Complex Dysexecutive Syndrome and Agonistic Dyspraxia in a Patient with Bullous Pemphigoid
*Marko G Department Of Neurology, Bulgaria
*Corresponding Author: Marko G
Department Of Neurology, Bulgaria Email:email@example.com
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).
The development of delayed and reversible white matter leukoencephalopathy (WMLEP) with a wide spectrum of personal, cognitive, and motor disturbances is a common observation following toxic-hypoxemic incidents [1-4]. It could be caused by a variety of toxins, therapeutic or illicit drugs . The occurrence of BP followed by an immune-mediated encephalitis due to a CO exposure, however, were very unusual observations [6, 7]. BP is one of the most common autoimmune subepidermal blistering diseases [6, 8, 9]. It can be associated with different neurological conditions, stroke, multiple sclerosis, dementia, parkinsonism, and epilepsy [8-11]. It was rarely reported in association with antineuronal antibody-mediated encephalitis [7,10,11]. Agonistic dyspraxia (AD) as a specific type of “alien hand” syndrome is also rare but well-described disorder observed in patients having corpus callosum disconnection, parietal and frontal lesions with variable etiology [12,13]. Evolution and therapy of different cases of AD and complex cognitive deficit after toxic-hypoxemic encephalopathy have not been well studied.