Atypical idiopathic NBIA (neurodegeneration with brain iron accumulation) associated with treatment-resistant bipolar mania responding to clozapine.

Abstract

Management of treatment-resistant bipolar mania has not been discussed much in the literature. The underlying degenerative neurological disorders could be one of the reasons for treatment resistance. A 27-year-old single male presented with 13 years of episodic illness characterised by typical manic symptoms with poor interepisodic recovery. His neurological examination was normal. Most of the commonly used antipsychotics, mood stabilizers and their combination failed to sustain the response to manic symptoms, including when psychotropics were combined with electroconvulsive therapy. Severe adverse effects such as pancreatitis with valproate and hyponatremia with oxcarbamazepine further challenged the management of treatment-resistant bipolar mania. Radiological evaluation revealed the presence of brain mineralisation in bilateral dentate, substantia nigra, red nuclei, globus pallidi and posterolateral putamen. Based on the clinical and radiological findings, a possibility of atypical idiopathic Neurodegeneration with Brain Iron Accumulation (NBIA) associated with treatment-resistant bipolar mania was made. Clozapine was initiated and titrated up to 300mg. Clinically significant improvement was seen in manic symptoms, and the response was sustained over two years. The presence of atypical idiopathic NBIA could be one of the factors for treatment resistance in bipolar mania. Clozapine has a good response in treatment-resistant bipolar disorder and can be effective even in the presence of the underlying neurodegenerative condition.