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Biomedical subjects

J Bojakowski

Publications and source records attributed to J Bojakowski.

12 recordsLinked to original sources

A double-blind randomised placebo-controlled evaluation of three doses of botulinum toxin type A (Dysport) in the treatment of spastic equinovarus deformity after stroke.

BACKGROUND/OBJECTIVES: Calf muscle hypertonicity following stroke may impair walking rehabilitation. The aim of this study was to assess botulinum toxin (Dysport) in post-stroke calf spasticity. METHODS: A prospective, multicentre, double-blind, placebo-controlled, dose-ranging study was performed to evaluate dysport at 500, 1,000 or 1,500 units in 234 stroke patients. They were assessed at 4-week intervals over 12 weeks. RESULTS: The primary outcome measure, 2-min walking distance and stepping rate increased significantly in each group (p < 0.05, paired test), but there was no significant difference between groups (including placebo). Following dysport treatment, there were small but significant (p = 0.0002-0.0188) improvements in calf spasticity, limb pain, and a reduction in the use of walking aids, compared to placebo. Investigators' and patients' assessments of overall benefit suggested an advantage for dysport over placebo, but this was not significant. Sixty-eight patients reported 130 adverse events, with similar numbers in each group. The few severe events recorded were not considered to be treatment-related. CONCLUSION: Dysport resulted in a significant reduction in muscle tone, limb pain and dependence on walking aids. The greatest benefits were in patients receiving dysport 1,500 units, but 1,000 units also had significant effects. Dysport 500 units resulted in some improvements. Since few adverse events were reported, this therapy is considered safe and may be a useful treatment in post-stroke rehabilitation of the leg. Possible reasons why functional improvements in gait parameters were not observed are also discussed.

Adult↗

[Corticospinal tract assessment in ALS: transcranial magnetic stimulation].

Results obtained during transcranial magnetic stimulation (TMS) in 79 patients with ALS were compared to those obtained in 10 healthy volunteers. M and F responses evoked by peripheral stimulation of the ulnar and peroneal nerves and then motor evoked potentials (MEP) induced by TMS were recorded in the abductor digiti quinti (AV) and tibial anterior (TA) muscles bilaterally. Central motor conduction time (CMCT) was calculated using MEP and F response latencies. The MEP/M x 100% amplitude ratios were calculated. Mean CMCT was significantly (p < 0.0001) prolonged in patients with ALS (9.6 +/- 4.1 ms for AV and 20.4 +/- 9.6 ms for TA) and MEP/M x 100% amplitude ratios were higher (45 +/- 75% for AV-p < 0.001 and 27 +/- 32.7% for TA p = 0.2). In a sub-group of patients with predominant upper motor neuron (UMN) involvement, the CMCT was significantly prolonged (14.2 +/- 5.8 ms for AV and 28.5 +/- 9.1 ms for TA), but MEP/M x 100% amplitude ratio was 24.1 +/- 37.6% for AV and 11.4 +/- 12.9% TA. These results confirm usefulness of combined analysis of CMCT and MEP/M x 100% amplitude ratio as the method of assessment of pyramidal tract function in ALS.

Adult↗

Dystrophinopathies in females.

Various laboratory tests were performed to establish carriership in 24 familial and sporadic carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The activity of creatine kinase was in all females but one, very high and significantly higher in isolated carriers; quantitative EMG indicated myopathic changes, muscle biopsies revealed different degrees of changes--from a variability of muscle fibers size and central nuclei to severe dystrophic features. Immunohistochemical evaluation of dystrophin revealed, in all females but one, mosaic pattern of staining--a mixture of dystrophin-positive and dystrophin-negative fibers, the latter consist 15-30% of all fibers. Quantitative evaluation of dystrophin showed a reduced abundance with normal or abnormal molecular weight. The abnormalities were more expressed in sporadic cases. The detection of sporadic carriers, particularly the non-manifesting clinical, is a very important progress--it permits the correct diagnosis (before, these females were diagnosed as limb girdle muscle dystrophy (LGMD) and supply them with the benefit of genetic counselling, which also requires some modification.

