Clinical Neuroscience

[Encephalodystrophy progressiva paranatalis]


OCTOBER 01, 1969

Clinical Neuroscience - 1969;22(10)

[The author analyses the histopathological picture of the subacute and chronic stages of paranatal asphyxia based on 3 observations. In the chronic stage, he suggests the name "status microcysticus" for the spongy loosening of the cortex and cortical bone, which should be distinguished from the van Bogaert and Leigh type "status spongiosus". The status microcysticus is the result of a functional or organic vascular lesion. Finally, he discusses Alpers' disease and considers that the majority of the findings to date do not correspond to Alpers' disease.]


  1. MÁV Kórház és Rendelőintézet prosectura



Further articles in this publication

Clinical Neuroscience

[Choosing how to explore in lumbar hernia operations, based on 373 cases over four years - 1965-1968]


[It is the authors' understanding that the most important criteria for the successful surgical treatment of lumbar hernias are the correct indication and timing of surgery, avoidance of myelography, minimal bone resection, but always complete root decompression. These criteria were applied in 373 operations between 1965 and 1968, with inter-arch exploration in 87.64% of cases. The situations which make each type of exploration possible or necessary are analysed. It is stressed that inter-arch exploration can be used to remove hernias causing cauda-unusual hernias and that this method of exploration can also be used in reoperations. ]

Clinical Neuroscience

[About granulomas in the dorsal root ganglia of the spinal cord]

BALÓ József

[In the study of spinal ganglion cysts, we have monitored granulomas that occur in the posterior gyri of the spinal cord. Although these granulomas also contain a chronic inflammatory component, they are also populated by endothelial, or more recently meningothelial, cells of the arachnoid. According to Verga and Rexed and Wennström, these granulomas are important as causative factors in the formation of cysts in spinal ganglia. Veith has observed granulomas in the root nerves in connection with chronic infectious diseases of other organs (chronic polyarthritis, chronic cholecystitis, and malignant tumours), which he considers to be of non-specific origin. He raised the question of whether Richter's tabes granulomas are specific or non-specific. In cases of pemphigus, granulomas similar to those described by Veith in various chronic infectious diseases occur in the dorsal root ganglia of the spinal cord. The question of whether Richter's specific syphilitic granulomas are identical to Veith's non-specific granulomas due to chronic infectious diseases needs further investigation. A similar finding was reported on the subject raised, that of a disease which started with chronic inflammation (tuboovarial abscess) and was associated with granulomas in the root nerves, the clinical course leading to death in the form of multiple neurotic gangrene of the skin.]

Clinical Neuroscience

Mélanoblastose et neurofibromatose

LUDO Van Bogaert

Les observations cliniques et génétiques rapportées indiquent que la blastomatose méningée et cutano-méningée, lorsqu'elle se présente comme une blastomatose isolable et sévère, entretient un lien, en apparence et surtout en familiarité, avec les dysplasies génétiques du système nerveux central. La conception mésoectodermique de l'ontogenèse de la mélanine explique non seulement l'apparition des neurinomes, des gliomes et des méningiomes, mais aussi la possibilité d'observer des processus mélaniques dans des phacomatoses telles que la neurofibromatose, la sclérose tubéreuse et l'angiomatose.

Clinical Neuroscience

Oro-lingual dyskinesia: a neurological enigma


Author draws attention to a specialised dystonic syndrome involving mainly the muscles of the mouth, lips and tongue. Spasms can become so extensive that they can prevent the patient from writing, walking and standing. In particular, the muscles of the face and tongue may be affected. The pathogenesis and pathogenesis are not uniform. In the past, most cases of the disease were late complications of encephalitis. Nowadays this pathology is unlikely. Some cases strongly suggest a psychogenic origin, others are more likely to be of degenerative or ischaemic origin. This is particularly the case in elderly patients. Finally, in a proportion of cases, an organic syndrome due to unusual sensitivity to prolonged use of phenothiazides or other medications is likely.

Clinical Neuroscience

[Cerebrovascular accidents with psychiatric symptoms]


[We have described and illustrated it with "model" cases: a) The acute reversible psychotic symptom complex of hypertensive encephalopathic meningo-cerebral oedema, which we reported in 1956, which presents as a cerebral vascular catastrophe and can be distinguished from the progressive form of meningo-cerebral oedema, the latter being known from an earlier date. b) Psychotic episodes of shorter or longer duration in the Binswanger-Grünthal form of cerebral vascular atherosclerosis, which, although presenting as cerebral vascular catastrophes, are considered to be a form of generalised circulatory insufficiency, beginning with cerebral circulatory insufficiency. c) We have also isolated, on the basis of clinical and EEG features, the temporal lobe Korsakow variant. In our case, we also found a diffuse cerebral small vessel sedation with acute onset and recurrent psychotic symptomatology over many years.]

All articles in the issue

Related contents

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. ]

Clinical Neuroscience

[Investigation of neuromuscular synapses in muscle diseases]


[From the muscle biopsy material of our clinic, the subneural apparatus of the myoneural junction was examined in 32 cases by the Koelle acetylthiocholine method, and the neural part by the Bielschowsky-Gross-Schultze method modified by Coers. The amount of material studied by these tests is small in relation to the number of biopsies performed in our clinic. However, also according to our previous results, we have seen that in ALS the subneural apparatus is relatively uniformly smaller, with variable size in the initial stage of dystrophia musculorum progressiva, with more large, hypertrophic forms, while in the later stage we find smaller, more irregular forms. Intramuscular nerve fibres showed irregular course, thinning in ALS, and irregular, degenerative axonal swellings in dystrophies. We do not want to draw any far-reaching conclusions from the material we have examined so far, but based on the literature and our initial experience, we see that further and more extensive studies in this direction are absolutely necessary to better understand the pathology of skeletal muscle. ]