A case of central nervous system vasculitis secondary to rheumatoid arthritis treated successfully with mycophenolate.

Here, we report a case of central nervous system vasculitis in rheumatoid arthritis (RA) treated successfully with corticosteroid and mycophenolate. A 53-year-old female with poorly controlled RA presented with unilateral lower motor neurone type seventh cranial nerve palsy. Magnetic resonance imaging (MRI) of the brain revealed bilateral signal intensity changes in posterior fossa, basal ganglia, and periventricular white matter with contrast enhancement. Rheumatoid factor, anti-cyclic citrullinated peptides, and C-reactive protein were high. Cerebrospinal fluid analysis showed pleocytosis with elevated protein. Digital subtraction angiography of the brain revealed a beading pattern in distal branches of anterior, middle, and posterior cerebral arteries bilaterally suggestive of central nervous system vasculitis. We treated the patient with intravenous methylprednisolone followed by oral prednisolone tapered off in 6 months. Mycophenolate mofetil was started at the dose of 2 g per day, along with prednisolone. Sulfasalazine and hydroxychloroquine were also started to treat her arthritis. The patient showed complete neurological improvement, along with a significant resolution of the lesions in MRI.

Is peripheral alpha synuclein a marker for gait velocity in Parkinson's disease?

BACKGROUND: The extent of gait abnormality is non-uniform across motor phenotypes of Parkinson's disease (PD). The biological basis of this heterogeneity remains intriguing. Moreover, the relationship of gait impairment with various neurodegenerative protein markers in PD is not well established. OBJECTIVES: Here, we aimed to explore the interplay between gait parameters and specific serum protein markers in PD. METHODS: A total of 62 PD patients were consecutively recruited. Blood samples and gait data were acquired from 37 and 34 patients respectively. Two-dimensional spatio-temporal gait parameters were estimated using an electronic walkway (GAITRite®, CIR Systems Inc., USA). Serum phosphorylated alpha synuclein (p-Ser129-a-syn) and total a-syn levels were measured using commercially available ELISA kit. Data was analyzed using SPSS Version 20 (IBM). RESULTS: We found that phosphorylated a-syn levels were significantly higher in PD patients with postural instability and gait difficulty compared to tremor dominant variant. Significant reduction in gait velocity was also observed with increasing levels of this pathological form of a-syn. Regression modelling showed that phosphorylated a-syn is an independent predictor of gait velocity. DISCUSSION: Our findings indicate that concentrations of peripheral p-Ser129-a-syn but not total a-syn could be a potential contributor of gait impairment in PD. Further investigation on the systemic role of phosphorylated a-syn on gait would bridge the gap between central and peripheral mechanisms underlying phenotypic variability in PD.

Efficacy of leflunomide as a steroid-sparing agent in treatment of Indian giant cell arteritis patients: A 2-year follow-up study.

OBJECTIVES: To evaluate the effectiveness of leflunomide as a steroid-sparing agent among Indian patients with giant cell arteritis (GCA) and to assess the changes of "halo sign" within affected arteries, detected ultrasonographically, after remission. METHODS: In this prospective observational study, patients fulfilling American College of Rheumatology criteria for GCA and having halo sign in temporal artery ultrasound were treated with leflunomide and predefined tapering dose of prednisolone. Ultrasounds of temporal and axillary arteries were done at baseline and after remission were achieved. RESULTS: Twenty-two GCA patients were followed up for a median duration of 24 months (interquartile range, IQR: 18-33). All patients showed clinical improvement and steroids could be stopped in 17 out of 22 patients. Median time to achieve remission (symptom-free with normal inflammatory markers) was 3 (95% confidence interval [CI]: 2.4-3.6) months. Median time to achieve reduction to prednisolone dose <5 mg/d was 9 months (95% CI: 7-11). Prednisolone dose could be reduced in all patients while on leflunomide, suggesting steroid-sparing effect and a steroid-free remission could be achieved after a median of 14 months (95% CI: 9.4-18.6). Ultrasonographically, all patients showed improvement of halo signs, after 8 weeks (IQR 7.25-12). Seven patients experienced a clinical relapse after 12 months (IQR: 5-21) of initial remission. The predictors of relapse were duration of symptoms before initiation of immunosuppression therapy and delayed achievement remission by strict criteria. CONCLUSION: This study showed efficacy and safety of leflunomide as a steroid-sparing agent in Indian GCA patients.

The ring in the neck-managing paraparesis in cervical intramedullary tuberculoma: Inputs from a neurorehabilitation desk.

Central nervous system (CNS) tuberculosis is a less common entity even in endemic countries like India. Involvement of spine is much less frequent than brain, but concurrent involvement of brain and spine by tuberculoma is rare. A 23-year-old female with paraparesis was diagnosed as having cervical ring enhancing lesion in MRI suggesting intramedullary tuberculoma along with long segment cervicodorsal edema. On brain screening she had multiple intracerebral tuberculomas without any evidence of tuberculosis elsewhere in the body. She was treated with a multidisciplinary approach including neurological management and neurorehabilitation, with remarkable clinical recovery. In presence of acute neurological deficit, early start of rehabilitation along with medical management can give outstanding results in terms of neuro-recovery and improvement of residual neurodeficits. Surgical intervention can be avoided in many cases.

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.

A randomized, open labeled study comparing the serum levels of cobalamin after three doses of 500 mcg vs. a single dose methylcobalamin of 1500 mcg in patients with peripheral neuropathy.

