Posterior Reversible Leucoencephalopathy Syndrome: Case Series, Comments, and Diagnostic Dilemma.

PURPOSE OF REVIEW: To report a series of patients with clinical and radiological features suggestive of posterior reversible encephalopathy syndrome (PRES) related to diverse etiologies emphasizing its pathophysiological basis. RECENT FINDINGS: Posterior reversible encephalopathy syndrome (PRES) may present with a broad range of clinical symptoms from headache and visual disturbances to seizure and altered mentation. Typical imaging findings include posterior-circulation predominant vasogenic edema. Although there are many well-documented diseases associated with PRES, the exact pathophysiologic mechanism has yet to be fully elucidated. Generally accepted theories revolve around disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury induced by ischemia from a vasoconstrictive response to rising blood pressure or toxins/cytokines. While clinical and radiographic reversibility is common, long-standing morbidity and mortality can occur in severe forms. In patients with malignant forms of PRES, aggressive care has markedly reduced mortality and improved functional outcomes. Various factors that have been associated with poor outcome include altered sensorium, hypertensive etiology, hyperglycemia, longer time to control the causative factor, elevated C reactive protein, coagulopathy, extensive cerebral edema, and hemorrhage on imaging. Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. Diagnosis of PRES in some circumstances can be challenging and structural imaging may not be sufficient to distinguish it from other differential diagnostic considerations like ADEM. Advanced imaging techniques, such as MR spectroscopy or positron emission tomography (PET) can provide additional information to determine the diagnosis. Such techniques are more useful to understand the underlying vasculopathic changes in PRES and may answer some of the unresolved controversies in pathophysiology of this complex disease. Eight patients with PRES resulting from different etiologies varying from pre-eclampsia/eclampsia, post-partum headache with seizures, neuropsychiatric systemic lupus erythematosus, snake bite, Dengue fever with encephalopathy, alcoholic liver cirrhosis with hepatic encephalopathy, and lastly reversible cerebral vasoconstriction syndrome (RCVS). Additionally, a diagnostic dilemma between PRES and acute disseminated encephalomyelitis (ADEM) was notable in one patient. Some of these patients did not have or only very transiently had arterial hypertension. PRES may underlie the clinical conundrum of headache, confusion, altered sensorium, seizures, and visual impairment. PRES need not necessarily be always associated with high blood pressure. Imaging findings may also be variable. Both clinicians and radiologists need to familiarize themselves with such variabilities.

Bickerstaff encephalitis: An uncommon sequalae of a bacterial infection.

Bickerstaff brainstem encephalitis (BBE) is a rare neurological disorder characterised by the presence of the triad of ophthalmoplegia, ataxia and altered consciousness. It is thought to be associated to an autoimmune condition triggered by an antecedent infection. Scrub typhus is a zoonotic disease caused by Orientia tsutsugamushi, and it is associated with myriads of neurological complications. We hereby present an unusual case of BBE in a young lady, who was probably a sequalae of scrub typhus infection. Bickerstaff brainstem encephalitis in association with scrub typhus has not been reported till date.

Neurological Manifestations of Scrub Typhus.

PURPOSE OF REVIEW: The occurrence of cases of scrub typhus is on the rise in South Asian and Southeast Asian countries. The present review discusses neurological complications following scrub typhus to appraise clinicians practicing in endemic regions about considering this treatable disease in the differential diagnosis of acute febrile illnesses, especially when accompanied with clinical neurological features. RECENT FINDINGS: While the association of aseptic meningitis, encephalitis, and meningoencephalitis with scrub typhus is well known, more recently described neurological syndromes associated with scrub typhus include acute disseminated encephalomyelitis, various cranial nerve palsies, cerebellitis, cerebrovascular diseases including cerebral venous sinus thrombosis, transverse myelitis, longitudinally extensive transverse myelitis, Guillain-Barré syndrome, opsoclonus-myoclonus syndrome, parkinsonism, and many more. Early diagnosis is key to successful treatment. While diagnostic confirmation is generally made by the detection of IgM antibody by either ELISA or indirect fluorescent antibody tests, conventional PCR using 56 kDa gene (cPCR) and loop-mediated isothermal amplification assay (LAMP assay), as well as a newly introduced metagenomic next-generation sequencing (mNGS), are currently available for detection of Orientia tsutsugamushi infection in clinically suspected cases. Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi. The cutaneous hallmark of the disease is the "eschar." Scrub typhus results in multisystem involvement. Neurological compromise is present in about 20% of scrub typhus patients and affects both the central nervous system and the peripheral nervous system. The postulated underlying mechanisms include direct invasion of the organism, a vasculitis-like process, or an immune-mediated injury. Diagnosis of scrub typhus is confirmed by detection of O. tsutsugamushi IgM antibody in serum. Awareness among clinicians regarding the varied presentations of this disease is very important in order to reduce morbidity and mortality. Co-infection with dengue and/or chickungunya viruses may occur in endemic regions. The history of an acute febrile illness preceding the neurological illness is crucial. A very careful search for the eschar is essential; however, the absence of the skin lesion cannot exclude the diagnosis of scrub typhus. Neurological manifestations mostly respond to doxycycline therapy.

