Clinical Neuroscience

[Dystrophia musculorum progressiva in siblings]


DECEMBER 01, 1967

Clinical Neuroscience - 1967;20(12)

[In the light of recent knowledge on myopathies, the authors present their data on a pair of siblings with dystrophia musculorum progressiva and their clinically asymptomatic but enzymopathic family members, the first in the Hungarian literature.]


  1. Szegedi Orvostudományi Egyetem Ideg- és Elmekórtani Klinikája és Ideggyógyászati és Lélektani Gyermekgondozó Intézet



Further articles in this publication

Clinical Neuroscience

Données neurophysiologiques concernant la maturation de l’hippocampe


En dehors des recherches neurophysiologiques sur l'ontogenèse corticale (Elligson, Purpura), l'ontogenèse des connexions sous-corticales-corticales est peu étudiée, à l'exception de la formatio reticularis (Scheibel). Dans son étude, l'auteur cherche à répondre à la question de savoir comment l'ontogenèse de l'organe ammonien se reflète dans l'activité électromotrice pendant le sommeil et l'éveil (1) et comment le processus de maturation de la décharge de l'organe ammonien se reflète dans l'activité épileptique (2). Matériel d'étude : 80 chats, âgés de quelques heures à 2 mois. Durée du test : de quelques jours à plusieurs semaines. Électrodes implantées en stéréotaxie dans le cortex et le sous-cortex. Traitement histologique du cerveau après la fin de l'expérience pour localiser les électrodes.

Clinical Neuroscience

[About myopathies]


[Due to the nature of the presentation and the short time available, I can only briefly summarise the pathogenetic and clinical problems associated with myopathies.]

Clinical Neuroscience

[A case of chronic thyrotoxic myopathy]

HÓDI Miklós, HEINER Lajos

[The authors describe a rare form of chronic thyrotoxic myopathy. No palpable goitre, tachycardia or exophthalmos were observed in the presence of severe myopathy. They briefly discuss the pathogenesis of chronic thyrotoxic myopathy and the possibilities of establishing a pathogenesis in similar cases.]

Clinical Neuroscience

[Neuromyopathy of diabetes in breast meat]

CSAPÓ Gábor, HERPAY Sándor

[Authors have found, in agreement with the literature, that neuromyopathy is relatively common in diabetes. A systematic search for lesions is expected to increase the number of cases. The exact cause of the process is unknown, but its course can be objectively followed by EMG and measurement of nerve conduction velocity. In its management, a good control of diabetes, massive doses of vitamins and physiotherapy, in addition to the attempt of Thioctacid in refractory cases, is justified.]

Clinical Neuroscience

[Cases of myasthenia gravis in the elderly]

SOMLÓ Zoltán, KATONA József, HEINER Lajos

[The authors describe five cases of myasthenia gravis that started over the age of 60. They briefly summarise the diagnostic and therapeutic issues related to myasthenia in old age.]

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[Psychometric properties of the Hungarian Adult Attachment Scale]

ŐRI Dorottya, KAPORNAI Krisztina, BAJI Ildikó, KISS Enikő

[The revised Adult Attachment Scale (AAS) developed by N. L. Collins is a widely used questionnaire to measure adult attachment. However, its psychometric properties have not been investigated in Hungary. We aimed to confirm the key psychometric properties of the Hungarian version of the AAS focusing on reliability indices on a population that consis­ted of depressed and non-depressed young adults. The AAS is a self-report questionnaire, in which two different dimensional evaluating systems are possible: the original (close, depend, and anxiety) and the alternative scoring system (anxiety, avoidance). Our study population consisted of young adults with a history of major depression (n = 264, median age = 25.7 years) and their never-depressed biological siblings (n = 244, median age = 24.0). The internal consistency of close, anxiety, and avoidance scales were satisfactory (Cronbach-α >0.7). The consistency of the depend scale was slightly lower than expected (Cronbach-α = 0.62). Test-retest reliability was good for all of the scales, it ranged from 0.73 to 0.78 after 14 months of follow-up period. The scale showed good discrimination as tested by the differences of close and anxiety attachment dimensions between the groups (p<0.01). More­over, we were able to differentiate the currently dep­res­sed subjects based on these attachment dimensions. Explo­ra­tory and confirmatory factor analyses were conducted, and a bifactor solution proved optimal model fit. The three dimensions of the AAS has not been confirmed. However, the close and anxiety scales of AAS were found to be adequate. Our results also indicate that attachment features correlate with major depressive episodes in adulthood.]

