Jacobs Journal of Clinical Case Reports

Complex Dysexecutive Syndrome and Agonistic Dyspraxia in a Patient with Bullous Pemphigoid

Published on: 2016-02-19


We are presenting a difficult diagnostic clinical case of a patient who developed a complex dysexecutive and disconnection syndrome with a rare form of hand apraxia, and bullous pemphigoid (BP). The differential diagnosis included a subacute toxic leukoencephalopathy and autoimmune encephalitis. The patient had a moderate exposure to propane-butane/CO, caused by a gas leak from a faulty household appliance. Reversible diffuse leukoencephalopathy with delayed evolution and toxic-hypoxemic etiology was our final diagnosis. It was made after a detailed study of our patient’s medical history, and the extensive examinations conducted simultaneously. The possibility of autoimmune antibody-mediated non-vasculitic encephalitis, during severe BP, was excluded with serum, CSF, and antinuclear antibodies examination. The treatment assigned was non-specific, leading the patient to a very good recovery after a six-month period. Some of the key elements that helped us to identify the correct diagnosis and consequently the appropriate therapy were: 1) Time-led graphic model of all the changes occurring throughout the development of his condition, providing us with a specific visual time-frame; 2) Analysis of the nonspecific brain MRI white matter changes; 3) The unique pattern of hand dyspraxia. This is a case of a rare primary neurological pathology and secondary skin involvement with BP. It also illustrates a favorable recovery after a broad white matter injury with a corpus callosum disconnection which caused a neuropsychiatric disorder and agonistic dyspraxia (AD).


Agonistic Dyspraxia; Bullous Pemphigoid; Toxic-Hypoxemic Leukoencephalopathy.