Clinical Neuroscience

[Paralysis spinalis spastica familiaris]

OZSVÁTH Károly1, KOVÁCS Miklós1

FEBRUARY 01, 1965

Clinical Neuroscience - 1965;18(02)

[The authors observed spastic paraparesis starting at the age of 6-7 years in five of eight children (four boys, one girl) of a family, with nystagmus and oligophrenia in the three older children and optic atrophy in the three older ones. The mother and three other children have abortive syndrome. A similar disease occurs on the maternal side. A sketchy clinical review of the literature data of 1004 patients of 250 Strümpell-Lorrain's disease families is given.]

AFFILIATIONS

  1. Néphadsereg Egészségügyi Szolgálata

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[The neuroanatomical work of Károly Schaffer]

HORÁNYI Béla

[The author reports on the neuroanatomical work of Károly Schaffer.]

Clinical Neuroscience

[The role of forebrain structures in the organisation of conscious activity]

LISSÁK Kálmán

[The author reports on the role of forebrain structures in the organization of conscious activity.]

All articles in the issue

Related contents

Clinical Neuroscience

REM sleep, REM parasomnias, REM sleep behaviour disorder

SZÛCS Anna , MUTTI Carlotta, PAPP Anikó, HALÁSZ Péter, PARRINO Liborio

We review the literature on REM parasomnias, and their the underlying mechanisms. Several REM parasomnias are consistent with sleep dissociations, where certain elements of the REM sleep pattern emerge in an inadequate time (sleep paralysis, hypnagogic hallucinations and cataplexy) or are absent/partial in their normal REM sleep time (REM sleep without atonia, underlying REM sleep behavior disorder). The rest of REM parasomnias (sleep related painful erection, catathrenia) may have other still unclear mechanisms. REM parasomnias deserve attention, because in addition to disturbing sleep and causing injuries, they may shed light on REM sleep functions as well as the heterogeneous etiologies of parasomnias. One of them, REM sleep behavior disorder has special importance as a warning sign of evolving neurodegenerative conditions mainly synucleinopathies (some cases synucleinopathies themselves) and it is a model parasomnia revealing that parasomnias may have by autoimmune, iatrogenic and even psychosomatic etiologies.

Clinical Neuroscience

[Symptoms and pathology of anosognosia]

MOLNÁR László, SIMONYI Gusztáv

[There are different symptomatic forms or degrees of anosognosia for paralysis. Focal areas in the region of the posterior strial arteries of the j.o. typically cause anosognosia. In such cases, the insula and partly the projection fibrosis of the centropartiental and temporal regions are affected, but also the associative fibres. The anosognosia syndrome is therefore not related to the destruction of the thalamus or the wall region. The affection of the superior temporal gyrus and its fibres is permanently visible and therefore of crucial importance.]

Clinical Neuroscience

[Lumbar anaesthesia for severe neurological complications]

JOÓ Béla, TAKÁCS Piroska

[While in our first case the flaccid paresis and neuralgia pains of the left lower limb are most probably the result of a direct nervous system injury, in our second case we can exclude a direct nervous system injury. The acute headache, dizziness, nausea and tinnitus after the operation were probably the result of meningeal excitement. The symptoms suggest an accumulation of cerebrospinal fluid in the cerebellar spinal cord (meningitis serosa, circumscripta). The paralysis may be caused by meningeal irritation accompanied by arachnitis with secondary sweating. Consequent symptoms may be due to cerebral dysregulation caused by aseptic meningitis. According to the literature, abducens paralysis occurs between 2 and 12 days after anaesthesia. In most cases, paralysis resolves within days or a few weeks. It is possible and, as our case demonstrates, permanent paralysis. In such cases, an allergic mechanism may play a role in triggering the more severe meningitis.]

Clinical Neuroscience

[Further studies to detect human-brain corticosteroid fractions]

FAZEKAS I. Gyula, FAZEKAS Attila

[1. Lobar pneumonia, sudden cardiac death from paralysis, sudden cardiac death from paralysis in alcoholic state, self-harm, lightning strike, apoplexy cerebri, carcinosis peritonei, isonicide poisoning, cyanide poisoning, barbiturate poisoning, CO poisoning, Wofatox poisoning, alcohol poisoning+freezing deaths 26-92 years of age from 5 brain areas of 18 people (11 males and 7 females) were extracted 1 1/2-10 h after death, paper chromatographed in Bush 5 system, followed by chromatography in 2 n NaOH and 0.1% tetrasolium blue 9: 1. Evaluation was based on the tetrasolium blue reaction, NaOH fluorescence, Rf. values, standards and native blue fluorescence. 2 Brain extracts were run with different amounts of tetrahydrocortisol, tetrahydrocortisol, cortisol, cortisone, corticosterone and 11-dehydrocorticosterone as standards. The brain areas tested were : 1. bridge+brain, 2. cerebellum, 3. cerebellar nuclei, 4. cerebral cortex, 5. cerebral white matter. 3. 10 corticosteroid fractions were identified in total, 6 fractions were all identical to the standards, the other 4 unidentified steroids were labelled X1, X2, X3, X4. 4. These steroids were detected in different qualities and quantities in different brain areas depending on the deaths. 5. cortisol (=hydrocortisone) was detected in almost all brain areas tested in all 18 cases. Cortisol was found in most brain areas of those who died of heart failure, self-harm, lightning, apoplexy cerebri and various poisonings, but could not be detected in brain areas of those who died of heart failure in an alcoholic state and of cyanide poisoning. Other fractions were present in varying numbers and quantities in different brain areas. 6. Most fractures and the largest number were found in the brain areas of those who died of carcinosis peritonei and pneumonia. Fewest fractures and least amount were found in brain areas of those who died of cardiac arrest in alcoholic state and various intoxications in alcoholic state, and alcohol poisoning + frostbite. 7. Consistent with our previous animal studies and our studies on human subjects, we observed in the present study that alcohol induced significantly fewer corticosteroid fractions and quantities in all areas of the human brain compared to those in the non-alcoholic state. 8. In the cases studied, the total steroid content of each brain area does not show a consistent quantitative pattern that is characteristic of each death. 9. No lawful correlations between age, sex, adrenal weight, body weight, body height and the quantity and quality of corticosteroid content in the brain were found. 10. No decrease in brain corticosteroid content within 10 hours after death was observed with increasing time.]

Lege Artis Medicinae

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

JANKA Zoltán, SZAKÁCS Réka

[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. ]