Andrzej Bogucki

Introduction: Full-thickness rotator cuff tear is present in almost 50% of patients over age 65 years, and its degree is known to be a good predictor of the severity of muscle-wasting (MW) sarcopaenia, also known as fatty degeneration (FD). A FD CT grade > 2° is recognized as a borderline of...

Introduction. The effectiveness of the currently utilized therapies for FoG is limited. Several studies demonstrated a beneficial impact of Nordic walking (NW) on several gait parameters in Parkinson’s disease, but only one paper reported reduction of freezing.Research Question. In the present study, the question is whether NW is an effective therapeutic intervention in FoG.Methods. Twenty PD subjects trained NW for 12 weeks, with a frequency of twice per week. Each session lasted about 60 minutes. Twenty patients in the control group did not use any form of physiotherapy (no-intervention group). Freezing of Gait Questionnaire (FOGQ), the Timed Up and Go (TUG) test, and the Provocative Test for Freezing and Motor Blocks (PTFMB) were performed at baseline, immediately after the end of NW program, and three months later.Results. The results of FOGQ, TUG, and total PTFMB revealed significant improvement after completing the exercise program, and this effect persisted at follow-up. The results of the PTFMB subtests showed a different effect of NW on particular subtypes of FoG. Start hesitation, sudden transient blocks that interrupt gait, and blocks on turning improved considerably, while motor blocks, when walking through narrow space and on reaching the target, did not respond to NW training.Significance. The results show, for the first time, that FoG during turning and step initiation, two most common forms of this gait disorder, has been significantly reduced by NW training. Different responses of particular subtypes of FoG to NW probably reflect their different pathophysiologies.Conclusions. The present study showed that NW training had a beneficial effect on FOG in PD and that the achieved improvement is long-lasting. Future research should clarify whether the observed improvement limited to FoG triggered by only some circumstances reflects different pathomechanisms of FoG subtypes.

Holmes’s tremor (HT) is assumed to be the result of coexistence of nigrostriatal dopaminergic system impairment and the lesion of cerebello-thalamic pathways. It was suggested that dopaminergic deficiency is responsible for rest tremor, and lack of compensatory cerebellar function leads to spill of tremor into voluntary movements. Cases of HT with and without abnormalities of the presynaptic part of dopaminergic nigrostriatal were published and these findings raised the question of possibility of the postsynaptic lesion. Three patients with HT diagnosed according to criteria of Consensus Statement on Tremor were studied. In all of them SPECT imaging with ligands of presynaptic (I 123-FP CIT—DaTSCAN) and postsynaptic (I 123-iodobenzamide—IBZM) nigrostriatal dopaminergic neurons was performed. Indices of uptake in caudate and putamen normalized to nonspecific uptake in occipital cortex and indices of asymmetry for each whole striatum as well as for putamen and caudate separately were calculated. SPECT studies did not reveal asymmetry of DaTSCAN and IBZM binding in striatum in all studied subjects. The current clinical diagnostic criteria of HT are presumably insufficiently specific and when using them we identify patients both with and without the involvement of dopaminergic system. These two groups may represent tremor disorders of similar phenomenology but of different pathomechanism.

Progressive muscular atrophy (PMA), or the lower motor neuron disease, is a sporadic disorder characterized by onset in adulthood, pure lower motor neuron involvement and relatively benign course. Muscle atrophy and weakness may be symmetrical or asymmetrical, but they are always bilateral. We present a male patient with exclusively left-side flaccid paresis due to lower motor neuron disease without electromyographic evidence of neurogenic lesion of contralateral muscles and with no signs of corticospinal tracts involvement. The rapid disease progression was typical of the generalized phenotype of PMA and it suggested the relation to the aggressive course of classical ALS.

Apomorfina jest najsilniejszym agonistą dopaminergicznym, o skuteczności porównywalnej z lewodopą. Po podskórnym podaniu wchłania się szybko i całkowicie. Maksymalne stężenie w surowicy osiąga po 5–15 minutach, a efekt kliniczny ujawnia się po 20 minutach. Pojedyncze podskórne wstrzyknięcia apomorfiny są skuteczne jako leczenie doraźne przerywające nieprzewidywalne stany off w zaawansowanej chorobie Parkinsona. Obecnie apomorfina częściej stosowana jest w postaci ciągłych wlewów podskórnych, co zapewnia ciągłą stymulację dopaminergiczną. Wyniki badań zgodnie wskazują na skuteczność tej terapii w odniesieniu do stanów off, jednak są mniej jednoznaczne w przypadku dyskinez. Dwie terapie infuzyjne (apomorfina i dojelitowa lewodopa) oraz głęboka stymulacja mózgu są skuteczne w zaawansowanej chorobie Parkinsona z opornymi komplikacjami ruchowymi. Wlewy apomorfiny powinny być brane pod uwagę u chorych, u których zastosowanie głębokiej stymulacji mózgu nie jest możliwe ze względu na zaburzenia funkcji poznawczych i przeciwwskazania neurochirurgiczne. Apomorphine is the most potent dopamine receptor agonist and its symptomatic effectiveness is comparable to levodopa. Subcutaneous apomorphine is rapidly and completely absorbed. Plasma peak concentrations are achieved after 5–15 minutes and onset of clinical effect is within 20 minutes. Apomorphine intermittent subcutaneous injections are effective as rescue therapy for unpredictable off periods in advanced Parkinson disease (PD). More often apomorphine is administered as a subcutaneous infusion which secures the continuous dopaminergic stimulation. The benefit on ‘off’ periods is consistent across all studies, but dyskinesia improvement is not so obvious. Two infusion therapies (apomorphine and intraduodenal levodopa) and deep brain stimulation (DBS) are effective in advanced PD patients with untreatable motor complications. Apomorphine infusions should be considered in patients unable to undergo DBS because of cognitive impairment and neurosurgical contraindications.

Both disease progression and pulsatile stimulation of dopaminergic receptors are responsible for development of fluctuations and dyskinesia in about 50% of patients with Parkinson disease (PD) after 4-6 years of therapy with levodopa. In order to prevent motor complications, the ideal therapy should secure continuous dopaminergic stimulation (CDS). The concept of CDS is supported by the results of both experimental and clinical studies. Several treatment options are available to achieve CDS. Dopamine agonists have a longer half-life than levodopa and the development of dyskinesia is delayed when they are used as monotherapy in early PD. Continuous delivery of agonists can be improved with prolonged-release oral preparations, a transdermal delivery system or continuous subcutaneous infusion. Continuous enteral infusion of levodopa is another way to achieve CDS and it is very effective in reducing motor complications in advanced PD.

Multifocal motor neuropathy with conduction block (MMNcb) is a relatively rare disease characterized clinically by asymmetric limb weakness with spared sensation and electrophysiologically by persistent focal motor conduction block. We present the case of a 40-year-old male patient with six-year history of progressive, asymmetric weakness of upper and lower extremities without sensory symptoms. Electroneurography revealed definite or probable motor conduction block in several nerves. However, features of axonal lesion of sensory fibres were also found. Laboratory studies were unremarkable apart from an abnormal glucose tolerance test, and type 2 diabetes was diagnosed. In the presented case the differential diagnosis should take into consideration MMNcb with coexisting diabetic sensory polyneuropathy and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM).