Haemophilic neuromyopathy.

Defaria, Carlos Roberto; Melo-Souza, Sebastião Eurico de; Pinheiro, E D

doi:10.1136/jnnp.42.7.600

Cited by 9 publications

(7 citation statements)

References 4 publications

(4 reference statements)

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“…The Pettersson radiologic score increases by 1 point for every three joint hemorrhages occurring after 5 years of age [43]. Furthermore, as joint damage progresses, the patient experiences pain [44], reduced range of motion [45], muscle atrophy [46][47][48], ankylosis, osteoporosis [33,34,[37][38][39][40], cartilage degeneration with collapse of the joint space [3,[49][50][51][52][53][54][55], and cyst formation [56,57].…”

Section: Joint Hemorrhage and Pathologic Findingsmentioning

confidence: 99%

Blood‐induced joint disease: the pathophysiology of hemophilic arthropathy

Valentino

2010

Journal of Thrombosis and Haemostasis

254

285

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Section: Joint Hemorrhage and Pathologic Findingsmentioning

confidence: 99%

Blood‐induced joint disease: the pathophysiology of hemophilic arthropathy

Valentino

2010

Journal of Thrombosis and Haemostasis

254

285

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“…For reasons of its non‐invasive character, SEMG is an appropriate method to examine patients with bleeding disorders. Although EMG is a commonly used method to evaluate muscle activation processes, studies using EMG are rare in the clinical diagnostics of haemophiliacs [9–14]. As SEMG measurements are used as a mirror of neuromuscular control, they can be used to evaluate neuromuscular characteristics in haemophilic patients.…”

Section: Introductionmentioning

confidence: 99%

“…As SEMG measurements are used as a mirror of neuromuscular control, they can be used to evaluate neuromuscular characteristics in haemophilic patients. The few existing EMG studies focused on case reports [9], exercise interventions with active [12] and passive modalities [14] and without any exercise‐related interventions [10,11,13]. Needle EMG as an invasive method was used by two groups [9,10].…”

Section: Introductionmentioning

confidence: 99%

“…The few existing EMG studies focused on case reports [9], exercise interventions with active [12] and passive modalities [14] and without any exercise‐related interventions [10,11,13]. Needle EMG as an invasive method was used by two groups [9,10]. However, it is necessary to collect data in various everyday situations to define a status quo in patients’ suffering from haemophilia.…”

Section: Introductionmentioning

confidence: 99%

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SEMG activation patterns of thigh muscles during upright standing in haemophilic patients

et al. 2011

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Although electromyography (EMG) is a common method to evaluate muscle activity, studies utilizing EMG in haemophilic patients are rare. The haemophilic arthropathy, resulting in altered afferent information is expected to cause disturbed activation and inter-muscular coordination patterns in haemophilic subjects. The aim of this study was to determine differences of selected knee muscles between haemophilic patients and non-haemophilic subjects during upright standing. Surface EMG (SEMG) amplitudes of rectus femoris, vastus medialis (VM), vastus lateralis (VL) and biceps femoris (BF) muscles of both sides were measured in 27 haemophilic patients (H) and 26 control subjects (C) while standing on an even surface. Data from both sides were pooled in C, but data of H were subdivided further according to major (H-MA) and minor (H-MI) affected joints. To normalize the data, amplitude ratios (percentage of cumulated activity) were calculated as well. Regardless of whether H-MA or H-MI was compared with C, amplitudes of all extensor muscles reached significantly higher levels in H (P < 0.05). SEMG amplitude ratios also differed between H and C. Independent of subgroup, BF showed significantly reduced activation ratios (P < 0.01). Only the ratios of VM and VL of H-MA could replicate the observed amplitude differences to C (P < 0.05). These findings show that while standing, haemophiliacs maintain the necessary stability demands through increased extensor activities and modulated coordination patterns. Although all thigh muscles of haemophiliacs are characterized by distinct atrophy, increased amplitude levels could be proved for the knee extensor muscles only. Therefore, general atrophy-related effects cannot explain these results.

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“…Die Methode zur Ableitung der elektrophysiologischen Prozesse im Muskel ist als Bestandteil der Motorikforschung in den Bereichen Sport (13,14), Prävention (15)(16) und Rehabilitation (17,18) bereits etabliert, kam jedoch als Diagnostik bei erwachsenen hämophilen Patienten selten zum Einsatz (9,(19)(20)(21).…”

Section: Oberflächenelektromyographieunclassified

Altersabhängige Ansteuerung der Muskulatur im Stand bei Patienten mit Hämophilie

Göhler¹,

Stäuber²,

Brunner³

et al. 2014

Hamostaseologie

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S36 OriginalarbeitInitialer Faktor der hämophilen Arthropathie stellt die Gelenkblutung dar, welche nach rezidivierenden Blutungsereignissen eine Synovitis mit folgender Zerstörung des Gelenkknorpels nach sich zieht. Dieser Prozess wird von den Patienten als sehr schmerzhaft wahrgenommen und kann sich nach einem längeren Zeitraum zu einem chronischen Schmerzzustand entwickeln.Studien aus der eigenen Arbeitsgruppe zeigen, dass hämophile Patienten gegenüber einer nicht-hämophilen Kontrollgruppe eine höhere Schmerzsensibilität auf der Seite des betroffenen Gelenkes aufweisen (1). SummaryThe haemophilic arthropathy affects the function of the knee joint muscles. The aim of this study was to investigate the myoelectrical signal of knee joint muscles in different age stages during upright standing. Surface EMG (SEMG) amplitudes of quadriceps, hamstrings and gastrocnemii were measured in 191 patients with severe haemophilia A (n=164) and B (n=27) while standing on an even surface. After an age-based classification of patients into the subgroups H A : 17-29 (n = 37), H B : 30-39 (n = 50), H C : 40-49 (n = 61), H D : 50-70 in years (n = 43) the clinical WFH score for the ankle and knee joint was determined. To normalize the SEMG values amplitude ratios (percentage of cumulated activity) were calculated with respect to the specific limb. With increasing age, the patient showed descriptively a deterioration of the joint situation. The extensors of the knee joint reached significantly higher absolute and percentage levels in the muscle activity with increasing age (p < 0.05). The absolute amplitude values of the Mm. gastrocnemii showed no differences in the age groups while the relative levels were decreased. The present study shows that patients with increasing age and degree of haemophilic arthro pathy deve lop a modified control strategy during upright standing, in the form of a shift from the plantar flexors to the extensors of the knee joint. Schlüsselwörter

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Haemophilic neuromyopathy.

Cited by 9 publications

References 4 publications

Blood‐induced joint disease: the pathophysiology of hemophilic arthropathy

Blood‐induced joint disease: the pathophysiology of hemophilic arthropathy

SEMG activation patterns of thigh muscles during upright standing in haemophilic patients

Altersabhängige Ansteuerung der Muskulatur im Stand bei Patienten mit Hämophilie

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