Post-infectious cerebellar ataxia following COVID-19 in a patient with epilepsy.

Background: Various neurological manifestations have been described in relation to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and coronavirus disease 2019 (COVID-19). However, the development of cerebellar ataxia after recovery from COVID-19 is rare. We present a case of cerebellar ataxia 3 weeks after recovery from COVID-19. Case Presentation: A 70-year-old male patient from an urban area of India presented with ataxia. He was hypertensive and had been receiving treatment for post-traumatic epilepsy for the previous 3 years. He had previously had laboratory-confirmed COVID-19 infection with mild symptoms that resolved within 2 weeks. However, 3 weeks after symptom improvement, he developed severe pan-cerebellar ataxia. Investigations were suggestive of post-infectious cerebellar ataxia. Other causes of ataxia were excluded. He responded well to pulse methylprednisolone therapy and was discharged with mild tremor and ataxia. Conclusion: Post-infectious cerebellar ataxia is an unusual presentation after COVID-19. The clinician should be aware of such complications following COVID-19 infection as early diagnosis and proper management leads to better outcomes in many patients.

Seizure after recovery from Covid-19.

There are various neurological presentations of covid-19 but here we report a 27-year-old female developed first episode of seizure, 14 days after documented SARS-CoV-2 infection. She had generalized tonic-clonic seizure with multiple injuries. She had no previous or family history of seizure but she had generalized anxiety disorder and was doing well on selective serotonin reuptake inhibitor (SSRI). Electroencephalogram (EEG) showed theta waves background (likely post-ictal discharges). Magnetic resonance Imagining (MRI) Brain showed features of focal cerebritis on both the frontal lobes. She was started with anti-epileptic drug. After 6 weeks, repeat MRI Brain and EEG were completely normal.

Neuropsychiatric lupus with posterior reversible encephalopathy syndrome: a rare presentation.

Systemic lupus erythematosus presenting with neuropsychiatric symptoms (NPSLE) along with posterior reversible encephalopathy syndrome (PRES) is rare. A young woman of 29 years presented with various neuropsychiatric symptoms along with low-grade fever, occasional headache, skin rash, arthralgias and gradually became non-ambulatory over last 6 months. After admission, she had an episode of generalised tonic-clonic seizure, followed by drowsiness. She was normotensive. Investigations revealed no evidence of any underlying infection, normal renal functions and electrolytes; but other parameters were supportive to a diagnosis of NPSLE. MRI brain showed vasogenic oedema characterised by symmetrical hyperintensities over posterior brain regions in T2 and fluid attenuated inversion recovery images with no restricted diffusion in diffusion weighted image suggestive of PRES. A diagnosis of NPSLE presenting with PRES, particularly in the absence of hypertension and abnormal renal functions was made, which is a rare presentation. She responded well to immunomodulatory therapy with methylprednisolone and monthly cyclophosphamide.

Long-Term Morbidity and Functional Outcome of Japanese Encephalitis in Children: A Prospective Cohort Study.

OBJECTIVE: To describe the long term morbidity and functional outcome of Japanese encephalitis in children. METHODS: Laboratory-confirmed Japanese encephalitis cases were enrolled in the study from January, 2016 to September, 2017 and surviving cases were prospectively followed up for 2.5 years to document various morbidities. Outcome was functionally graded at discharge and during follow-up using Liverpool outcome score. RESULTS: Out of 56 children enrolled, 10 (17.9%) died during hospital stay; severe sequelae was observed in 17 (30.4%) at discharge. At the end of study, among 37 children under follow-up, 23 (62.2%) recovered fully, 2 (5.4%) showed minor sequelae, 3 (8.1%) had moderate sequelae, and 9 (24.3%) were left with severe sequelae. Common long term morbidities were abnormal behavior (n=10, 27%), post encephalitic epilepsy (n=8, 21.6%), poor scholastic performance (n=8, 21.6%) and residual motor deficit (n=7, 18.9%). Improvement of morbidities was noted mostly within initial 1 year of follow-up. CONCLUSION: More than half of the Japanese encephalitis survivors recovered fully, most within the first year after discharge.

Scrub typhus with cerebral venous sinus thrombosis: a rare presentation.

