile cognitive impairment, it can pose a unique diagnostic challenge. The study highlights the importance of recognizing the disease early and broadens the clinical, genetic, and imaging spectrum of CSF1R gene mutation.

EEG-based P300 in mesial temporal lobe epilepsy and its correlation with cognitive functions: A case-control study.

OBJECTIVE: P300 is an event-related potential, being explored as an objective tool to assess cognition. This study aimed to investigate the characteristics of auditory and visual P300 in patients with TLE having unilateral HS using electroencephalography (EEG) and to study its correlation with cognition. METHODS: This is a cross-sectional case-control study, where P300 characteristics in thirty patients with unilateral hippocampal sclerosis with refractory epilepsy were compared with fifteen age-, gender-, and years of education-matched healthy controls (M: F-10:5, mean age-28 ±â€¯4.76 years). Among patients, 15 belonged to the right HS group (M: F-9:6, age at onset-12.92 ±â€¯10.22 years, duration of epilepsy-16.67 ±â€¯9.38 years) and 15 to the left HS group (M: F-8:7, age at onset-10.62 ±â€¯7.18 years, duration of epilepsy-15.53 ±â€¯10.14 years). All subjects underwent EEG-based auditory and visual oddball tasks and cognitive assessment. The P300 latencies (in milliseconds) as well as amplitudes (in microvolts) were predicted in EEG and were correlated with cognitive scores. Source localization of P300 was performed with the CLARA algorithm. RESULTS: The auditory P300 latencies in controls, right HS, and left HS were 323.93 ±â€¯40.28, 351.06 ±â€¯47.23, and 328.80 ±â€¯36.03, respectively (p = 0.18) and its amplitudes were 2.3040 ±â€¯1.46, 2.77 ±â€¯1.19, and 2.68 ±â€¯1.78, respectively (p = 0.48). Visual P300 latencies in controls, right HS, and left HS were 365.87 ±â€¯47.37, 359.67 ±â€¯64.45, and 376.00 ±â€¯60.06, respectively (p = 0.51) and its amplitudes were 3.93 ±â€¯2.28, 2.09 ±â€¯1.45, and 3.56 ±â€¯1.74, respectively (p = 0.014). Further, when compared to the control group the cognitive scores were lower in the patient group (p < 0.05). SIGNIFICANCE: In comparison to the controls, patients with right HS recorded lesser amplitude on visual P300 and lower scores on cognitive tests. P300 and cognitive parameters exhibited varied relationship. P300 could be a complementary objective tool to assess cognition in patients with TLE.

Posterior Quadrant Disconnection for Childhood Onset Sub-Hemispheric Posterior Head Region Epilepsy: Indications in an Indian Cohort and Outcome.

BACKGROUND: Posterior quadrant disconnection (PQD) is an under-utilized surgical technique in the management of refractory epilepsy. There is a dearth of data pertinent to post-PQD seizure outcomes. METHODS: This retrospective study analyzed patients with drug-resistant childhood-onset epilepsy who underwent PQD at our center from 2009 to 2018. The clinical, imaging, and electrophysiological data were reviewed. The seizure outcome was noted from the latest follow-up in all patients. RESULTS: Fifteen patients underwent PQD, with a mean age at onset of epilepsy of 3.3 ± 4.6 years. All patients had seizure onset in childhood with focal onset of seizures, and in addition, 5 had multiple seizure types. All cases underwent presurgical workup with MRI, video-EEG, psychometry, while PET/MEG was done if required. Engel Ia and ILAE I outcomes were considered to be favorable. The histology of the specimen showed 9 patients (60%) had gliosis, 4 (26.7%) had focal cortical dysplasia (FCD), while 1 patient had nodular heterotopia and another had polymicrogyria-pachygyria complex. Postoperative follow-up was available in 14 cases. One patient was lost to follow-up. Mean follow-up duration for the cohort was 45 + 24 months. At last, follow-up (n = 14), 66.7% (10 cases) had favorable outcome (Engel Ia). At the end of 1-year follow-up, up to 73% (n = 11) of the patients were seizure-free. Four patients developed transient hemiparesis after surgery which improved completely by 3-6 months. CONCLUSIONS: Gliosis was more common etiology requiring PQD in our series than Western series, where FCD was more common. PQD is a safe and effective surgical modality in childhood-onset epilepsy with posterior head region epileptogenic focus.

Clinical Characteristics and Outcomes of Critically Ill Neurological Patients with COVID-19 Infection in Neuro-intensive Care Unit: A Retrospective Study.

