Clinical Neuroscience

[Rarely recognised peripheral symptoms on the upper limb]

SZŐKE Tamás, HAFFNER Zsolt

JANUARY 01, 1968

Clinical Neuroscience - 1968;21(01)

[It is quite common to find such diagnoses in the medical reports of the STD specialists, in the final hospital reports; brachialgia, ischialgia, radial paresis, ulnar paresis, etc. without an aetiological analysis of the symptoms thus indicated. And then almost stereotypical therapias suggestion: B, and B, vitamin inject. Most of the time there is not even the need for aetiological clarification. This is particularly surprising when we think: in the case of abducens, oculomotorius, facial paresis, etc., we are far from being satisfied with the indication of the symptom in the diagnosis, but try to exclude as many pathologies as possible: neoplasm, aneurysm, diabetes, lues, arteriosclerosis, etc. ]

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Clinical Neuroscience

[Pemphigus cases with lesions found in the spinal ganglia ]

BALÓ József

[Based on our experience with zoster cases, we have examined the spinal ganglia of 82 cases of pemphigus over the last 20 years to see if there are any phenomena that could explain the skin lesions. The lesions found, partly macroscopic but mainly histopathological, suggest that such a link between lesions in the spinal ganglia and skin disease exists. In addition to the acute signs of inflammation, there are also lesions that can be classified as chronic, such as those involving nerve fibres, nuclei, supporting tissue of the ganglia and lesions of the meninges. Diseases of the spinal ganglia are projected onto the skin, which makes pemphigus a cutaneous trophoneurosis. In addition to the morphological phenomena, the question is what aetiological factors are involved in its creation. This remains to be determined in the future. ]

Clinical Neuroscience

Sur la sémiologie des idioties amaurotiques du type Tay-Sachs en survie prolongée

L. van Bogaert, J. J. Martin

Etude clinique d'une idiotie amaurotique de Tay-Sachs à évolution prolongée sous l'angle des signes de décérébration, des réflexes primitifs et des manifestations d'automatisme médullaire.

Clinical Neuroscience

Observations upon the So-colled Idiots Savants

CRITCHLEY Macdonald

In drawing the foregoing generalisations it is necessary to realise their limitations. We must agree with the conclusions arrived at by Mitchell, who recognised at least three psychological categories: (1) the “calculating prodigies — who may be persons of inferior intellectual calibre and who rely upon ingenious shortcuts; (2) arithmetical prodigies like Colburn, and Dase, with a moderately well developed knowledge of arithmetic; and (3) mathematical geniuses, such as the elder Bidder. These are endowed with exceptional abilities, and their knowledge of pure mathematics is profound.

Clinical Neuroscience

Thorium granulomas in the brain

FREEMAN Walter

Thorotrast was used in 1936 and 1937 to demonstrate the lesions of prefrontal lobotomy. Four patients came to autopsy after 10-22 years, and in each, one or more thorium granulomas were found. These masses ranged from 6 X 8 mm to 8X12 mm in size, were composed of hyaline material enclosed by a thick capsule of mixed connective and glia tissue, and surrounded in part by large phagocytes filled with thorium dioxide particles. Dense connective tissue developed in sulci where thorotrast escaped into the subarachnoid spaces, and marked gliosis with desquamation of the ependyma occurred when it entered the ventricles. The phagocytes in the cases with longer survival often showed vacant cavities where the nuclei should have been. Neurons in the vicinity showed no obvious lesions. The material was described as containing "a very strong thorium source.” It is believed that the alpha particles given off by the thorium are responsible for the formation of the granulomas and, after many years, for the death of the phagocytes. Thorium can safely be used in the brain only for the demonstration of cysts and abscesses which can then be completely removed. A case of such employment was described by Lehoczky in 1939.

Clinical Neuroscience

Télangiectasies de la moelle dorsale révélées à l'âge de 75 ans par une myelopathie transverse, avec une digression sur l'atrophie spinale segmentaire

GARCIN Raymond, LAPRESLE Jean

L'observation que nous rapportons tire son intêret de la révélation extrêmement tardive (75 ans) d'un angiome de la moelle de type capillaire, resté jusque là cliniquement muet. Elle illustre la longue latence possible des mal formations vasculaires de la moelle, et elle montre qu'il faut toujours penser à cette étiologie devant une affection médullaire dont la cause nous échappe.

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

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Clinical Neuroscience

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

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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

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

Clinical Neuroscience

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

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Clinical Neuroscience

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

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[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.]