Treatment of psoriatic arthritis with tumor necrosis factor inhibitors: longer-term outcomes including enthesitis and dactylitis with golimumab treatment in the Longterm Extension of a Randomized, Placebo-controlled Study (GO-REVEAL).

OBJECTIVE: To assess longer-term outcomes, including enthesitis and dactylitis, in patients with active psoriatic arthritis (PsA), in a study of golimumab treatment. METHODS: Adult patients with active PsA were randomized to receive subcutaneous injections of placebo (n = 113), golimumab 50 mg (n = 146), or golimumab 100 mg (n = 146) every 4 weeks through Week 20. All patients received golimumab 50 mg or 100 mg from Week 24 onward. Entheses tenderness was scored in 15 body sites using the PsA-modified Maastricht Ankylosing Spondylitis Enthesitis Score (MASES). Dactylitis was assessed in 20 digits of the hands and feet. RESULTS: Among the 405 randomized patients, 77% presented with enthesitis and 34% dactylitis at baseline. At Week 24 of the placebo-controlled study phase, significant differences were observed between golimumab 50 mg and/or 100 mg and placebo for mean percent improvement in the PsA-modified MASES [46% (p < 0.001) and 52% (p < 0.001) vs 13%, respectively] and the dactylitis score [66% (p = 0.09) and 82% (p < 0.001) vs 28%, respectively]. By Week 52, improvements were maintained among patients randomized to receive golimumab (mean improvements of 54% for PsA-modified MASES and 77% for the dactylitis score). Those given placebo who had enthesitis or dactylitis at baseline and who crossed over to golimumab at Week 16 or 24 had somewhat less improvement at Week 52 (i.e., 39% for the PsA-modified MASES, 57% for dactylitis score). CONCLUSION: Treatment of PsA patients with the TNF inhibitor golimumab was effective across all components of disease, including enthesitis and dactylitis, and efficacy was maintained over longer-term followup.

Effective uric acid reduction with probenecid and febuxostat in a patient with chronic kidney disease.

A 33-year-old male with poorly controlled chronic tophaceous gout and chronic kidney disease (CKD) with estimated glomerular filtration rate (GFR) of 37 cc/min. His uric acid was 11 mg/dL despite maximal dosing of febuxostat. He had previously failed pegloticase infusions as well. This patient had a reduction in his uric acid level to less than 6 mg/dL following addition of probenecid to his febuxostat regimen. Most guidelines recommend against utilisation of probenecid therapy in patients with GFR <50, but there is no obvious contraindication to its use, provided renal calculi do not develop. Our case illustrates the synergistic effect probenecid can add to maximal xanthine oxidase inhibitor therapy for patients with refractory hyperuricaemia in a patient with CKD stage IIIb. With the approval of a new uricosuric medication, lesinurad, probenecid may remain a suitable alternative for patients with financial limitations to achieve target uric acid levels.

Planovalgus foot deformity in cerebral palsy corrected by botulinum toxin injection in the peroneus longus: Clinical and radiological evaluations in young children.

