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

JANUARY 01, 1968

Clinical Neuroscience - 1968;21(01)

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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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

[Maladies infectieuses extraneurales du système nerveux complications]

KÖRNYEY István

[Overview of neurological complications of common infectious diseases it is most useful to start from the following classification of encephalomyelitis based on its pathophysiological features, although only certain types are associated with our thymus: 1. meningo-encephalitis; 2. metastatic nodular encephalitis (abscess); 3. diffuse encephalitis, mainly involving the cerebral cortex; 4. polioencephalomyelitis, with pre-dilection areas of the brainstem disease of the prefrontal lobes of the brain; 5. panencephalitis; 6. leukoencephalitis.]

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Clinical Neuroscience

Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Lege Artis Medicinae

[Cardiovascular prevention 2021 – Guidelines of European Society of Cardiology 2021. Cardiovascular prevention at individual level]

VÁLYI Péter, KÉKES Ede

[In August 2021, the European Society of Cardiology (ESC) published its practical guidelines for cardiovascular prevention. In Part 1 of our publication, we discussed cardiovascular risk factors, risk assessment, cardiovascular risk assessment in presumably healthy individuals of different ages, people with proven atherosclerotic cardiovascular disease, people with diabetes mellitus, and factors influencing risk. In Part 2, conditions that influence cardiovascular risk were detailed. In the current Part 3 of this publication, we review personalised cardiovascular prevention, non-pharmaceutical and pharmaceutical treatment of specific risk factors, emerging treatment options, and par­ticipation in cardiovascular rehabilita­tion and prevention programmes based on ESC guidelines.]

Clinical Neuroscience

Diagnostic values of conventional conduction parameters in ulnar neuropathy at elbow

ALEMDAR Murat

Conventional parameters used in electrodiagnosis of ulnar neuropathy at elbow (UNE) are: (i) absolute across-elbow ulnar nerve motor conduction velocity (MCV), (ii) reduction rate of composed muscle action potential (CMAP) amplitude from above to below elbow stimulation, and (iii) MCV difference between forearm and across-elbow segment. We aimed to search the diagnostic accuracy values of these parameters on UNE, and their correlations with axonal dysfunction of ulnar nerve fibers. Arms with clinical signs of UNE and two-fold healthy controls were included. We detected the best cut off points of the measured parameters and their possible combinations. Their diagnostic accuracy values and correlations with parameters reflecting the axonal functions were analyzed, statistically. Totally, 118 arms with UNE and 236 controls were included. Absolute across-elbow MCV yielded a higher accuracy than MCV difference and reduction rate of CMAP amplitude (p = 0.010 and p˂0.001, respectively). Besides, combining it with other parameters did not increase the diagnostic yield. Correlation analyses revealed that the only parameter having positive linear correlations with sensory nerve action potential amplitudes both in the control and the disease groups is the absolute across-elbow MCV. The absolute across-elbow MCVs have also positive linear correlation with CMAP amplitudes in disease group. The absolute across-elbow MCV is the most valuable conventional parameter for the electrodiagnosis of UNE. It is also the most correlated parameter with the electrodiagnostic parameters reflecting the axonal functions of the ulnar nerve fibers.

Clinical Neuroscience

[European treatment recommendation of neuromyelitis optica spectrum disorders: critical remarks and case discussion]

KÕSZEGI Edit, ILLÉS Zsolt

[Neuromyelitis optica is a demyelinating disease of the central nervous system mediated by antibodies against the waterchannel aquaporin4 (AQP4). In a number of cases the clinical manifestation is spatially limited. Such events of separate longitudinally extensive transverse myelitis (LETM) or relapsing/bilateral optic neuritis (RION/BON) are defined as NMO spectrum diseases. The diagnosis is further challenged by anti-AQP4 seronegative cases. While chronic immunosuppressive therapy should be introduced in definitive NMO, treatment strategy of the NMO spectrum is less defined. Recent EFNS guidelines recommend chronic immunosuppressive treatment of NMO spectrum diseases depending on the clinical course even in AQP4-seropositive cases. Presenting a case with relapsing optic neuritis, here we emphasize the importance of early immunosuppressive therapy in all seropositive NMO spectrum diseases regardless of relapse severity, in order to prevent an upcoming devastating relapse, i.e. NMO conversion.]