BACKGROUND: Vitamin B12 deficiency has been associated with peripheral neuropathy, loss of sensation in the peripheral nerves, and weakness in the lower extremities. Methylcobalamin is the most effective analogue of vitamin B12 used to treat or prevent the complications associated with vitamin B12 deficiency. The current study aimed to compare the serum cobalamin levels after administration of two different regimes of methylcobalamin in peripheral neuropathy patients. METHODS: The present study was a prospective, randomized, comparative study. The study consisted of two parallel groups, group A (methylcobalamin 500 µg injection intramuscularly three times a week) and group B (methylcobalamin 1500 µg injection intramuscularly once a week). A control group of healthy volunteers was also included. RESULTS: A total of 24 patients (12 in each group) were included in the study. Five healthy volunteers were also included as a control in each group. At the end of treatment, serum cobalamin levels were significantly (P = 0.028) higher in group A (1892.08 ± 234.50) as compared with group B (1438.5 ± 460.32). The serum cobalamin levels in Group A healthy volunteers were also two times higher than that of group B (P = 0.056). Both the LANSS scale and DN4 questionnaire reported similar results at end of treatment. CONCLUSIONS: The 500 µg methylcobalamin thrice weekly regime is more effective in increasing the serum cobalamin levels as compared to the 1500 µg methylcobalamin once weekly regime.

Clinico-radiological profile and outcome of dengue patients with central nervous system manifestations: A case series in an Eastern India tertiary care hospital.

BACKGROUND AND OBJECTIVE: Dengue, an acute viral disease, transmitted by Aedes mosquitoes, has a variable clinical spectrum ranging from asymptomatic infection to life-threatening dengue hemorrhagic fever and dengue shock syndrome. However, neurological complications, in general, are unusual but have been observed more frequently in the recent past, and some studies highlighted varied neurological complications during the course of illness. Although dengue is classically considered a nonneurotropic virus, there is increasing evidence for dengue viral neurotropism. In this study, we have evaluated clinico-radiological profile and outcome of nine serologically confirmed dengue patients having varied manifestations of central nervous system (CNS) involvement. MATERIALS AND METHODS: All the consecutive patients presented with neurological complications with positive serology for dengue infection (IgM positivity) in Department of Medicine, in a tertiary care hospital in Eastern India from August 2013 to October 2014 were included in the study. These patients were subjected to a detailed clinical evaluation, laboratory assessment including complete hemogram, coagulation profile, liver function test, serum electrolytes, and routine CSF (Cerebrospinal Fluid) study with the exclusion of other common neuroinvasive pathogens. RESULTS: Out of 9 patients with neurological complications associated with confirmed dengue infection, 2 (22%) patients had dengue encephalopathy, 5 (56%) patients have dengue encephalitis, 1 (11%) patient had dengue meningitis, and 1 (11%) patient had postdengue immune-mediated CNS involvement. CONCLUSION: This case series reaffirms the occurrence of varied CNS manifestations in dengue virus infection and underlines the importance of inclusion of dengue in the differential diagnosis of acute encephalitis syndrome.

A rare presentation of Hodgkin's lymphoma in a very young child, with involvement of the appendix.

Hodgkin's lymphoma involving the appendix in young children is an exceptionally rare disease. We report a case of a child less than 3 years who presented to us with gradual weight loss, progressive pallor and diffuse abdominal pain. The symptoms were preceded by a history of varicella infection about 6 months ago. The clinical progression was marked by intermittent episodes of acute abdominal pain and fever, mimicking acute intra-abdominal inflammatory process such as appendicitis. Investigations revealed that the child had direct Coomb's test positive haemolytic anaemia, raised platelet counts, lymphopenia and hypergammaglobulinaemia. The CT of the abdomen showed the presence of significant lymph nodes. Abdominal laparoscopy and biopsy of the lymph nodes showed mixed cellularity Hodgkin's lymphoma that also involved the appendix. Subsequent staging detected an advanced stage IV disease. The child was referred immediately to a specialised oncology centre for further management. Unfortunately he was lost in follow-up.

A case of adult onset Still's disease with flare of arthritis.

Adult onset Still's disease is a febrile illness of unknown aetiology, accounting for about 6% cases of fever of unknown origin. It was first described by Eric GL Bywater in 1971, and since then few case series have been reported from India and other parts of the globe. A case of adult onset Still's disease who presented with fever and polyarthralgia of 18 months duration with flare of joint symptoms during fever spikes has been reported.

Papillon-Lefevre syndrome-like presentation in chronic arsenicosis: A rare mimicry.

Chronic arsenicosis is a major health and occupational problem in rural parts of West Bengal such as in parts of the Gangetic plain of India. Chronic arsenicosis occurs due to accidental ingestion of repeated amounts of small doses by those working with metal or by taking food or drink in which there are traces of arsenic. Chronic exposure may result accumulation in the hair, nail, and skin. Arsenic can also cross the placenta. Papillon-Lefèvre syndrome is a rare disease characterized by skin lesions caused by palmar-plantar hyperkeratosis and severe periodontal destruction involving both the primary and permanent dentitions. Until date, more than 200 cases have been reported worldwide. Palmoplantar hyperkeratosis is a major manifestation in both chronic arsenicosis and Papillon-Lefèvre syndrome. We report herein a rare case of chronic arsenicosis in a patient from rural Bengal, whose all features mimic Papillon-Lefèvre syndrome. It is probably the first case of Papillon-Lefevre syndrome-like presentation in chronic arsenicosis from India.