BACKGROUND: In children with cerebral palsy (CP), overactivity of the peroneus longus (PL) muscle is a major contributor to pes planovalgus. This retrospective study assessed whether abobotulinumtoxinA injections into a PL showing premature activity on electromyography (EMG) clinically improved foot morphology in children with CP. METHODS: Study participants were <6 years old, had a diagnosis of CP, good functional abilities (Gross Motor Function Classification System level 1 or 2), equinovalgus (initial contact with the hallux or head of the first metatarsal) and overactive PL on EMG. The fore-, mid- and hindfoot were evaluated clinically and radiologically before and after injection of abobotulinumtoxinA (6-7 U/kg) into the PL. Radiological data were compared with reference values for children without pes planovalgus. RESULTS: In total, 16 children (8 males; 10 hemiplegia, 6 diplegia; mean age: 3.2±1.5 years) received treatment. Mean pre-and post-treatment angles in clinical assessment of dorsiflexion of the talocrural articulation did not differ with both knees flexed (24.4±7.5 vs. 22.2±8.0 degrees; P=0.19) or extended (17.2±8.0 vs. 16.6±6.8 degrees; P=0.36). Radiographic data pre-treatment versus reference data revealed forefoot pronation (metatarsal stacking angle 2.1±8.3 vs. 8.0±2.9 degrees; P=0.002), midfoot planus (lateral talo-first metatarsal 28.5±15.0 vs. 13.0±7.5 degrees; P<0.001; talocalcaneal angle 54.6±8.6 vs. 49.0±6.9 degrees; P=0.004) and significantly decreased calcaneus dorsiflexion, without hindfoot equinus (calcaneal pitch angle 7.9±6.0 vs. 17.0±6.0 degrees; P<0.001). After treatment, the metatarsal stacking angle did not differ from reference values (P=0.15). As compared with before treatment, treatment improved mean angles for metatarsal stacking (2.1±8.3 vs. 7.1±3.9 degrees, respectively, P=0.002), lateral talo-first metatarsal and talocalcaneal (both P<0.001), with no change in the hindfoot. CONCLUSION: PL may be an early target for abobotulinumtoxinA treatment in pes planovalgus associated with premature PL activity in children with CP.

Chronic tophaceous gout in the elderly.

Gout in the elderly seems to represent a special subgroup. The presentation is often atypical and drug treatment poses special difficulties. We present two elderly patients with large subcutaneous tophi leading to grotesque deformities. Their clinical features, risk factors, diagnosis and treatment are discussed. Although the appearance and radiographic changes caused by tophi are characteristic, the definitive diagnosis of tophaceous gout is made by aspiration and crystal analysis, as is illustrated by colour photographs.

[Chronic inflammatory demyelinating polyneuropathy in childhood--a case with markedly hypertrophic nerves and pes cavus].

We describe a 9-year-old boy with chronic inflammatory demyelinating polyneuropathy (CIDP), whose distal dominant, slowly progressive motor sensory involvement developed since infancy. Thickened peripheral nerves were visible in his neck and palpable in the extremities. The hand muscles were atrophic and both feet showed pes cavus deformity. He was unable to walk. Nerve conduction study revealed no sensory potentials. Compound muscle potential (CMP) was absent in the legs. In the arms, CMP showed markedly low amplitude and temporal dispersion. Motor conduction velocities were 1.7 to 2.7 m/sec, showing extremely high threshold to electrical stimuli. Conduction block was not convincing, nevertheless the asymmetric conduction abnormalities strongly suggested multifocality of the lesion. In the biopsied sural nerve no myelinated fibers were found. Considerable improvements in muscle power and electrophysiological findings were brought about by corticosteroid therapy. CIDP in childhood may masquerade hereditary neuropathies presenting hypertrophic peripheral nerves and pes cavus deformity. A careful nerve conduction study is thus desirable to find out electrophysiological multifocality.

The Madura foot: an "innocent foot mycosis"?

With the increased movement of the world population, acquaintance with the clinical picture of the Madura foot is of growing importance beyond its original endemic areas. The characteristic triad of symptoms consists of indurated swelling, multiple sinus tracts with purulent discharge filled with grains and localization at the foot. An increasing number of new etiologic agents are recognized today. For a better choice of therapy an adequate diagnostic procedure is essential ; a deep biopsy for histology appears to give a more substantial contribution to identification of the causal organism than culture. The treatment which should be started early, is at first essentially a drug treatment. However, in spite of high expectations with regard to new antimycotic drugs, amputation or disarticulation is often inevitable even today, particularly when the lesion is caused by Eumycetes. The first two documented patients with this disease in the Netherlands are described. They developed serious deformities of the lower extremity despite long-term use of antimycotic and antibiotic medication.

Chronic mucocutaneous candidiasis: a case report.

