Ocular complications in cryptococcal meningitis (CM) are commonly attributed to elevated intracranial pressure (ICP). We report a case of reversible vision loss complicating acquired immunodeficiency syndrome (AIDS) related to CM with a normal ICP. The patient had sudden onset painless blindness during the anti-retroviral therapy (ART) and anti-fungal therapy. On evaluation, clinical and radiological findings of optic neuritis were present. While reviewing the literature for causes of blindness in CM, we concluded the cause was optic neuritis due to immune reconstitution inflammatory syndrome (IRIS) because of concomitant ART intake by the patient. We witnessed dramatic visual improvement after the use of systemic corticosteroids. The potential significance of this case report is to highlight the possible role of corticosteroids in the prevention of blindness due to CM.

Adenylyl cyclase 5 (ADCY5)-related dyskinesia is a rare disorder characterized by early-onset paroxysmal choreoathetosis, dystonia, myoclonus, or a combination thereof, primarily involving the limbs, face, and neck. Other core clinical features include nocturnal ballistic bouts and facial dyskinesias, often followed by remission during adolescence, as well as axial hypotonia. Any mixed movement disorder accompanied by the aforementioned core features should prompt genetic testing for ADCY5 mutation. We report the case of a 31-year man from North India with a childhood-onset history of clumsy, random, and jerky body and facial movements, interfering with his speech and object-handling and walking-related functional abilities. He improved symptomatically with zonisamide and was offered caffeine as a maintenance option. Here, we review the literature on this entity, while reporting this first case of ADCY5-related dyskinesias from North India. The phenotypic spectrum linked to ADCY5 mutations has significantly broadened since its initial description in a family with “familial dyskinesia and facial myokymia.” This phenotypic variability could be responsible for a proportion of “idiopathic” hyperkinetic movement disorders. Gaining a better understanding of its clinical manifestations helps in identifying complex or uncommon cases more effectively.

Aim: The purpose of the study was to characterize the focal liver lesions on contrast enhanced USG on the basis of enhancement pattern and establishing the potential of this technique and determining sensitivity and diagnostic accuracy of this technique in comparison with CECT/biopsy. Material and methods:Prospective study was conducted from Oct 2018 to Mar 2020 at tertiary care hospital and CEUS was done for 34 lesions in 30 patients. Lesions characterized on the basis of enhancement pattern in arterial, portal and porto-venous phases. Final diagnosis was established on the basis of CECT or/and biopsy/FNAC of lesion (if available). Out of 34 focal li Results: ver lesions, 24 were malignant (HCC and metastasis) and 10 were benign (hemangioma). 32 lesions were correctly characterized by CEUS examination in comparison with CECT/tissue diagnosis. Overall sensitivity, PPV and diagnostic accuracy of CEUS with 95% CI was 94.12 (80.32 -99.28), 100 (89.11 – 100%) and 94.12% (80.32 – 99.28) respectively. Our study showed that CEUS is a sensitive modality to c Conclusion: haracterize focal liver lesions. CEUS can objectively demonstrate contrast enhancement comparable and corresponding to enhancement of same lesion on CECT.

A 39-year-old male without any preceding medical ailment presented with 12 day history of fever followed by behavioral changes with left sided weakness. He was found to have Herpes Simplex Virus-1 encephalitis (polymerase chain reaction positive) and with Magnetic Resonance Imaging finding of asymmetrical frontotemporoparietal (right side affected more than left side) involvement with patchy enhancement with atypical nodular enhancement and subtle diffusion restriction. Nodular enhancement is rare in acute inflammations and is reported mainly with chronic granulomatous infections.

