Mycophenolate mofetil as induction and maintenance immunosuppressive therapy in adult primary central nervous system vasculitis: A prospective observational study.

To observe the clinical and angiographic effectiveness of mycophenolate mofetil (MMF) as induction and maintenance immunosuppressive therapy in primary central nervous system vasculitis (PCNSV). In this open-label prospective study done at a tertiary care neurology centre, adult patients with PCNSV, diagnosed by Calabrese's criteria, were recruited from 2017 to 2021 and treated with glucocorticoids, MMF and standard of care. Patients were followed-up and clinical and angiographic changes were recorded. Total 26 patients were recruited with median age 39 years (34-49) with a slight female predilection (61.5%). Angiographic diagnoses were: small vessels disease 11.5%; large vessels disease 42.3% and both in 46.2%. Median duration of follow-up was 24.5 months (14.25-38). Proportion of patients with severe disability (modified Rankin Score (mRS) 4-6) at baseline was 73.08% (19/26) which reduced to 7.69% (2/26) (p < 0.001). At the last follow-up mRS = 0 was achieved in 38.5% (10/26) and mRS of ≤ 1 was achieved in 69.2% (18/26). Median time to achieve a mRS ≤ 1 was 12 months (95% CI: 6.8-17.2). Angiography was repeated in 16 patients after a median duration of 13 months (10.5-19.7), out of which 10 (62.5%) showed improvement and 5 (31.2%) showed non-progression of lesions. MMF may be an effective immunosuppressive therapy in adult PCNSV as both induction and maintenance. Serial DSA of brain may be useful to monitor the effect of treatment. Key Points • Mycophenolate mofetil is effective as induction and maintenance immunosuppressive therapy in PCNSV. • Repeat angiogram may be useful to monitor treatment response in PCNSV.

CNS Melioidosis: A Diagnostic Challenge.

Melioidosis is an emerging tropical disease. Central nervous system (CNS) melioidosis may present as a brain abscess or encephalomyelitis mimics tuberculosis. Early diagnosis and management decreases mortality as well as morbidity. This case of brain abscess and encephalomyelitis in a young man with no known comorbidities was a diagnostic challenge. The surgery helped in debulking as well as isolating the causative organism. Appropriate antibiotic therapy for melioidosis was lifesaving and prevented further complications.

Bruns Syndrome - An Unusual Presentation.

Bruns syndrome is characterized by attacks of sudden severe headache, vomiting, and vertigo precipitated due to abrupt movements of the head due to presence of mobile deformable intraventricular lesion causing episodic obstructive hydrocephalus. Proposed underlying mechanism is intermittent or positional CSF obstruction resulting from ball-valve mechanism. Most common etiologies are NCC and intraventricular tumors. Here we present an unusual case of Bruns syndrome that was initially MRI negative.

A Novel Wearable Device for Motor Recovery of Hand Function in Chronic Stroke Survivors.

Background. In monkey, reticulospinal connections to hand and forearm muscles are spontaneously strengthened following corticospinal lesions, likely contributing to recovery of function. In healthy humans, pairing auditory clicks with electrical stimulation of a muscle induces plastic changes in motor pathways (probably including the reticulospinal tract), with features reminiscent of spike-timing dependent plasticity. In this study, we tested whether pairing clicks with muscle stimulation could improve hand function in chronic stroke survivors. Methods. Clicks were delivered via a miniature earpiece; transcutaneous electrical stimuli at motor threshold targeted forearm extensor muscles. A wearable electronic device (WD) allowed patients to receive stimulation at home while performing normal daily activities. A total of 95 patients >6 months poststroke were randomized to 3 groups: WD with shock paired 12 ms before click; WD with clicks and shocks delivered independently; standard care. Those allocated to the device used it for at least 4 h/d, every day for 4 weeks. Upper-limb function was assessed at baseline and weeks 2, 4, and 8 using the Action Research Arm Test (ARAT), which has 4 subdomains (Grasp, Grip, Pinch, and Gross). Results. Severity across the 3 groups was comparable at baseline. Only the paired stimulation group showed significant improvement in total ARAT (median baseline: 7.5; week 8: 11.5; P = .019) and the Grasp subscore (median baseline: 1; week 8: 4; P = .004). Conclusion. A wearable device delivering paired clicks and shocks over 4 weeks can produce a small but significant improvement in upper-limb function in stroke survivors.

