The article was sent to an Austrian periodical but it was rejecte

The article was sent to an Austrian periodical but it was rejected with commentary that the immunological analysis was too insufficient to allow the disease to be declared immunologic. We published the case in Extenso in 1984 (13). We continued to discover many patients with similar main symptoms: for example, there was a patient with hypoparathyroidism, generalized stiffness and abundant spontaneous electromyographic activity (14), then there was a baby and a child with neuromyotonia (15, 16) and four adolescent patients – two sporadic and two hereditary (17). All of them improved on carbamazepine. In the two sporadic adolescents, even an extensive Inhibitors,research,lifescience,medical remission on corticosteroids was recorded. In one hereditary case,

intensive Inhibitors,research,lifescience,medical contracture in the arm joint disappeared after a local curare test (18). Pronounced contractures in the wrist area and interphalangeal joints, in four adult patients with

dystrophic myotony improved also following a regional curare test. The result pointed to the possible origin in distal nerve irritation (19). Correctible contractures, with lasting extensive improvement The observed contractures developed in a short time, limited to the third, fourth Inhibitors,research,lifescience,medical and fifth finger and wrist, with some sensibility damage in the ulnar nerve innervation region. In two cases, the compression was differentiated in the elbow region; in the one, it was on the lower arm. Instead of tenotomy or an operation of the muscle, neurolysis was done. The location was indicated by MS-275 in vivo plurisegmental electroneurography. Contracture disappeared very soon after the operation (20). Persistent contractures Very precise differential diagnostics of a slowly progressive diffuse contracture of the spine (“rigid spine syndrome” Inhibitors,research,lifescience,medical ) was conducted, associated with thorax deformity, mainly proximal myopathy and delayed sexual maturity, in three unrelated patients (21). In 1989 (22), I published an article about progredient syndrome in two generations, when the syndrome had not yet been described in the literature.

It had been considered as joint Inhibitors,research,lifescience,medical disease. However, it was obviously a disease of the muscle. A very slow progredient contracture Calpain of the fingers is the dominant symptom. Percussion of muscle causes depression only on the tongue, while on the extremities it causes extreme high skin bulging. On forced, passive extension of muscles, repetitive EMG activity occurred as registered by special bipolar wire electrodes used in my other kinesiologic studies. On the local curare test the contracture disappeared and the percussion response was reduced. Paroxismal, generalised, very painful spasms in full consciousness After occurring for five days, at intervals of a few minutes, throughout the day and night, generalised spasms almost led to the death. Individually adjusted and prolonged therapy by methyl-prednisolone led to complete remission with the patient living a normal life for years afterwards.

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