Introduction The aim of the research is to evaluate the clinical outcome among moderate-severe COVID-19 patients treated with standard drug therapy plus LMW Heparin as compared to those not given LMW Heparin. It has several objectives such as to evaluate the clinical outcome among moderate-severe COVID-19 patients admitted to RUHS-HMS and treated with LMW Heparin along with Standard Treatment Protocol by assessing their in-hospital course of illness, duration of hospital stays, recovery, and mortality. To identify the possible risk factors associated with COVID Patients. Methods A retrospective, observational study is undertaken. The study is conducted in the hospital attached to RUHS College of medical sciences, Jaipur, Rajasthan. The study is conducted over a period of eight months. The data which is achieved from the study isanalyzed statistically. For this, the usage of SPSS version 17.0 software is done. The test which is used is the standard deviation. The normal distribution of the continuous variables between the groups isanalyzed with the help of a t-test the signicance level is set up at less than 0.05. ResultsIt revealed that the adminstration of heparin was associated with lower mortality in patients admitted with COVID-19 as in group A patients who received LMW Heparin, mortality was 11% with or without comorbidities whereas in group B it was found 18.68%. The presence of the disease is found to be associated with several other underlying conditions like liver, cardiovascular, kidney disease, and others. Conclusion It is concluded that the heparin usage is helpful and effective while treating the infection of COVID-19. Moreover, it is accountable for declining the rate of mortality in the patients. The current study also states that the underlying cardiovascular diseases are responsible for increasing the threat of mortality in patients who are suffering from COVID-19.

Treatment-induced neuropathy in diabetes (TIND), better known as insulin neuritis, is an uncommon, iatrogenic, small fibre neuropathy occurring with an abrupt betterment in glycaemic control in patients of chronic hyperglycaemia. TIND, as such, is a less reported entity often missed by clinicians on initial presentation, but is an important consideration in patients with rapid correction of their hyperglycaemic status. TIND is characterised by an acute onset length dependent or generalised neuropathic pain often accompanied by autonomic symptoms and signs. This mostly occur after 2-8 weeks of initiating glycaemic control with insulin and is associated with a decline in glycosylated haemoglobin (HbA1c) by ≥2% points over 3 months. In fact, these patients on presentation, have reasonably satisfactory glycosylated haemoglobin profiles, as this condition is seen with acute control of glycaemic status. We herein discuss details of this entity as a review of literature with an overview of its pathophysiology, clinical features, and management.

Background The coronavirus disease 2019 (COVID-19) pandemic has been impacting individuals throughout the world. Millions have been affected, and while many have recovered, a growing number of recovered COVID-19 patients are reportedly facing neurological symptoms, described as "slow thinking," "difficulty in focusing," "confusion," "lack of concentration," "forgetfulness," or "haziness in thought process." These experiences of mental fatigue, associated with and related to mild cognitive impairments, may be conceptually defined as "brain fog." Objective To study the prevalence and severity of these brain fog symptoms in COVID-19 recovered patients, and examining their association with age, gender, and COVID-19 symptom severity. Methods A total of 300 patients who tested positive for Real-Time Reverse Transcriptase-Polymerase Chain Reaction (RT-PCR) for SARSCoV-2 during April-August 2020 were included in our study after complete recovery from their acute illness. They were assessed for brain fog symptoms using the 9-item validated Wood's mental fatigue inventory. Results/Conclusions The overall cumulative prevalence of any components of brain fog was 34%, with a mean score of 6.11 +/- 1.7 in those who experienced it. Males were more affected than females (42.3% vs. 29.1%) with males scoring higher than females. The mean score was higher in severe ill and Intensive Care Unit (ICU) patients and those who required oxygen or were on a ventilator.

Background Primary nerve sheath tumor of cranial nerve is a rare intracranial space occupying lesion that most commonly involves vestibular nerve. Oculomotor nerve schwannoma, without neurofibromatosis, is an extremely uncommon entity especially in children. Though any cranial nerve can be involved by schwannoma except I and II nerve as these cranial nerves lack a Schwann cell sheath. Case presentation We herein report a 14-year-old boy presenting with an oculomotor schwannoma in the absence of features of neurofibromatosis, manifesting as progressive diplopia, ptosis, and blurring of vision. Conclusions Most similar previous case reports in literature reported oculomotor schwannoma in adults, unlike our case. The detection rate of such rare lesions has, however, increased in the last two decades due to neuroimaging advances.

