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.
:Infarction of the basal ganglia after minor head injury �is a� well described entity .�Mineralising angiopathy� is a term to describe mineralisation along� the lenticulostriate vessels that predisposes infants to stroke,even after trivial head injury.(1)The prognosis in these children is excellent.The supratentorial compartment is relatively mobile in comparision to the fixed infratentorial compartment �offering greater shearing forces between the perforating vessels and the brain parenchyma following �an acute trauma.(2)The acute angulation of the lenticulostriate perforators is further responsible for its propensity of the basal ganglia for the insult.(3) The current report is unique as it presents a young boy with hemorrhagic stroke in the�� basal ganglia after a trivial trauma and �imaging findings suggestive of mineralising angiopathy
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.
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