Patterns of peripheral neuropathy in Sjogren's syndrome in a tertiary care hospital from South India.

INTRODUCTION: Sjogren's syndrome (SS) is a systemic autoimmune disease that apart from involving the exocrine glands can affect any organ. Involvement of the peripheral nervous system results in a wide spectrum of neuropathic manifestations. OBJECTIVE: To evaluate the clinico-electrophysiological patterns as well as pathological characteristics of neuropathy in SS patients presenting to a neuromuscular clinic in a tertiary hospital from South India. MATERIALS AND METHODS: This is a retrospective study from the Departments of Neurology, Rheumatology, and Pathology from Nizam's Institute of Medical Sciences. Twenty-one patients with the diagnosis of SS and peripheral neuropathy, seen between 2010 and 2016 were analyzed. Clinical records, conventional nerve conduction studies, and lip and nerve biopsy reports were collected. RESULTS: Twenty one patients with SS had associated neuropathy. Female-to-male ratio was 2:1. In 14 (66.7%) patients, neuropathy was the initial manifestation, while in 4 (20%), exocrinopathy preceded neuropathy. The patterns of neuropathy included mononeuropathy multiplex (MNM) in 7 patients (30%), ganglionopathy in 4 (20%), length-dependant trigeminal autonomic neuropathy, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in 2 (10%), and cranial neuropathy in 1 (10%). Eighteen (86%) were seropositive with either anti Ro/SS-A or anti La/SS-B antibodies. Schirmer's test was positive in 13 (61.9%) patients. Nerve biopsy showed vasculitis in 5 patients and demyelinating and axonopathy in 2 patients each. CONCLUSIONS: We conclude that neuropathy is frequently the initial presentation of SS. MNM is the most common pattern followed by ganglionopathy. The pattern of neuropathy helps in arriving at the diagnosis of SS. Serology is a useful initial laboratory test. However,confirmation of SS is not by mere serology. Schirmer's test and lip biopsy are equally essential for the diagnosis, especially in seronegative patients when the clinical index of suspicion is high.

A Series of Biopsy-proven Patients with Immunoglobulin G4-related Neurological Disease.

AIM: To study the clinical presentation, radiological findings, and therapy responsiveness of patients with biopsy-proven immunoglobulin G4 (IgG4)-related neurological disease. METHODS: The study was conducted between January 2016 and March 2018 from the Department of Neurology and Pathology of Nizam's Institute of Medical Sciences. Patients with neurological symptoms and biopsy suggestive of IgG4-related disease (IgG4-RD) were included. These patients were studied for their demographic pattern and clinical presentation. The presence of serological markers such as vasculitic profile and IgG4 levels was analyzed. Radiological findings were studied in detail. Therapeutic agents used and the response to therapy were assessed. RESULTS: There were six cases with IgG4-related neurological disease which were all hypertrophic pachymeningitis. The age ranged from 35 to 64 (mean = 46) years. The clinical presentation was acute in one, subacute in two, and chronic in three patients. The most common presenting symptom was headache (4), followed by gait and/or urinary disturbances (2), paraparesis (1), and diplopia (1). IgG4 levels were elevated in 50% of them. Pseudotumor-like mass and sinovenous thrombosis, not described previously, were seen in one patient. All the patients were treated with oral or intravenous steroid. Rituximab was given in three patients; azathioprine was the steroid-sparing agent in one patient. Those with acute/subacute onset of presentation had an excellent response to steroids. All the patients with a chronic duration of their symptoms received empirical anti-tuberculous therapy before a definitive diagnosis of Ig G4-RD was made. CONCLUSIONS: The characterization of patients with IgG4-related neurological disease based on the understanding of the clinical spectrum increases the confidence in the clinician to resort to early immunosuppression, thereby having prognostic implications.

Longitudinally Extensive Transverse Myelitis Due to Toxoplasma: An Autopsy Study.

Toxoplasma is an obligate intracellular parasite that remains asymptomatic in humans but, at times, can cause devastating disease. Here, we describe an autopsy study of a young immunocompetent gentleman with no comorbidities whose presentation was acute transverse myelitis. Magnetic resonance imaging spine showed longitudinally extensive spinal cord lesion (LESCL) that mimicked neuromyelitis optica with normal brain imaging at presentation. Investigations showed albuminocytological dissociation which prompted a course of parenteral steroid. However, the lesion relentlessly progressed to involve the brain stem and cerebrum leading to toxoplasmic encephalitis that terminated fatally. This report highlights that toxoplasma can present as LESCL and needs to be considered in the differential diagnosis of atypical myelitis.