Aims: To assess retinal nerve fiber layer thickness by optical coherence tomography (OCT) in various central nervous system demyelinating diseases. Methods and Material: A total of 40 patients were selected. There were 8 patients of multiple sclerosis, 12 patients of neuromyelitis optica spectrum disorders (NMOSD), 9 patients of optic neuritis, and 11 patients of longitudinally extensive transverse myelitis (LETM). The fast retinal nerve fiber layer (RNFL) thickness protocol was used to compute the overall average RNFL thickness for each eye. Average RNFL thickness for 360 degrees around the optic disk, for the superior, nasal, inferior, and temporal quadrants around the optic disk as well as in the twelve 30 degrees segments (with 3 o'clock position as nasal) were recorded. Results: Average RNFL thicknesses were lower in NMOSD group. Superior, inferior, and temporal quadrants are the preferentially affected while 8 o'clock position RNFL was the thinnest. MS involved 8 o'clock position and temporal quadrant. Of 9 o'clock position and temporal quadrant were the thinnest in LETM. In idiopathic optic neuritis, lowest RNFL value was recorded in the nasal quadrant. In 3 o'clock position, maximum thinning was noted in idiopathic ON. At 9 o'clock position, maximum thinning was seen in LETM patients. Conclusions: Most o'clock positions and superior, inferior, and temporal quadrants were affected in NMOSD. MS mainly involved 8 o'clock position and temporal quadrant. LETM preferentially affected 9 o'clock position and the temporal quadrant. Hence, observing the pattern of RNFL thinning with OCT in CNS demyelinating diseases can intimate the etiological cause. However, larger studies are needed further to confirm the same.

A peculiar brachial plexus syndrome was first described in 1924 by Pancoast, 1 (N Engl J Med, 1997,337) wherein an apical pulmonary tumor invades the C8 and T1 extraspinal nerves, the sympathetic chain, stellate ganglion, and adjacent structures. Echinococcal cysts are slowly enlarging masses, generally remaining clinically silent until their expanding size or their space‐occupying effect in an involved organ elicits symptoms. These infections are prevalent in most areas where livestock is raised in association with dogs. Pulmonary hydatid cysts are also usually only evident when they are large enough to exert mechanical compression or are complicated due to their rupture. (Clin Chest Med, 2002,397) Pancoast syndrome due to infectious causes have been previously less described, (Am J Med Sci, 2011, 333) and hence, we report pulmonary hydatid cyst as a treatable cause of this syndrome.

CNS Tuberculosis, the most rampant infection of the developing world, clinicopathologically can occur alone or combined as Tubercular Meningitis, Tuberculomas (intracranial and/or spinal), Arachnoiditis (basal, optochaismatic, spinal) or Myelitis. The viscous exudates in basal cisterns cause Optochiasmatic arachnoiditis[1], and nodular, thickened, clumped nerve roots called Lumbosacral arachnoiditis.[2] We herein propose use of intrathecal hyaluronidase, as adjuvent for symptomatic as well as impending arachnoiditis.

Background Cerebral arterial thromboses or ischemic strokes may be caused by cumulative or independent effects of a variety of risk factors. High factor VIII level is one of those important but less known risk factors for arterial and venous thrombosis. We hereby provide a comprehensive review of the role of high factor VIII levels as a risk factor of arterial thrombosis. Moreover, we present our views on inclusion of factor VIII testing in the etiology workup protocol of young patients with ischemic strokes and their treatment with anticoagulant therapy. Case presentation We illustrate a case of 32-year-old North Indian female patient with Ischemic stroke whose only identifiable risk factor was revealed to be an elevated factor VIII level. She was treated with oral anticoagulant with an uneventful follow-up of 6 months. Conclusions Elevated factor VIII levels have their independent and additive effects in causation and prognosis of arterial strokes. We herein discuss the mechanism of this association, the feasibility and yield of routine testing, appropriate cut-off levels, and further treatment protocol especially in young stroke patients.

Cerebral small vessel disease (CSVD) is a well-known cause of vascular dementia. Though a majority of these cases are sporadic, familial monogenic causes are being frequently identified as well. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a rare autosomal recessive CSVD, caused by mutation in HTRA 1 gene on chromosome 10q (10q25.3-q26.2) in homozygous or compound heterozygous form. Indian literature has been quite scant with very few case reports of CARASIL, and only three familial cases were confirmed with mutational analysis. Testing facilities of HTRA 1 genetic mutation are now more widely available in India than before, and should be encouraged for appropriate patients. This would help in diagnosing, prognosticating and avoiding unnecessary further investigations and medications for these patients. We herein review the Indian scenario and our previously reported experiences of this disorder, while adding a case from north India with a befitting clinical history, family history, neuroimaging and documented HTRA1 genetic mutation.