Adolescent↗

Coexistence of various vascular malformations within the brain.

Authors present two cases of basilar artery aneurysm accompanied by different development failures of blood vessels. In both cases anomaly in formation of brain base vessels, angioma consisted of different size thin-walled vessels and arterio-venous angioma within brain stem were stated. Besides, conglomerates of abnormal vessels, angiosis within pia matter, diffused lacunar and fetal as well as thin-walled venous vessels were found. Pathological vessels, their conglomerates were present in brain stem, cerebellum and cerebral hemispheres. The variability of vascular malformations seems to point at long-lasting action pathogenic factor during ontogenesis. Authors try to refer particular developmental anomalies to proper stage of ontogenesis.

Aged↗

Features of motor control in patients with proximal childhood spinal muscle atrophy (pilot study).

The differences in the motor performance during different tasks between 19 subjects suffering from SMA and 10 healthy controls were observed. The simultaneous EMG activity of twelve lower limbs and lower trunk muscles was recorded with surface electrodes. EMG data were automatically reduced and compared with data evaluated from performed by physiotherapist manual testing of muscle strength. Results showed characteristic differences between healthy and spinal muscular atrophy (SMA) subjects: 1. SMA patients display generally more activity occurring in numerous muscle groups and more spinal levels are activated. 2. SMA patients reveal a disturbed functional relation between the posterior and anterior compartments of muscles. 3. EMG activity in SMA patients is spreading out also to the contralateral muscle groups even during slight, unilateral singlejoint movements. Oligosegmental, plurisegmental and brain sources are probably responsible for mentioned phenomena. The reciprocal influences between reduced number of motoneurons (in SMA) and function of central movement generators results in different mode of movement execution in SMA patients.

Adolescent↗

Familial hemifacial spasm.

We present a family in which hemifacial spasm involving in all cases the left side of the face occurred in five persons in three generations. Blink reflexes recorded in two cases demonstrated an unexpected R1 component on the affected side during stimulation of the contralateral side.

Adult↗

[F-wave studies in spinal muscular atrophy].

For checking the hypothesis on increased excitability of the spinal motor neurons in patients with spinal muscular atrophy the F wave was analysed in 12 patients. The hypothesis has been confirmed, but increased excitability of motor neurons is not equally pronounced in all motor nuclei of the spine.

Adolescent↗

[Familial cerebellar ataxia: clinical, radiological and electrophysiological findings].

A family with cerebellar ataxia of late onset occurring in four generations was observed. Neurological abnormalities included signs of cerebellar ataxia, pyramidal tract damage and damage to the peripheral motor neuron. Computerized tomography demonstrated in five out of six studied patients an image suggesting olivo-ponto-cerebellar atrophy. In the cerebellar structures, brainstem and cerebral hemispheres evidence of atrophy was detected. No correlation was demonstrated between the intensity of the clinical signs and the progression of changes in CT image. Electrophysiological investigations demonstrated changes compatible with damage to the motor and sensory fibres in the peripheral nerves and signs suggesting damage to the spinal motor neurons and pyramidal tract. These observations confirm the multilevel development of the process. The use of similar diagnostic methods will permit a more accurate classification of cerebellar ataxia and obtaining of better information for prognostication of individual cases.

Adult↗

[Syndrome of amyotrophic lateral sclerosis after thymectomy performed for myasthenic syndrome].

A case of amyotrophic lateral sclerosis was observed developing three months after thymectomy in a woman aged 52 years. The patient had been referred for thymectomy because of myasthenic signs increasing in intensity since 2 years. Myasthenia was confirmed by electrophysiological investigations, and the diagnosis was: myasthenic syndrome and suspected thymoma. During nearly 5 years of follow-up the following observations were made: there was a correlation between thymectomy and the development of ALS syndrome, thymosine administration produced clinical improvement, thymosine administration improved the function of thymus-dependent lymphocytes, thymosine withdrawal (due to non-availability) produced exacerbation of symptoms.

Amyotrophic Lateral Sclerosis↗