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Background: Porphyrias are a group of inherited metabolic disorders, resulting from the deficiency of specific enzyme in the hemebiosynthesis. Among these, acute intermittent porphyria (AIP) is the most common type with neuropsychiatric features encountered in the clinical practice. In this study, we describe the presentation of AIP in five different patients admitted in our hospital. Materials and Methods: We prospectively collected the data of five patients of AIP admitted in our department in the past 2 years and studied in detail the causes, precipitants, clinical presentation, diagnosis, and management of AIP. Results: All five cases were males in second to third decades of life having a history of prior gastrointestinal symptoms for variable period before presenting to us with neurological manifestations of which one had abdominal pain for 2 years and had undergone appendicectomy also. All patients had seizures and psychiatric manifestations. Four patients had axonal polyneuropathy and respiratory failure requiring prolonged ventilator support. One patient had a past and family history of AIP. Sepsis as a trigger is noted in two patients. All patients had positive urine test for porphobilinogen. All had autonomic dysfunction, hyponatremia, and three patients expired after prolonged intensive care unit stay of which two had status epilepticus. Two patients survived with complete recovery and are under regular follow-up. Conclusion: Porphyria screening should be done in all patients presenting with unexplained motor neuropathy. Avoidance of triggers, early suspicion of the acute attack, and starting treatment immediately will prevent the complications and thus reduce morbidity and mortality due to the acute attack.

Introduction: Stroke is the second most common cause of death worldwide after ischemic heart disease Socioeconomic burden of stroke can he reduced by proper treatment of modifiable risk factors and maintaining adherence to treatment. The aim of this study is to identify factors associated with non-adherence to treatment of modifiable risk factors in elderly stroke patients. Methods: Prospective observational study with 100 acute ischemic stroke patients admitted in Neurology department. Government Medical College Kota between June to September 2018. Risk factors for stroke and reasons for non-adherence to anti-hypertensives, anti-diabetics and antiplatelet agents were studied in detail. Results: Various modifiable risk factors for stroke present in study subjects were smoking (35%), alcoholism (16%), hypertension (82%) and diabetes mellitus (23%). Past history of stroke was present in 14% cases. Total 59 patients were non-adherent to treatment. Reasons for non-adherence include poor knowledge (47%), superstitious belief (32%), intentional withdrawal (25%), due to miscommunication (12%), pill burden (7%), side effects (3%), pill size (1%) and poor family care(l%). Conclusions: Understanding of various factors associated with medication intake and creating awareness among patient could help in medication adherence.

Leprosy has high incidence in tropical and subtropical countries. We document a case of 26-year-old female with an 18-month history of insidious onset progressive weakness and wasting of small muscles of both hands with decreased sensation distal to elbows and trophic changes without thickened nerves. The patient was on anti-leprosy treatment for the past 1 year with no response to treatment. Examination revealed diminished reflexes in the upper limbs and preserved reflexes in the lower limbs. Skin biopsy was normal. Magnetic resonance imaging of the spine was suggestive of Arnold–Chiari malformation type I with large cervico-dorsal syrinx which was ultimately treated by the surgical management. We suggest that all cases of leprosy should include syringomyelia as a differential diagnosis so that unnecessary prolonged exposure to anti-leprosy drugs with potential side effects can be prevented.