Clinical Neuroscience

[Neurological complications of multiple myeloma]

AMBRÓZY György1, HINDY Iván1

APRIL 01, 1969

Clinical Neuroscience - 1969;22(04)

[1. Authors describe neurological complications caused by multiple myeloma and plasmocytoma. They were the first symptom of disease in 15% of the total patient population [53] and in 33% of cases with neurological complications. 2. The clinical symptoms, pathogenesis and course of 21 patients with multiple myeloma with neurological complications and 3 patients with plasmocytoma are analysed. 3. X-ray examination, cytological tests (bone marrow smear, bone biopsy) and other laboratory tests (red blood cell count, peripheral blood count, serum and cerebrospinal fluid paper and immunoelectrophoresis) are used to detect early stage disease. Bence-Jones protein was found in urine in only 1/3 of the cases reported here. 4. Un. complex (non-specific robotic, irradiation, cytostatic, in some cases surgical or conservative orthopaedic) treatment of neurological complications was associated with a disease duration of approximately 21.2 months (living patients) and 26.4 months (dead patients) from the time of admission. ]

AFFILIATIONS

  1. Budapesti Orvostudományi Egyetem I. Neurológiai és Psychiatriai Klinika és az Országos Onkológiai Intézet

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Clinical Neuroscience

[The histological structure of human muscle spindles and innervation ]

ABONYI Eszter, STIPULA Magda

[We used a large muscle biopsy from our clinic to investigate how muscle spindles behave in different muscle diseases. However, in many cases, the muscle fragments excised for diagnostic purposes did not contain muscle spindles or only a small number of them. The data in the literature refer mainly to animal muscle spindles (see further as ms). Therefore, the histopathological evaluation of the observed muscle spindle images was difficult. There have been few studies on pathological lesions of human ms. For this reason, we aimed to collect first of all experiences on normal human io. The present study deals with the structure and innervation of normal human io. Our next paper will deal with the development of the ms, and our third paper will discuss the pathophysiological changes of the ms in different neurologically important muscle diseases. ]

Clinical Neuroscience

[Arrangement of the Rorschach test items]

PETHŐ Bertalan

[In summary, the record paints a picture of a mentally mildly and unevenly retarded, eccentric, self-contained, not very well hidden from others and ambivalently experienced autistic world of a psychopath. The most recognisable is the antisocial psychopath described by Boss (1913), characterised by passivity and lack of self-control, and the data on the quality of his individual world. Circular psychosis, schizophrenia and organic psychosyndrome can already be ruled out by conventional evaluation. In the course of the test, a certain positive development of the personality is to be expected, but there are serious forces working against this, such as the F-unbest. descriptio at the end of the test and the TA response, which emerged in the post-test during the relaxation of censorship, and which indicates a serious generational-familial conflict. In any case, psychotherapy, especially on the basis of further exploration, now gaining new thematic fodder, has an absolute indication, because it allows us to put into practice the gains that a more human, comprehensive and deeper understanding of the patient beyond the label "oligophrenia" or "postprocessual schizoidia" has given us in the extended test evaluation.]

Clinical Neuroscience

[Data for submicroscopy of panencephalitis. B Intranuclear inclusions, virus-like particles in demyelination and its course]

ÓVÁRY Imre, GOMBI Róza, BENKŐ Károly, SORSZEGI Pál, GÖDÉNY Sára, KAPPÉTER István

[In the second stage of processing of biopsy material from the right peristriatai of the right peripheral area of a patient with panencephalitis, the intranuclear inclusions found are more often classified as group II, less often as group I, according to Bouteille's classification, usually resembling a twisted cotton-mat ring with a clear halo around it. Osmiophilic, electrodense-centred, "viral-mimetic" particles are found in large numbers, most often in neurocytes. Their number suggests that the particulate is predominantly transported to the extracellular space from the disintegration of neurocytes. In our study, 27% of the particles are able to reach the CSF space. This puts the extraction of particles from liqur in perspective. The myelin degradation starts from the Ranvier's confinement and follows an axofugal direction. Electrophysiologically, as well as clinicopathologically, the study of panencephalitis is attributed to pathological informative, pathological integrative, and pathological energetic factors. Further studies are ongoing.]

Clinical Neuroscience

[Scientific meetings]

VÁMOSI Bertalan, HALÁSZ Péter, ZSADÁNYI Ottó, VELOK Gy., HIDASI J., BOCZÁN G., TOMKA Imre, SARKADI Ádám, ZETTNER Sebő, CALCAINAU C., POPESCU-TISMANA G., BUDAI R., HASZNOS Tivadar, GOMBI Róza, ENGÁRT Gizella, BOCZÁN Gábor, SZÁK János, BOCZÁN János, SZAMOSI Mária, B. SÁRMÁNY Judit, TÓTH Sándor, SZLÁVIK László, HARSÁNYI Ferenc

[Authors report on scientific sessions held at the XII EEG Congress.]

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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

[What happens to vertiginous population after emission from the Emergency Department?]

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[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

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.

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]