Spontaneous palpebromandibular synkinesia: A localizing clinical sign

In the classical corneomandibular reflex (CMR), corneal stimulation elicits a bilateral eyelid blink and a brisk anterolateral jaw movement. We here describe 14 patients with a spontaneous palpebromandibular (eyelid–jaw) synkinesia (SPMS) in whom jaw movements, similar to those in CMR, regularly accompanied spontaneous eye blinks without an external corneal stimulus. Eleven of the patients with SPMS also had CMRs on corneal stimulation. Four patients had clinical and imaging evidence of brainstem lesions above the mid-pons, 5 patients had autopsy or imaging evidence of both bilateral cerebral and upper brainstem lesions, and 5 patients had clinical or imaging evidence of bilateral cerebral dysfunction. Topical corneal anesthesia administered to patients who had both CMR and SPMS blocked the CMR but had no effect on the SPMS. In patients with both SPMS and CMR, measurements of latency from onset of orbicularis oculi electromyographic activity to onset of lateral pterygoid EMG activity, and mandibular kinesiography of jaw velocity and direction showed that the eyelid–jaw synkinesias of CMR and SPMS had similar characteristics. We conclude that SPMS is pathophysiologically the same as the eyelid–jaw synkinesia of CMR and both synkinesias originate centrally, probably in the pons. In CMR, the jaw movement is primarily related to the blink rather than the corneal stimulus, but corneal stimulation may be necessary to overcome a higher threshold for expression of the synkinesia than in patients with SPMS. Like CMR, SPMS emerges in patients with upper brainstem or bilateral cerebral lesions and SPMS may therefore be a useful localizing clinical sign.     

Favorable responses to acute and chronic exercise in McArdle patients.

OBJECTIVE: This study reports acute exercise responses in a large (N = 46) series of patients with McArdle disease and responses to exercise training in a smaller (n = 9) set of patients. DESIGN: Patients were studied during both incremental and steady-state cycle ergometer exercise, using cardiopulmonary testing, and the patients were compared with age- and gender-matched controls. SETTING: The study was performed in a university setting (clinical exercise physiology laboratory). PARTICIPANTS: The 46 patients showed common features of McArdle disease. They were definitively diagnosed by histochemistry, biochemistry, and/or molecular genetic analysis. The 46 controls were healthy, sedentary individuals. INTERVENTION: Nine patients were studied before and after an 8-month supervised aerobic exercise training program (including five weekly sessions of walking and/or cycling exercise with a duration no greater than 60 minutes). MAIN OUTCOME MEASUREMENTS: The main indicators of exercise capacity that we measured were peak power output, peak oxygen uptake (VO2peak), and ventilatory threshold (VT). RESULTS: Exercise capacity (peak power output, 35% control; VO2peak, 44% control; VT, 66% control) was markedly depressed in the patients. The patients who trained improved peak power output (25%), VO2peak (44%), and VT (27%), with no evidence of negative outcomes from training. Although not achieving normal values, the response to training put the patients into the lower limit of normal controls. CONCLUSIONS: Under carefully controlled conditions, patients with McArdle disease may perform acute exercise safely, and they may respond favorably to training. This may offer an additional therapeutic option to help normalize the lifestyles of these patients.     

Localization of endogenous beta-galactoside-binding lectin as a means to distinguish malignant from benign skin tissue

The immunolocalization of a 30-kd endogenous lectin, referred to as galaptin, was studied in various skin tumor types. Basal cell carcinomas expressed little or no galaptin, whereas nonmalignant basaloid cells, squamous cell carcinomas, melanoma, nevi, and stroma showed more prominent galaptin immunostaining. Therefore, the immunodetection of galaptin may aid the dermatopathologist in difficult histologic diagnosis.     

Über die Entstehung der Gelenkkörper bei Arthritis deformans

Ohne Zusammenfassung (Mit Tafel I und 1 Abbildung.)     