BACKGROUND: There are insufficient data about clinical outcomes in critically ill neurological patients with concomitant coronavirus disease (COVID-19). This study describes the clinical characteristics, predictors of mortality, and clinical outcomes in COVID-19-positive neurological patients managed in a dedicated COVID-19 neurointensive care unit (CNICU). METHODS: This single-center, retrospective cohort study was conducted in critically ill neurological and neurosurgical patients with concomitant COVID-19 infection admitted to the CNICU at the National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, from July to November 2020. Patients' demographic, clinical, laboratory, imaging, treatment, and outcome data were retrieved from the manual and electronic medical records. Predictors of mortality and neurological outcome were identified using logistic regression. RESULTS: During the study period, 50 COVID-19-positive neurological patients were admitted to the CNICU. Six patients were excluded from the analysis as they were managed in the CNICU for <24 hours. A poor outcome, defined as death or motor Glasgow Coma Scale <5 at hospital discharge, was observed in 34 of 44 patients (77.27%) with inhospital mortality in 26 of 44 patients (59%). Worst modified sequential organ failure assessment (MSOFA) score, lactate dehydrogenase maximum levels (LDHmax), and lymphocyte count were predictors of inhospital mortality with an odds ratio (OR) of 1.88, 1.01, and 0.87, respectively, whereas worst MSOFA and LDHmax levels were predictors for poor neurological outcome with OR of 1.99 and 1.01, respectively. CONCLUSIONS: Mortality is high in neurological patients with concomitant COVID-19 infection. Elevated inflammatory markers of COVID-19 suggest the role of systemic inflammation on clinical outcomes. Predictors of mortality and poor outcome were higher MSOFA score and elevated LDH levels. Additionally, lymphopenia was associated with mortality. HOW TO CITE THIS ARTICLE: Surve RM, Mishra RK, Malla SR, Kamath S, Chakrabarti DR, Kulanthaivelu K, et al. Clinical Characteristics and Outcomes of Critically Ill Neurological Patients with COVID-19 Infection in Neuro-intensive Care Unit: A Retrospective Study. Indian J Crit Care Med 2021;25(10):1126-1132.

Cerebral small vessel disease with hemorrhagic stroke related to COL4A1 mutation: A case report.

Stroke is a major cause of mortality and morbidity with a wide variety of etiological risk factors. Cerebral small vessel disease (SVD) is an important cause of stroke in the young with several hereditary disorders affecting these small blood vessels. Mutations in the COL4A1 gene (COL4A1) have been shown to be associated with a broad range of disorders including hemorrhagic stroke, myopathy, glaucoma and others. We report a rare case of stroke in an intellectually disabled 18-year-old girl with radiological evidence of basal ganglia microbleeds, periventricular white matter signal changes and porencephalic cyst. Ophthalmic examination revealed bilateral microcornea and Axenfeld-Rieger anomaly. At autopsy there were hemorrhagic lesions at multiple sites within the brain. Histology revealed thickened small-caliber vessels which demonstrated disruption and fragmentation of the basement membrane by collagen type IV alpha 1 immunohistochemistry and by electron microscopy. A missense COL4A1 mutation involving glycine residue was detected in the patient. The present case illustrates the clinicopathological spectrum of COL4A1-related cerebral SVD presenting as hemorrhagic stroke in the young with porencephaly, intellectual disability, and Axenfield-Rieger anomaly and thus adds to the clinical heterogeneity of this genetic disorder.

Cough elixir overdose causing toxic leuco-encephalopathy and serotonin syndrome.

Acute toxic leukoencephalopathy and serotonin syndrome are rare neurological complications associated with various drugs and toxins, some of which overlap. However, the co-occurrence of these conditions is poorly documented. We present the case of a 14-year-old boy who suddenly developed altered consciousness and autonomic dysfunction after consuming excessive quantities of cough remedies containing dextromethorphan, chlorphenamine, dichlorobenzyl alcohol, and amylmetacreson. Magnetic resonance imaging of the brain revealed distinct white matter lesions. With supportive care, the patient rapidly improved, and the magnetic resonance imaging abnormalities disappeared. The swift resolution, typical magnetic resonance imaging findings, and a history of exposure to drugs affecting the central nervous system's serotonergic system suggested concurrent acute toxic leukoencephalopathy and serotonin syndrome. The components of cough medications can be hazardous in overdose due to their potential to enhance serotonin toxicity and cause direct or indirect central nervous system white matter damage. Early recognition and appropriate treatment are essential for recovery.

Hippocampal volume and recovery profile following propofol sedation in children undergoing magnetic resonance imaging: An exploratory study.

Background: Reduction in the hippocampal volume may contribute to agitated and delayed emergence after anesthesia in epilepsy surgery. We hypothesized that hippocampal volume and the duration of various recovery parameters after a short duration of sedation may be correlated. The primary objective was to evaluate the correlation between hippocampal volumes with time to recovery after the stoppage of propofol infusion. Methods: After obtaining Institute Ethical Clearance, we included all children of the age group 5-17 years, who needed sedation for brain magnetic resonance imaging (MRI) for at least 20-60 minutes for the evaluation of epilepsy. The hippocampal volume was estimated bilaterally in the pre-contrast volumetric magnetization-prepared rapid gradient-echo (MPRAGE) brain imaging by the radiologist using statistical parametric mapping. The correlation of hippocampal volume with recovery and time to discharge (assessed by the modified Aldrete score (MAS)) was obtained using Spearman's correlation coefficient (rho). A rho > ± 0.5 was considered a good correlation between the variables. Results: Data on a total of 18 children (10 males and 8 females) who required sedation for an MRI were studied over a period of six months. The correlation coefficients of right and left corrected hippocampal volumes with time to spontaneous eye opening were -0.052 and -0.195, respectively. The correlation coefficients of right and left corrected hippocampal volumes with time to respond to oral commands were -0.017 and -0.219, respectively. Conclusion: There was a weak negative correlation between hippocampal volumes and recovery parameters after a short duration of sedation with propofol in children.

