Clinical Neuroscience

[Carotid angiography detected during the procedure, signs of pathogenesis]

TUNKL Erika1, DÉNES Iván1

OCTOBER 01, 1969

Clinical Neuroscience - 1969;22(10)

[Authors have observed the resistance of the vessel wall and its surroundings, the distance of the blood column from the puncture needle, and the neurological and reflex symptoms of vessel compression during the technical performance of 960 carotid angiography. The empirical data thus obtained, in agreement with the signs found on angiograms, will help to clarify the vascular pathologies of the brain. ]

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Clinical Neuroscience

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

ANDRÁSOFSZKY T., MÁTHÉ Á., NAGY P., ROTH Gy., KOMJÁTSZEGI S., SZABÓ Á., KISGYÖRGY Á.

[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

[Encephalodystrophy progressiva paranatalis]

CSERMELY Hubert

[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.]

Clinical Neuroscience

Oro-lingual dyskinesia: a neurological enigma

MACDONALD Critchley

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.

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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

EVRAN Sevket, KATAR Salim

Far lateral lumbar disc herniations (FLDH) consist approximately 0.7-12% of all lumbar disc herniations. Compared to the more common central and paramedian lumbar disc herniations, they cause more severe and persistent radicular pain due to direct compression of the nerve root and dorsal root ganglion. In patients who do not respond to conservative treatments such as medical treatment and physical therapy, and have not developed neurological deficits, it is difficult to decide on surgical treatment because of the nerve root damage and spinal instability risk due to disruption of facet joint integrity. In this study, we aimed to evaluate the effect of transforaminal epidural steroid injection (TFESI) on the improvement of both pain control and functional capacity in patients with FLDH. A total of 37 patients who had radicular pain caused by far lateral disc herniation which is visible in their lumbar magnetic resonance imaging (MRI) scan, had no neurological deficit and did not respond to conservative treatment, were included the study. TFESI was applied to patients by preganglionic approach. Pre-treatment Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) scores of the patients were compared with the 3rd week, 3rd month and 6th month scores after the procedure. The mean initial VAS score was 8.63 ± 0.55, while it was 3.84 ± 1.66, 5.09 ± 0.85, 4.56 ± 1.66 at the 3rd week, 3rd month and 6th month controls, respectively. This decrease in the VAS score was found statistically significant (p = 0.001). ODI score with baseline mean value of 52.38 ± 6.84 was found to be 18.56 ± 4.95 at the 3rd week, 37.41 ± 14.1 at the 3rd month and 34.88 ± 14.33 at the 6th month. This downtrend of pa­tient’s ODI scores was found statistically significant (p = 0.001). This study has demonstrated that TFESI is an effective method for gaining increased functional capacity and pain control in the treatment of patients who are not suitable for surgical treatment with radicular complaints due to far lateral lumbar disc hernia.

Clinical Neuroscience

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study

NALBANTOGLU Mecbure, AKALIN Ali Mehmet, GUNDUZ Aysegul, KIZILTAN Meral

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

Clinical Neuroscience

[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.