Long-term control of choroidal neovascularization in quiescent congenital toxoplasma retinochoroiditis with photodynamic therapy: 4-year results

Purpose To evaluate the efficacy and safety of photodynamic therapy (PDT) in the long-term control of subfoveal choroidal neovascularization (CNV) associated with toxoplasmic retinochoroiditis. Methods The records of 13 patients with classic subfoveal CNV associated with toxoplasmic retinochoroiditis treated with PDT were reviewed. All patients were followed up for at least 48 months. Postoperative visual acuity was defined as a gain or loss of two or more lines of best-corrected visual acuity (BCVA), respectively. Post-treatment CNV size was dichotomized into “increased” if the major CNV diameter (CMD) had increased by ≥300 μm, and as “stable/reduced” if it had decreased by ≥300 μm or had not changed by >300 μm. Results Nine patients [four males (44.4%) and five females (55.6%)] with a mean age of 20.1 ± 4.3 years (range 14–27 years) were enrolled in the study. All had unilateral involvement. The median follow-up was 55 months (minimum 48, maximum 65 months). At the 48-month follow-up, all patients had stable/improved BCVA and a mean stable/reduced CMD (846 ± 326.5 μm), with the BCVA having improved significantly (p < 0.0001) from 0.29 ± 0.19 at baseline to 0.54 ± 0.16 at 48 months. Conclusion Photodynamic therapy seems to be a safe and effective approach to the long-term control of subfoveal CNV associated with toxoplasmic retinochoroiditis. Further trials are needed to validate these findings.     

Modulating the development of renal tubules growing in serum-free culture medium at an artificial interstitium

Little information on the structural growth of renal tubules is available. A major problem is the technical limitation of culturing intact differentiated tubules over prolonged periods of time. Consequently, we developed an advanced culture method to follow tubule development. Isolated tissue containing renal progenitor cells was placed in a perfusion culture container at the interphase of an artificial polyester interstitium. Iscove's modified Dulbecco's medium without serum or protein supplementation was used for culture, and the culture period was 13 days. Tissue growth was not supported by addition of extracellular matrix proteins. The development of tubules was registered on cryosections labeled with soybean agglutinin (SBA) and tissue-specific antibodies. Multiple SBA-labeled tubules were found when aldosterone was added to the culture medium. In contrast, culture without aldosterone supplementation displayed completely disintegrated tissue. The development of tubules depended on the applied aldosterone concentration. The use of 1 x 10(-6) M and 1 x 10(-7) M aldosterone produced numerous tubules, while application of 1 x 10(-8) M to 1 x 10(-10) M led to a continuous decrease and finally a loss of tubule formation. The development of labeled tubules in aldosterone-treated specimens took an unexpectedly long period of at least 8 days. The morphogenic effect of aldosterone appeared to be mineralocorticoid hormone-specific since spironolactone and canrenoate abolished the development. Finally, dexamethasone induced widely distributed cell clusters instead of tubules.     

<Emphasis Type="Italic">V</Emphasis>O<Subscript>2</Subscript>max during successive maximal efforts

The concept of VO₂max has been a defining paradigm in exercise physiology for >75 years. Within the last decade, this concept has been both challenged and defended. The purpose of this study was to test the concept of VO₂max by comparing VO₂ during a second exercise bout following a preliminary maximal effort exercise bout. The study had two parts. In Study #1, physically active non-athletes performed incremental cycle exercise. After 1-min recovery, a second bout was performed at a higher power output. In Study #2, competitive runners performed incremental treadmill exercise and, after 3-min recovery, a second bout at a higher speed. In Study #1 the highest VO₂ (bout 1 vs. bout 2) was not significantly different (3.95 ± 0.75 vs. 4.06 ± 0.75 l min⁻¹). Maximal heart rate was not different (179 ± 14 vs. 180 ± 13 bpm) although maximal V _E was higher in the second bout (141 ± 36 vs. 151 ± 34 l min⁻¹). In Study #2 the highest VO₂ (bout 1 vs. bout 2) was not significantly different (4.09 ± 0.97 vs. 4.03 ± 1.16 l min⁻¹), nor was maximal heart rate (184 + 6 vs. 181 ± 10 bpm) or maximal V _E (126 ± 29 vs. 126 ± 34 l min⁻¹). The results support the concept that the highest VO₂ during a maximal incremental exercise bout is unlikely to change during a subsequent exercise bout, despite higher muscular power output. As such, the results support the “classical” view of VO₂max.