Transcranial ultrasonographic evaluation of effect of ventriculoperitoneal shunt on intracranial dynamics: A prospective observational study.

BACKGROUND: Transcranial sonographic (TCS) evaluation of optic nerve sheath diameter (ONSD), third ventricular diameter (TVD) and mean flow velocities (Vm) and pulsatility index (PI) of middle cerebral artery (MCA) can provide important insights to the change in intracranial dynamics following ventriculo-peritoneal (VP) shunt surgery. The primary objective of this study was to observe changes in ONSD values following VP shunt at 12 h, compared to pre-VP shunt values. METHODS: After obtaining ethical approval, patients admitted with a diagnosis of hydrocephalus posted for a VP shunt surgery were prospectively enrolled. TCS evaluation was done before induction of anesthesia and 12-hour post-VP shunt surgery. We recorded the values of ONSD, TVD and Vm and PI MCA at both time points. RESULTS: Thirty-four patients (19 male) were evaluated for ONSD and for the improvement of symptoms. Transtemporal window could not be obtained in six patients. At 12 h following VP shunt, bilateral median ONSD values reduced significantly from their pre-VP shunt values [right ONSD- 0.62 (0.59-0.64) to 0.53 (0.5-0.54) mm (p < 0.001); left ONSD- 0.62 (0.59-0.63) to 0.53 (0.5-0.54) mm (p < 0.001)]. Similarly, the median TVD at 12 h post-VP shunt reduced significantly from its pre-VP shunt measurements [0.97 (0.85-1.09) to 0.74 (0.7-0.84) cm]. PI MCA values reduced significantly, while Vm MCA values increased significantly from the pre-VP shunt values. CONCLUSION: VP shunt reduced the ONSD, TVD, PI MCA and increased the Vm MCA after shunt surgery as early as 12hrs.

Primary Microgliopathy Presenting as Degenerative Dementias: A Case Series of Novel Gene Mutations from India.

Introduction: Microglia exert a crucial role in homeostasis of white matter integrity, and several studies highlight the role of microglial dysfunctions in neurodegeneration. Primary microgliopathy is a disorder where the pathogenic abnormality of the microglia causes white matter disorder and leads to a neuropsychiatric disease. Triggering receptor expressed on myeloid cells (TREM2), TYRO protein tyrosine kinase binding protein (TYROBP) and colony-stimulating factor 1 receptor (CSF1R) are genes implicated in primary microgliopathy. The clinical manifestations of primary microgliopathy are myriad ranging from neuropsychiatric syndrome, motor disability, gait dysfunction, ataxia, pure dementia, frontotemporal dementia (FTD), Alzheimer's dementia (AD), and so on. It becomes imperative to establish the diagnosis of microgliopathy masquerading as degenerative dementia, especially with promising therapies on horizon for the same. We aimed to describe a case series of subjects with dementia harbouring novel genes of primary microgliopathy, along with their clinical, neuropsychological, cognitive profile and radiological patterns. Methods: The prospective study was conducted in a university referral hospital in South India, as a part of an ongoing clinico-genetic research on dementia subjects, and was approved by the Institutional Ethics Committee. All patients underwent detailed assessment including sociodemographic profile, clinical and cognitive assessment, pedigree analysis and comprehensive neurological examination. Subjects consenting for blood sampling underwent genetic testing by whole-exome sequencing (WES). Results: A total of 100 patients with dementia underwent genetic analysis using WES and three pathogenic variants, one each of TREM2, TYROBP, and CSF1R and two variants of uncertain significance in CSF1R were identified as cause of primary microgliopathy. TREM2 and TYROBP presented as frontotemporal syndrome whereas CSF1R presented as frontotemporal syndrome and as AD. Conclusion: WES has widened the spectrum of underlying neuropathology of degenerative dementias, and diagnosing primary microglial dysfunction with emerging therapeutic options is of paramount importance. The cases of primary microgliopathy due to novel mutations in TREM2, TYROBP, and CSF1R with the phenotype of degenerative dementia are being first time reported from Indian cohort. Our study enriches the spectrum of genetic variants implicated in degenerative dementia and provides the basis for exploring complex molecular mechanisms like microglial dysfunction, as underlying cause for neurodegeneration.