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The second edition of the Baltic School of Neuromyology took place on August 26-27, 2022 in Riga (Latvia), in a somewhat peculiar atmosphere given the international situation. An opportunity for the authors to measure the accomplishments made by their Baltic counterparts in the diagnosis and management of neuromuscular disorders, a successful although challenging venture, which led to the integration of three Baltic neuromuscular reference centers to the European Reference Network Euro-NMD. Beyond this form of consecration, and even though a lot remains to be achieved, notably in the field of muscle histopathology, various Baltic teams showed truly remarkable pieces of clinical research that will be useful to the whole myology community worldwide., (© 2022 médecine/sciences – Inserm.)

One hundred patients presenting with exercise intolerance or rhabdomyolysis episodes have been examined successively by 31P Nuclear Magnetic Resonance Spectroscopy (MRS) of leg plantar flexor muscles with exercise test. In all cases a muscle biopsy was performed. At the end of investigations, diagnosis of a metabolic myopathy was made in 33 patients: glycogenolysis or glycolysis deficiency in 8 cases, mitochondrial myopathy in 24 cases and CPT II deficiency in one case. Muscular dystrophy or congenital myopathy were diagnosed in 6 cases. No precise etiology could be found in 30 patients with either high CK levels or muscle biopsy abnormalities. Seven patients had rhabdomyolysis related to excessive physical activities. Twenty-four patients had functional symptoms. The principal MRS parameters used for diagnosis were the values of intracellular pH at the end of exercise and the time constant of phosphocreatine resynthesis during recovery. Lack of acidosis after exercise was observed in all patients with blockade of glycogenolysis or glycolysis. A slowing in phosphocreatine resynthesis was found in 66 p.cent of patients with definite mitochondrial myopathy. The specificity of these parameters were respectively 92.4 p.cent and 85.5 p.cent for the two groups. In conclusion (31)P MRS allows the detection of muscular glycogenoses with a sensitivity close to 100 p.cent. However, its sensitivity was lower for the detection of mitochondrial myopathies, as is also known for the other in vivo metabolic investigations, reflecting the heterogeneity of expression of mitochondrial abnormalities in a given muscle. The integration of imaging in the examination protocol may help to orientate towards the diagnostic of a dystrophy in some patients.

The lack of effect of treatment of mild hypertension on the coronary heart disease has motivated researches for a better diagnosis of hypertension. One of the approaches presently under study uses the recording of ambulatory blood pressure using semi-automatic devices. The usefulness of these apparatus is however restricted by the lack of reference values recorded in normotensive control patients. We have recorded ambulatory blood pressure (PAA) in 24 normotensives, 22 untreated hypertensives and 45 treated hypertensive patients, and compared the data obtained to the blood pressure recorded during medical examination (PAC). If a good correlation is usually observed between PAA and PAC, very large and unpredictable discordances are frequently observed. No correlation is found between the difference PAA-PAC and the variability of PAA. This variability does not fully explain the difference observed between PAA and PAC. This variability expressed in mmHg increases with age and the level of BP. Ambulatory BP appears to be a very reproducible value which may allow to improve the definition of hypertension and there-fore the cardiovascular risk.