Recurrent Regional Autonomic Dysfunction as a Presentation of Relapsing Neuromyelitis Optica Spectrum Disorder, Responsive to Ketamine Infusion.

Diagnostic criteria of neuromyelitis optica spectrum disorders (NMOSD) have expanded to include many new symptoms since the original description of Devic's disease in 1894, and the formulation of diagnostic criteria in 2006. Herein, we describe a patient with serum aquaporin positive NMOSD with the unusual presentation of recurrent regional autonomic dysfunction, with concordant MRI findings. The symptomatic treatment of the patient's causalgia required IV ketamine infusion. Besides, as the long segment myelitis occurred in the setting of extensively drug-resistant tuberculosis (XDR TB), there was the added challenge of ruling out TB myelitis and choosing an immunosuppressant that would have the least risk of reactivation of TB.

Acrocallosal syndrome in a young hypertensive male.

Acrocallosal syndrome is an extremely rare genetic disorder with autosomal recessive inheritance. It is characterised by moderate to severe mental retardation, hypotonia, agenesis of the corpus callosum and preaxial polydactyly involving both feet and the facial features like broad forehead and hypertelorism. The authors report a case of a young hypertensive male who presented with unprovoked seizures for the first time who had multiple craniofacial, digital dysmorphic features with moderate mental retardation. The diagnosis of acrocallosal syndrome was arrived at after neuroimaging showed agenesis of corpus callosum with interhemispheric cysts.

Xanthogranulomatous pyelonephritis in a young postpartal female.

Xanthogranulomatous pyelonephritis (XGP) is a rare chronic inflammatory disorder of the kidney characterised by an infectious phlegmon arising in the renal parenchyma. It is seen in patients who have urolithiasis, urinary tract infection and immunocompromised status. The clinical presentation is variable and renal neoplasm is considered as a differential due to its characteristic extrarenal visceral invasion. The treatment is almost universally extirpative and can pose a formidable challenge to the treating physician and surgeon. The authors report a rare case of XGP in a postpartal woman who presented with multiple visceral abscesses whose diagnosis was arrived on histopathological examination.