Maria Pąchalska, Bogusław Frańczuk, Tadeusz Gałkowski, Bożena Grochmal-Bach, Henryk Knapik, Ewa Gostyk, Bruce MacQueen, Adam Pąchalski, Piotr Pąchalski, Rafał Stabrawa, Irena Jeleńska-Szyguła

Maria Pąchalska, Bogusław Frańczuk, Tadeusz Gałkowski, Bożena Grochmal-Bach, Henryk Knapik, Ewa Gostyk, Bruce MacQueen, Adam Pąchalski, Piotr Pąchalski, Rafał Stabrawa, Irena Jeleńska-Szyguła – Therapy for limb apraxia in a patient with the Melas syndrome. Fizjoterapia Polska 2001; 1(1); 26-38

Abstract
Introduction. The authors report the results of four years of research and therapy of apraxia occurring in a patient with a mitochondrial cytopathy known as the MELAS syndrome, which causes heterogeneous organic changes in the brain. This is an original contribution, since the clinical literature contains no references to apraxia therapy in MELAS. Slowly progressive apraxia of the upper and lower limbs is described, and the rehabilitative potential of a specially developed authorial program entitled “Semantically Controlled Patterns of Motion” (referred to as “SSWR” from the Polish abbreviation) is measured.Material and methods. The patient, K.S., female age 22, diagnosed with MELAS, is under treatment in the Department of Medical Rehabilitation at the Cracow Rehabilitation Center. The extraordinarily complicated clinical picture of her illness (one of the mitochondrial cytopathies) consists of episodic exacerbation overlaid on a patient of slowly progressive symptoms, including dementia, fluctuating aphasia, tactile agnosia, sensorineural deafness, cortical blindness, and slowly progressive ideomotor apraxia. The holistic therapy program involves the indicated pharmacological treatment and comprehensive neurorehabilitation aimed at the polymodal reintegration of higher cortical functions, including voluntary limb movements. The SSWR program is comprised of an authorial set of patterns of limb motion involving both gross and fine movements, along with descriptive humorous names for the individual patterns, in order to support the process of remembering and recalling, since the patient exhibits cognitive disturbances. The effectiveness of the therapy program developed for this patient was assessed by means of standard neuropsychological tests, including the Boston Test of Praxis and the Test of Activities of Daily Life (ADL). Results. It was found that despite the continued development of the illness as measured by the results of MRI examinations, the patient achieved considerable progress in respect to conscious control of limb movements, as indicated by significant improvement in the results from successive administrations of the Boston Test of Praxis. Improvement in general ability to adapt as well as emotional and social functioning, is indicated by the results from standard neuropsychological tests and from the Test of Activities of Daily Life (ADL). Discussion. The authors present the theoretical foundations of the program. Imitating a movement requires that a mental representation of the intended movement be evoked in the brain, after which a motor program (in the seanse of praxis) is formulated and executed. The significance of this discovery for contemporary physiotherapy will be demonstrated.

Key words:
MELAS syndrome, limb apraxia ideomotor apraxia, patterns of motion, neurorehabilitation

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