Therapy for limb apraxia in a patient with the Melas syndrome

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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The neurorehabilitation of hemispatial neglect in patients with traumatic brain injury

Maria Pąchalska, Bogusław Frańczuk, Jan Talar, Wiesław Tomaszewski, Bruce MacQueen, Kay Neldon

Maria Pąchalska, Bogusław Frańczuk, Jan Talar, Wiesław Tomaszewski, Bruce MacQueen, Kay Neldon – The neurorehabilitation of hemispatial neglect in patients with traumatic brain injury. Fizjoterapia Polska 2001; 1(2); 143-154

Abstract
Background. The purpose of this article is to evaluate the relative effectiveness of differentiated rehabilitation programs for patients with hemispatial neglect subsequent to traumatic brain injury (TBI). In the literature, neglect is classified into two basic types: egocentric (“body-centered”), in which the patient neglect object situated in a given area of his field of vision, and allocentric )”objected-focused”), in which the patient neglects one side of objects presented to him, regardless of their location. The authors’ clinical practice indicates that the visual scanning technique traditionally used in neglect therapy is generally ineffective in patients with egocentric neglect. A spatio-motor training program has therefore been developed by the authors, elaborated on the basic of their program of semantically controlled motion patterns for the rehabilitation of apraxia, whose goal is to reestablish the proper relations between automatic and voluntary movements, to improve interhemisphere cooperation by symmetrical and asymmetrical modeling of motor exercises with both healthy and affected limbs, and to increase patient motivation by establishing clearcut goals and rewarding success. Both programs, visual scanning and spatio-motor training, have been implemented in clinical practice in the represented by the authors. Material and methods. The article describes in detail the rehabilitation of two TBI patients (JK, male, age 27, and AS, female, age 28) treated by the authors in the chronic phase of recovery after automobile accidents, as a result of which both patients were comatose for more than 4 weeks. In neuropsychological testing JK and AS both presented with persistent and significant leftsided hemispatial neglect: allocentric neglect in JK, and egocentric in AS. A modified AB-BA experimental design was used, where A represents spatio-motor training, and B is visual scanning. For patient JK, program A was first applied, followed by B, while for patient AS the reverse sequence was used. Each program included 10 therapy sessions, with a four-week interval between programs. The degree of neglect was measured using standard neuropsychological tests for quantitative and qualitative assessment of neglect, along with the analysis of drawings made during therapy by both patients. The patients were tested four times, before and after each of the two programs. Results. As the authors had hypothesized, patient JK did not show any progress in terms of neglect after conclusion of program A, while after program B he showed virtually no lingering neglect. In the case of patient AS the results were exactly opposite: after concluding program B (which she received first) there was no significant change, while after program A (administered second) there were no signs of neglect.Conclusion. The theoretical and clinical implications of these results for neuropsychological practice are discussed, and a model solution is proposed to explain the basic pathomechanism of both allocentric and egocentric neglect. These two types of neglect are sufficiently different in etiology and symptomatology to necessitate differentiated therapy programs in the course of neurorehabilitation.

Key words:
hemispatial neglect, traumatic brain injury, rehabilitation

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