Biomedical subjects
Publications and source records attributed to Sławomir Budrewicz.
The authors described a case of young patient with primary central nervous system lymphoma (PCNSL). Unspecific initial symptoms and clinical course, as well as neuroimaging findings caused many diagnostic difficulties. The diagnosis of PCNSL was finally established on the basis of stereotactic brain biopsy, after 18 months of follow-up.
In patients with extrapyramidal diseases speech disturbances (dysarthria) are frequent. Dysarthria could have hypokinetic-hypertonic or hyperkinetic-hypotonic pattern. The frequency of them hesitates from 70 to 100% in patients with extrapyramidal symptoms. Clinimetric analysis of type and severity of dysarthria could be done by means of different scales useful in extrapyramidal diseases; Webster--Score Disability Rating Scale, Columbia Rating Scale or North Western University Disability Scale. The results obtained in these scales are subjective. Computer speech analysis could be useful in identification of speech disturbances. The waveforms and spectograms are used in assessment of speech intensity and frequency. The authors indicated to the usefulness of computer acoustic analysis in evaluations of speech disturbances in patients with extrapyramidal diseases as a repeatable and precise method.
Oromandibular dystonic movements may present a type of focal or segmental dystonia. They often cause the malnutrition problems and social isolation. The aim of the study was to evaluate the efficacy of Botulinum Toxin type A treatment in patients with oromandibular dystonia with estimation of nutrition status. 15 patients with oromandibular dystonia, 9 women and 6 men, mean age 56 years old, were investigated. In all patients Botulinum Toxin type A was administrated intramuscularly under the EMG control. In 14 patients the improvement was significant and in 11 increase body mass was observed within 6 month after injections. Botulinum Toxin type A is useful in treatment of oromandibular dystonic movements in patients with malnutrition.
Cortico-basal degeneration is a rare degenerative disease connected with Tau protein pathology. Epidemiology of cortico-basal degeneration is unknown. The authors present a case of 59 years old woman with suspicion of cortico-basal degeneration. The extrapyramidal symptoms mainly on the right side with "alien limb phenomenon" and dystonia of lower limb is observed in our patient. Cortico-subcortical brain atrophy was present in MRI scans. EEG was asymmetrical. No improvement was noticed after L-Dopa. Treatment of amantidine caused the transient improvement.
Authors present 6 cases of dopa responsive dystonia (DRD) with delayed diagnosis. DRD was firstly described by Segawa et al. in 1976, but in 90's its genetic linkage to chromosomes 14 and 11 was proven. It resulted in possible effective treatment (substitution with low dosages of levodopa). Nevertheless, the correct diagnosis is difficult. DRD usually starts as a foot dystonia with gait difficulties and subsequent overflow of dystonic movements to other muscles and parts of the body, with parkinsonian features and pyramidal signs in part of cases. Diurnal fluctuations with deterioration in the evening are typical for DRD. Dystonia starting in lower extremities may be misdiagnosed with cerebral palsy, what may have consequences in surgical interventions (as in our two cases). Authors present diagnostic difficulties in 6 cases with diagnosis made after 5 to almost 30 years after onset of symptoms and with several surgical interventions performed in 2 cases. The trial with levodopa is crucial for diagnosis and treatment, because low dosages are effective and there are no typical for juvenile parkinsonism treatment complications as dyskinesias and/or fluctuations. The take home message from literature and presented material should be not age dependent clinical trial with levodopa therapy in all cases of dystonia starting in extremities.