Clinical Neuroscience

[Measurement of muscle action potential propagation velocity in clinical patient material]


[The authors investigated the propagation velocity of muscle action potentials in 53 normal individuals, 18 neurogenic lesions, 15 patients with myopathy and 2 patients with myasthenia. The muscle conduction velocity was assessed in the m. deltoideus, m. biceps brachii, m. flexor digiti V., m. tibialis anterior and m. quadriceps. Stimulation was performed with two needle electrodes in bipolar mode and recording with a concentric bipolar electrode. The muscle conduction velocity was 4.48 + 0.09 m/sec in the normal control group, 3.28 + 0.21 m/sec in neurogenic lesion and 3.07 + 0.18 m/sec in myopathy patients. The difference was highly significant in both groups (P < 0.001). The muscle conduction velocity within the neurogenic group was found to be the same in peripheral nerve lesion (3.29 = 0.33 m/sec) and anterior horn lesion (3.25 + 0.26 m/sec). In the neurogenic cases, we found a conduction velocity value below 3.5 m/sec in 68% of the clinically affected muscles. A conduction velocity below 3.5 m/sec was observed in 66.6% of myopathic patients. Conduction velocity values found in Duchenne-type dystrophia musculorum progressiva, dystrophya myotonic, thyrotoxic myopathy, myopathia tarda and polymyositis were significantly different from normal controls. In dystrophia musculorum progressiva in facioscapulo humeralis and limb girdle, the deviation from normal control was not significant. In myasthenia cases, muscle conduction velocity was normal (4.57 I 0.21 m/sec). The authors discuss the factors that may lead to a decrease in muscle conduction velocity in neurogenic and myogenic laesio. ]

Lege Artis Medicinae

[Donizetti's ailment: mood disorder, Barbaja, or luetic derailment?]


[The occurrence of specific forms of mood disorders is significantly higher among eminent creative persons, thus the talent and creative skills show some connections or may have common roots with mood swings. This phenomenon can be observed among composers as well. Our paper briefly summarizes several aspects of Gaetano Do­ni­zetti’s life (1797-1848), and some characteristic features of his operas. After his musical studies and initial successes Donizetti was hired by the opera impresario Domenico Bar­baja who pressed him under contract to write operas in a considerable number. Do­ni­zetti’s personal tragedy was that his wife and three children have died young. He composed more than 70 operas, wrote the librettos for some of them, had drawing skills, and a sense for theatre Gesamtkunst. His composing technique was incredibly fast, partly due to the external pressure (es­pecially by the impresario Barbaja). Beside the feverish speed (hypomania) he suffered of episodes with genuine fever, headache, and nausea. From 1845 onward full apathy, depression (occurred already earlier), and paralysis (progressiva?) developed hindering any creative power and meaningful interpersonal contact, so he was admitted to a closed mental facility. His illness was considered syphilis, but prevailing bipolar mood disorder may strongly be presumed today which is supported by early emerging creativity and hypomanic signs, transient mood swings, the composing technique, his multimodal talent, the abundance of his works (output of 3-4 operas a year), the vein (mood) streaming from the operas (’maddening scenes’ alongside lax and sparkling characters) and by certain circumstances (he was able to write opera buffas during his greatest personal tragedies). To all these com­ponents there can certainly be added an exogenous environmental pressure factor: Domenico Barbaja, the ’prince’ (and taskmaster) of opera impresarios. ]

Clinical Neuroscience

[Ocular myopathy (Kiloh-Nevin)]

MAGYAR István, GRÓSZ István, ASZALÓS Zoltán

[The authors briefly review the literature on ophthalmoplegia externa chronica progressiva. On the basis of clinical, objective biopsy, biochemical, EMG findings, they consider the disease to be confirmed and the name ocular myopathy proposed by Kiloh-Nevin to be justified. In the light of the biochemical data (which are abundant in the literature of recent years), the pathogenesis is considered to be due to a disturbance in muscle metabolism.]

Clinical Neuroscience

[Three cases of juvenile pseudomyopathy of spinal muscular atrophy (Kugelberg-Welander type)]


[The author described a juvenile pseudomyopathic (Kugelberg-Welander) form of spinal muscular atrophy (SI) in 3 cases. Juvenile SI can be differentiated from progressive dystrophia musculorum by the presence of muscle fasciculopathies and neurogenic EMG and muscle biopsy findings. The recognition of pseudomyopathic SI is of practical importance because of its much better prognosis than muscular dystrophy. Kugelberg-Welander juvenile and Werdnig-Hoffmann infantile SI cannot be considered as separate genetic types. In our case 2, SI started at the age of 11 years. In the muscle biopsy at 14 years of age, in addition to neurogenic atrophy, we found so-called myopathic lesions and lymphorrhagia. The relationship between these three types of pathophysiological syndromes is left to conjecture. Primary muscle lesions are considered to be independent of SI. The latter, i.e. lymphorrhagias and muscle fibre degeneration, may have a common (autoimmune?) pathomechanism. In our second case, during the 4-year follow-up, physiotherapy and exercise resulted in an increase in muscle strength instead of the previous permanent decrease in muscle strength. In our 3rd case, juvenile SI was associated with myositis. Presumably it was a coincidence of the two lesions. Author summarizes in a table the data used to distinguish between the ascending form of Kugelberg-Welander SI and dystrophia musculorum progressiva. ]