A 65 year old male, with 2 years presented with history of slowness of daily activities, occasional backward falls, apathy & behavioral complaints, and double vision.

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with vasculitic neuropathy and being rare can present as subacute symmetric sensorimotor quadriparesis mimicking Guillain–Barre syndrome (GBS). It warrants timely diagnosis as treatment for both conditions is different and vasculitic neuropathy needs long-term immunosuppression. Nerve biopsy of our patient showed eosinophilic infiltration along with mononuclear infiltrate. Typical histopathological presentations of EGPA are different among different organs and eosinophilic infiltration is rarely observed in peripheral nerve and kidney involvements. Case presentation A 49-year-old female with a history of asthma with 3-week duration of acute onset ascending weakness, preceded by severe pain and burning in glove and stocking pattern. Nerve conduction studies could not rule out Guillain–Barre syndrome initially, but subsequent studies show axonal affection and she received intravenous immunoglobulin (IVIg) but her weakness progressed after slight improvement. Her bloodwork revealed marked eosinophilia (> 50%) with computed tomography (CT) paranasal sinuses showing pansinusitis with background history of asthma led us towards eosinophilic granulomatosis with polyangiitis and later antineutrophil cytoplasmic antibodies came out positive with nerve biopsy showing perivascular mononuclear inflammation with eosinophils. She was started on steroids immediately and then received intravenous rituximab in view of long-term immunosuppression with maintenance steroids and on follow-up she improved. Conclusion Eosinophilic granulomatosis with polyangiitis is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies with significant paranasal sinuses involvement. Mononeuritis multiplex is the most common presentation of vasculitic neuropathy of eosinophilic granulomatosis with polyangiitis, but they can mimic Guillain–Barre syndrome and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different.

Introduction: Rheumatoid Arthritis (RA) is a chronic multisystem immune mediated disease. Rheumatoid associated neuropathy causes significant disability and adds to the economic burden. Aim: To assess clinical determinants of peripheral neuropathy (diagnosed electrophysiologically using nerve conduction studies) among patients with RA. Additionally, it was also aimed to study the various patterns of peripheral neuropathy in patients with RA. Materials and Methods: A cross-sectional study was conducted on 100 consecutive adult patients with RA between 01stFebruary, 2020 to 02nd January, 2021 at medicine and neurology departments of SMS Medical College and Hospital, Jaipur, Rajasthan, India. Inclusion and exclusion criteria were followed and eligible patients after appropriate laboratory evaluation underwent nerve conduction studies. Statistical analysis was performed using student’s unpaired t-test and Chi-square test for continuous and categorical variables respectively. Results: Mean age of the study population was 42.4±14.2 years with 88 females and 12 males. Mean duration of RA was 7.0±7.4 years. Nerve conduction studies detected neuropathy in 18 patients, of these only four patients were symptomatic with tingling, pins and needles sensation and numbness. Fourteen patients had subclinical neuropathy. Patients with neuropathy had significantly longer disease duration (p=0.0001), were older (p=0.014) with more joint deformities (p=0.0008). Conclusion: Subclinical neuropathy is not infrequent in RA patients. Those with advanced age, longer disease duration, higher Erythrocyte Sedimentation Rate (ESR), erosions and deformities should be assessed electrophysiologically for neuropathy.

Annals of Indian Academy of Neurology ¦ Volume 24 ¦ Issue 4 ¦ July-August 2021 624 Dear Editor, Bickers taff bra ins tem encephal i t i s (BBE) is an immune-mediated, rare form of encephalitis traditionally characterized by progressive, relatively symmetrical external ophthalmoplegia, ataxia, and disturbance of consciousness. Positive anti-GQ1b antibodies are found in 66% and abnormal brain MRI in 30% of patients. We herein report our patient who unusually had external plus internal ophthalmoplegia, hyperreflexia (not altered consciousness), normal brain imaging, and negative GQ1b profile. He was still diagnosed “Possible BBE” in accordance with postulated criteria for central nervous system involvement. We, therefore, with this report, emphasize on the clear-cut diagnostic criteria defining this disorder and also on its differentials based on acute bilateral ophthalmoplegia.