Autonomic dysfunction in a welder following manganese toxicity: a case report

Background: Manganese induced autonomic dysfunction is one of the chronic debilitating entities known to be seen primarily in welders exposed to fumes at work place. Welders absorb these fumes via inhalational route. Manganese in these fumes gets deposited in the basal ganglia and cause neuronal loss predominantly in Globus Pallidus. Unfortunately, it is under diagnosed due to lack of awareness of the disease. Though path breaking researches have been carried out in the field of Parkinson’s disease (being main differential diagnosis of manganese toxicity), still very few advancements in the management of manganese toxicity is seen and only supportive therapy is helpful. Primarily, this autonomic dysfunction does not or poorly respond to levodopa which is a characteristic distinguishing factor from Parkinsonism. Case: We are describing a case of a 50-years male presenting with complaints of slowness in the movement, gait instability with frequent falls. The patient has had persistent orthostatic hypotension. He had no significant family history and his work up showed no other cause of autonomic dysfunction. The patient had poor response to Levodopa. He had been exposed to welding fumes for 25 years. His MRI showed non-specific demyelination foci while SPECT scan was normal. The patient was diagnosed with occupational manganese toxicity based on history and Canadian consensus guidelines. The patient improved with oral fludrocortisone and bilateral lower limb elastic stockings. Conclusion: Large scale promotion on the use of Personal Protective equipment (PPE) is required. Early intervention in these cases could save the patient from detrimental effect, decrease social economic burden and make better health related quality of life as only supportive care can be given once diagnosed

Pulmonary choriostoma in a case of tuberous sclerosis complex

A 52 years old lady was diagnosed to have Tuberous Sclerosis Complex (TSC) on the basis of 2 major and one minor criterion. She had family history of similar complaints in her sister and two sons. There was involvement of kidney in the form of angiomyolipoma, skin in the form of facial angiofibroma and teeth with a dental pit. She had an unusual lung involvement in the form of multiple small choristomas. Choristoma was diagnosed on transbronchial lung biopsy and was present in the form of disorganised striated muscles. The reported pulmonary manifestations of TCS i.e. lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH) are types of hamartomas. Hamartomas and choristomas are both types of disorganized tissue. ′Choristoma′of lung in TSC however is not reported. Clinopathological correlation of pulmonary hamartoma and choristoma, and treatment in TSC has been discussed