Objective:

Describe a case of Manganism encountered in Zambia.

Background:

Manganism is a neurodegenerative syndrome due to manganese toxicity and its excessive accumulation in the brain.

Design/Methods:

Case presentation

Results:

A 37-year-old man presented with a four-month history of mild, intermittent headache and three-month history of chest pain, dry cough and symmetrical distal-predominant paresthesias. He developed progressive cognitive decline associated with slowness in talking, low volume speech and difficulty completing activities of daily living two months prior to presentation. He reported fever and night sweats. Past medical history was unremarkable. Social history only notable for working as a manganese smelter for the past eight years.

Neurological exam revealed memory and language deficits with psychomotor slowing, subtle left upper motor neuron facial weakness, hypomimia, bradykinesia and had a hyperkinetic tongue and upper limb tremor with normal tone and power in all limbs. Deep tendon reflexes were normal, but frontal release signs and an applause sign were present. Small and large fiber length-dependent sensory loss was noted with a cock-walk gait.  MRI brain with gadolinium showed non-enhancing symmetric T1 hyperintensities in the globus pallidum and midbrain with corresponding T2 hypointensities in the same areas.

Conclusions:

A diagnosis of Manganism was made based on clinical history, strong occupational exposures, and neurological examination and imaging findings. Blood manganese levels and other heavy metals could not be obtained due to unavailability of tests in our setting, although blood manganese levels are often normal when obtained in cases of Manganism. The cough, headache, chest pain and fever were ascribed to metal fume fever and improved after a three-month withdrawal of manganese exposure, though neurologic symptoms did not improve during this period. This case demonstrates the importance of thorough history (including social history), examination, and correlation with limited investigations in diagnosing even rare diseases in resource-limited settings.