This is a rare presentation of scrub typhus with cerebral venous thrombosis. A 32-year-old woman presented with signs of raised intracranial tension. Examination revealed maculopapular skin rashes and an 'eschar' over the right thigh. Nuchal rigidity and bilateral papilloedema were found. Scrub typhus was diagnosed by the presence of IgM antibody in serum. CT scan of the brain showed cerebral oedema. MRI of the brain was normal. Magnetic resonance venography of the brain showed thrombosis of several venous sinuses. Cerebrospinal fluid analysis revealed lymphocytic pleocytosis with raised protein level. Other causes of prothrombotic states were ruled out by doing specific test results. There was no history of hormonal contraception and prolonged bed rest. A case of scrub typhus complicated with meningoencephalitis and cerebral venous thrombosis was diagnosed. She responded to treatment with doxycycline, anticoagulants, antipyrectics and intravenous saline. Early identification of such atypical neurological involvement in scrub typhus was helpful in satisfactory outcome.

Hypoparathyroidism in a Case of Transfusion Dependent Thalassemia.

Repeated blood transfusions in transfusion dependent thalassemia (TDT) leads to iron overload-related endocrine complications. Hypoparathyroidism (HPT) with severe signs of hypocalcemia is a recognized complication among these patients. A 14-year-old thalassaemic boy, on regular transfusion and on anticonvulsant therapy with a presumptive diagnosis of epilepsy for the last 1 year, was admitted with high fever and severe muscle cramps with positive Trousseau's sign. He was diagnosed as a case of primary HPT and magnesium deficiency on the basis of low serum calcium, high phosphate, normal alkaline phosphates, very low intact parathyroid hormone (iPTH), normal serum vitamin D and very low serum magnesium level. His calcium, magnesium and phosphate level normalised following treatment with intravenous magnesium and calcium. His iPTH improved but remained at low normal. He was discharged from hospital with oral calcium, calcitriol, and magnesium supplementation. The anticonvulsant (Phenobarbitone) was successfully withdrawn gradually over the next six months without any recurrence of seizure in the subsequent 3 years of follow up. Acquired HPT (apparently from hemosiderosis) is a common cause of hypocalcemia; and magnesium depletion further complicated the situation leading to severe hypocalcemia with recurrent episodes of convulsion. Magnesium replacement improved the parathyroid hormone (PTH) value proving its role in acquired HPT. Very high phosphate level on admission and poor PTH response with respect to the low serum calcium, indicates intrinsic parathyroid pathology. Metabolic abnormalities should always be evaluated in thalassaemic subject with seizure disorder and it appears that the initial convulsive episodes were due to hypocalcemia. Muscle pain, cramps or convulsion may occur from HPT and simultaneous magnesium deficiency in transfusion dependent thalassaemic subjects. Metabolic correction is more important than anticonvulsant medication. Calcium and magnesium should both be assessed routinely in transfusion dependent thalassemic patients.

Guillain-Barré Syndrome developing in a patient with Graves' Disease.

Graves' disease (GD) and Guillain-Barré syndrome (GBS) are both autoimmune disorders and are triggered by interactions between genetic and environmental factors. GBS in patients who suffer from other autoimmune diseases is rarely reported, and the development of atypical GBS with cranial nerve involvement in a patient with GD has never been previously reported. Herein, we report a patient with GD and a rare form of pharyngo-cervico-brachial variety of GBS.

Psychometric properties of Bengali version of QOLIE-10 in epileptic patients.

UNLABELLED: Assessment of Quality of Life in Epilepsy has currently been emphasized to provide comprehensive care to patients. AIM: To develop and standardize and assess the psychometric properties of Bengali version of QOLIE-10 and to assess the relationship of quality of life with seizure variables and presence of psychiatric morbidity. DESIGN: English QOLIE-10 was translated into Bengali by a translation committee using translation-re-translation technique. Inter-rater reliability between the English and Bengali version was assessed during initial practice session held amongst 20 bilingual patients. It was found that item 3 (related to driving) was reported to have difficulty in answering by all the patients as none drove any vehicle. Thus, this item was dropped. The inter-rater reliability of the resultant 9 item scale was found to be high (kappa = 0.9). One hundred and seven epilepsy patients attending the Epilepsy clinic were selected for the study if they met the following criteria: age >15 years, duration of seizure >1 year, regular intake of antiepileptic drugs, presence of informant and ability to read Bengali. For each patient, demographic and clinical data (seizure frequency, last seizure date, seizure type as per record, medicine intake history and records of past investigations such as EEG) was collected. Each patient were administered QOLIE-9 (Bengali) and SRQ-24 Bengali version to screen for psychiatric morbidity. RESULTS: The Cronbach's Alpha coefficient for QOLIE-9 was 0.81, which did not improve if any item was dropped. All items showed strong correlation with the total score. The instrument showed stable factor structure with three factors (Limitation, Depression, Illness effects). However, the item with regard to memory problem did not fit into any of the factors. The QOLIE-9 total showed a significant correlation with the seizure frequency (r = 0.76**). SRQ positive (i.e., suspected psychiatric morbidity) cases had higher QOLIE-9 score (thus, poorer quality of life) in comparison to non-psychiatric cases. CONCLUSION: Bengali QOLIE-9 is a valid and reliable instrument to assess the quality of life in patients suffering from epilepsy.