A Curious Case of Ankylosing Spondylosis and Motor Neuron Disease: A Mere Coincidence or Correlation?

Ankylosing spondylitis (AS) is a chronic (progressive) painful inflammatory rheumatic disease with genetic predisposition. Genetic susceptibility and common expression cause susceptibility to other inflammatory diseases such as psoriasis, ulcerative colitis, and Crohn's disease. However, cases of motor neuron disease (MND) in patients of biologically treated patients of AS have been rarely reported. AS does not follow the same course in everyone; even among affected members of one family, the outcome varies. Here, we present a case of an unusual AS without expression of human leukocyte antigen-B27 genetic marker who subsequently develops amyotrophic lateral sclerosis the most common form of MND. This mere correlation of one noncurable disease with one potentially treatable chronic rheumatological condition adds our knowledge to existing literature.

Tolosa-Hunt Syndrome and Ocular Myasthenia: A Rare Coexistence or Real Association.

Tolosa- Hunt syndrome is a rare steroid responsive disorder caused by granulation tissue involving the cavernous sinus or superior orbital fissure presenting as painful ophthalmoplegia and facial pain. In this report, we describe coexistence of Tolosa-Hunt syndrome with ocular myasthenia which may point towards an autoimmune etiological basis behind the cavernous sinus granulation tissue formation and also offered therapeutic challenge to ameliorate the symptoms.

Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Negative Small Vessel Vasculitis: A Rare Cause of Pulmonary Renal Syndrome.

Pulmonary renal syndrome (PRS) is characterized by both diffuse alveolar haemorrhage and glomerulonephritis as pathological features. Several immunologic and non-immunologic mechanisms including anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus are commonly involved in the pathogenesis of the syndrome. We herein present a 60-year-old woman, non-smoker, who presented with fever, polyarthralgia, and the spreading of generalized purpuric rashes. The patient displayed rapid deterioration over the following two weeks marked by progressive declining renal function followed by haemoptysis. The patient was subsequently diagnosed with PRS, which was confirmed by the radiological evidence of alveolar haemorrhage and the histopathological evidence of pauci-immune glomerulonephritis. All immune markers including ANCA were negative. The patient was successfully treated with hemodialysis and immunosuppressive therapy. ANCA-negative vasculitis is a rare entity and even more rare as an etiology of PRS. An early diagnosis of this disease and its timely intervention is crucial.

Quality of Life and Clinical Response to On-Demand Maintenance Doses of Infliximab in Patients With Ankylosing Spondylitis.

AIM: The aim of this study was to study the effect of modified maintenance doses (MDs) of infliximab on the quality of life (QoL) of patients with ankylosing spondylitis (AS) over a period of 3 years. METHODS: Medical records of AS patients (n = 25) who received a normal induction dose but modified MDs as required were retrospectively analyzed. After induction dose and the first MD, patients were followed up every month and were treated with infliximab whenever Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) score was 4 or greater. The study end points were the percentage of responders defined as reduction of 40% or greater in BASDAI score and improvement in QoL defined by mean change in SF-36 Physical Component Summary score, SF-36 Mental Component Summary score, and Ankylosing Spondylitis Quality of Life (ASQoL) values at week 6 and after the last MD (ie, at the end of 3 years) compared with baseline. RESULTS: Majority of the patients were males (n = 20), and the mean age of the analysis population was 40.6 ± 10.79 years. At the end of 6 weeks and after the last MD, BASDAI 40 scores were achieved in 100% and 92% of the patients, respectively. From baseline, the mean change in BASDAI score at the end of 6 weeks and after the last MD is -3.56 and -3.40, respectively. The overall mean change in scores (BASDAI, SF-36 Physical and Mental Component Summary, and ASQoL) versus baseline, at 6 weeks, and after the last MD was statistically significant (P < 0.0001). CONCLUSIONS: The results of the study suggest that initial induction and an on-demand MD regimen of infliximab based on BASDAI were associated with significant improvement in disease activity and QoL.

Hashimoto's Encephalopathy Presenting with Chorea.

Hashimoto's encephalopathy (HE) is a steroid-responsive relapsing neuropsychiatric disorder associated with high titers of antithyroid antibody with or without thyroid dysfunction. We report a case of HE in a 78 year old female who developed sudden onset behavioral abnormalities associated with choreiform movement of extremities. All causes of vascular, infective, metabolic, autoimmune, paraneoplastic and toxic encephalopathy were excluded. Anti-thyroid peroxidase (anti-TPO) antibody was found to be raised with very high titre. A diagnosis of HE was made. Prompt treatment with high dose steroid led to dramatic improvement of symptoms including choreiform movement.

Spectrum of peripheral neuropathy in eastern India.

Peripheral neuropathies constitute an important group of disorders in neurological practice. Very few systematic studies on peripheral neuropathies are available from India. Hence we conducted a prospective study in two large hospitals from Kolkata, the biggest city of eastern India in order to find out the spectrum of peripheral neuropathy. This prospective study was carried out from June 1998 to January 2003 on admitted patients with symptoms and signs of peripheral neuropathy. Two hundred and twenty-five patients were evaluated (one hundred and twenty-five from an industrial hospital and one hundred from an academic tertiary care institution at Kolkata). Result showed that most of the cases of peripheral neuropathy belonged to fourth decade with men dominance. Common varieties of neuropathy were Guillain-Barré syndrome, diabetes mellitus, hereditary motor sensory neuropathy, chronic inflammatory demyelinating neuropathy, drugs and toxin related. Unusual varieties were Isaacs's syndrome and X-linked hereditary motor-sensory neuropathy. One case of neuropathy due to Plasmodium vivax has received intravenous immunoglobulin therapy. The type of peripheral neuropathy in hospital-based patients in eastern India was similar to other parts of country excepting some sporadic types due to infective or genetic causes and a large number of cases of undetermined aetiology exist despite detailed investigations.