Chronic mucocutaneous candidiasis is a immuno deficiency disorder primarily due to T cell dysfunction characterized by persistent candidal infection of mucous membrane, skin, scalp and nails. Chronic mucous membrane candidiasis has an onset in infancy or childhood; the primary affected site is the oral cavity; however, lesions may occur on trunk, hands, feet and scalp. This paper describes a 12-year-old girl with candidial infection of the oral mucosa and extra oral involvement of fingers, nails, toes and intertragus area.

Idiopathic fluctuating dystonia: a case of foot dystonia and writer's cramp responsive to L-dopa.

A forme fruste of progressive idiopathic dystonia is reported in a girl, starting at eight years and followed to 24 years of age. The first symptom was isolated dystonia of the left foot and later writer's cramp developed in the hands. Fluctuations of the dystonia occurred later in the course of the disorder, which were not related to the sleep-walking cycle. Complete remission of symptoms was obtained with low dosage of L-dopa. This case broadens the spectrum of fluctuating dopa-sensitive progressive childhood dystonias and provides evidence for writer's cramp being a form of focal dystonia.

[Local injection treatment with botulinum toxin A in severe arm and leg spasticity]. / Lokale Injektionsbehandlung mit Botulinum-Toxin A bei schwerer Arm- und Beinspastik.

In patients with predominantly focal spasticity, oral antispastic drugs are relatively ineffective or cause unwanted side effects of central origin. Therefore we treated patients disabled by focal spasticity with local injections of Botulinum-Toxin A (Porton Products BOTOX). Efficacy, dosage, side-effects and injection technique were examined. 11 patients (mean age 48 years) with severe focal spasticity of the flexor muscles of the hand and arm (5 patients), the adductor muscles of the legs (5) or the plantar flexors of the foot (1) due to multiple sclerosis, cervical myelopathy or stroke-related hemi-paresis were treated with BOTOX. Rating scales, including Ashford spasticity scale, pain scale and a hygienic rating scale, were used to evaluate the efficacy. 25 to 30 ng (1000-1200 MU Porton) were injected in the flexor group of the hand or arm and 42 to 50 ng (1680-2000 MU Porton) BOTOX in the adductor group of one leg. 10 of the patients showed an improvement of at least one point on the scales for spasticity, pain and hygiene. Effects could be observed after 4-7 days and lasted for 6-13 weeks. There were no unwanted side-effects. We conclude that BOTOX is an alternative to the systemic application of antispastic drugs. Focal spasticity and pain can be successfully reduced and hygienic care is facilitated.

Management of spasticity in cerebral palsy with botulinum-A toxin: report of a preliminary, randomized, double-blind trial.

In order to evaluate further the efficacy of local intramuscular injections of botulinum-A toxin (BAT-A) in the management of dynamic equinus deformity associated with cerebral palsy, a randomized, double-blind, placebo-controlled study was undertaken. When evaluated using our Physician Rating Scale, 83% (five of six) of patients receiving toxin showed improvement, versus 33% (two of six) receiving placebo. There were no major complications. BAT-A injections appear to be safe and effective in children, and merit further prospective study.

[Radiologic progression of rheumatoid arthritis in early basic drug therapy]. / Die radiologische Progression der rheumatoiden Arthritis bei frühzeitiger Basistherapie.

For therapy and follow-up control in early disease modifying antirheumatic drug (DMARD) treated rheumatoid arthritis (RA), objective quantification is still lacking. Therefore, radiological analyses are considered the most appropriate method. One aim of this study was to retrospectively determine the time-dependent progression of joint damage in RA patients on DMARDs. Outpatient records and radiographs from hands and feet of 54 RA patients on DMARDs were evaluated. Radiographs were quantified by using the original Larsen score and a newly developed computer-assisted quantification software. Our observations showed that radiologically-detectable damage in all patients, regardless of their treatment, is most pronounced during the first year of disease, being mitigated and generally progressing linearly in the subsequent years. Cumulative ESR correlated with RA progression, and its reduction with therapeutic efficacy.