Nightmares with a starry sky - Treating neurocysticercal encephalitis, how far to go.

Cysticercosis, an infection caused by the larval stage of tapeworm Taenia solium, is the most common parasitic disease of the human nervous system and the single most common cause of acquired epileptic seizures in the developing world. Here, we describe the stormy course of a 67-year-old female with neurocysticercosis (NCC) having a recurrent encephalitic presentation. She went through the most severe spectrum of this disease, namely NCC encephalitis and disseminated cysticercosis and had a classical starry sky brain in neuroimaging. In contrary to the popular practice of avoiding antihelminthic drugs in such extreme presentation, as a desperate measure, we had to use albendazole in this case, which showed clinical and radiological improvement.

The Relationship Between Enhanced Reticulospinal Outflow and Upper Limb Function in Chronic Stroke Patients.

BACKGROUND: Recent evidence from both monkey and human studies suggests that the reticulospinal tract may contribute to recovery of arm and hand function after stroke. In this study, we evaluated a marker of reticulospinal output in stroke survivors with varying degrees of motor recovery. METHODS: We recruited 95 consecutive stroke patients presenting 6 months to 12 years after their index stroke, and 19 heathy control subjects. Subjects were asked to respond to a light flash with a rapid wrist flexion; at random, the flash was paired with either a quiet or loud (startling) sound. The mean difference in electromyogram response time after flash with quiet sound compared with flash with loud sound measured the StartReact effect. Upper limb function was assessed by the Action Research Arm Test (ARAT), spasticity was graded using the Modified Ashworth Scale (MAS) and active wrist angular movement using an electrogoniometer. RESULTS: StartReact was significantly larger in stroke patients than healthy participants (78.4 vs 45.0 ms, P < .005). StartReact showed a significant negative correlation with the ARAT score and degree of active wrist movement. The StartReact effect was significantly larger in patients with higher spasticity scores. CONCLUSION: We speculate that in some patients with severe damage to their corticospinal tract, recovery led to strengthening of reticulospinal connections and an enhanced StartReact effect, but this did not occur for patients with milder impairment who could use surviving corticospinal connections to mediate recovery.

Mucocutaneous manifestations of HIV infection.

BACKGROUND AND AIMS: Human immunodeficiency virus (HIV) is associated with various mucocutaneous features, which may be the first pointer towards the existence of HIV infection. This study was done to note the different mucocutaneous lesions present in the HIV population in eastern India. METHODS: Four hundred and ten HIV seropositive patients attending the outpatient and inpatient departments were included in the study. RESULTS: Out of 410 HIV positives, 40% had mucocutaneous involvement at presentation. The mean age of the study population was 29 years and male to female ratio was 2.5:1. The common mucocutaneous morbidities included oral candidiasis (36%), dermatophytosis and gingivitis (13% each), herpes zoster (6%), herpes simplex and scabies (5% each). A striking feature, noted in 36% males, was straightening of hairs. Genital herpes was the commonest genital ulcer disease. Lesions associated with declining immunity included oral candidiasis, oral hairy leukoplakia and herpes zoster with median CD4 counts of 98, 62 and 198/ L respectively. CONCLUSION: Early recognition of mucocutaneous manifestations and associated STDs help in better management of HIV/AIDS.

People living with HIV infection / AIDS - a study on lymph node FNAC and CD4 count.

Between July 1997 and December 2000, 1616 HIV seropositive persons were identified by Western Blot test at the School of Tropical Medicine, Kolkata. Four hundred seventy two (29.2%) of them had generalized lymphadenopathy. CD4 count could be done in only 54 of these 472 subjects (11.4%). These 54 patients, consisting of 40 males (74%) and 14 females (26%) were the subjects of the study. Their mean age was 29.5 years. In all these subjects, FNAC was done from the enlarged lymph nodes (non-inguinal). Reactive hyperplasia was seen in 30 cases (55.5%) whose absolute CD4 count varied between 411-945 cells/microL (median value 670 cells/microL). Evidence of tuberculous lymphadenitis was detected in 22 (41%) with CD4 counts varying between 113 and 422 cells/microL (median value 212 cells/microL). Non-Hodgkin lymphoma was diagnosed in 2 cases (3.7%) with CD4 count 79-113 cells/microL. All patients had evidence of HIV-1 infection, excepting one case of dual infection (HIV-1 and 2).