Clinical Neuroscience

[Meningoencephalitis in Behcet's syndrome]

DOMBAY Margit1, NÁDRAI Ágnes2

NOVEMBER 01, 1965

Clinical Neuroscience - 1965;18(11)

[Authors have detected the same disease in a pair of 32 and 20-year-old brothers. Both eyes showed chronic uveitis, acute iritis, inveterate chorioretinitis nodules. One eye of the younger boy was already completely amaurotic. The latter also had meningoencephalitis, which was not favorably affected by cortisone. No virus was isolated from fresh mouth aphthae. Based on the literature and our own observations, the neurological symptoms were presumably allergic in origin.]

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  1. Budapesti Neurologiai Klinika és II. sz. Szemészeti Klinika
  2. Budapesti Neurologiai Klinika és II. sz. Szemészeti Klinika

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Clinical Neuroscience

[Megfigyelések a mittenes alvás klinikai diagnosztikai értelmezésére vonatkozóan]

HALÁSZ Péter, NAGY A. Tibor, ZSADÁNYI Ottó, KAJTOR Ferenc

[Based on our analysis of one hundred patients with mittening, it seems that mittening sleep is indicative of a personality disorder manifested by recurrent psychic decompensations and conduct disorder at the level of organic cerebral lesions. Mitten sleep is not a disorder associated with a single clinical pathology unless it occurs in combination with the following factors : 1. a personality disorder with intermittent neurotic or psychotic decompensations or behavioural disturbances. 2. pathological vegetative phenomena. 3. diffuse cerebral lesions (ventricular dilatation and/or cortical atrophy). 4. minimum age : 15-34 years. ]

Clinical Neuroscience

[Induced paranoid psychosis]

HAITS Géza

[In addition to a brief review of the relevant literature, the author analyses two separate cases of induced paranoid psychosis in a family (mother-in-law, husband, wife, son) and a married couple. He points out the role of environmental influences and personality structure. He draws attention to the reflection of the superstitious-mystical social transmission (witchcraft) in the content of the delusions, despite the proximity of the capital, and the importance of the irrational element in the functioning of the suggestive mechanisms. ]

Clinical Neuroscience

[Nerve damage in caisson workers]

RÓZSAHEGYI István, ROTH Bedrich

[The high sensitivity of the central nervous system to decompression trauma is evident, and damage can occur not only during manifest neurological disease, but also latently, behind the onset of other forms of disease, or even without any manifest symptoms of decompression. The only effective causal therapy for decompression sickness of the central nervous system is recompression, carried out in a timely manner, with great care and, if necessary, with persistent patience. After unsuccessful recompression, the prospects for recovery are poor; permanent damage is caused and can lead to both somatic and functional psychiatric pathologies. In view of the risk of serious and permanent neurological lesions, the most careful prevention and therapy is essential. Among the preventive measures to be considered is limiting the length of time spent under hyperbaric pressure to prevent saturation of the slowly saturating lipid-containing tissues. The rate of evisceration should be strictly adhered to as prescribed. The implementation of recompression is the decisive factor in the therapy: after successful recompression, very slow decompression and, if necessary, oxygen inhalation should be used. In the compensation of occupational diseases in caisson workers, the possibility of an organic origin of functional conditions should also be considered. ]

Clinical Neuroscience

[Spinal paralysis caused by electrical trauma in cats]

KÓMÁR József, IFJ. KÓMÁR Gyula

[Authors have observed spinal paralysis in a cat caused by 220 V electric shock. The condition is thought to be related to Panse's spinal atrophic paralysis. The clinical presentation of the case is supported by histopathological studies. The expected neurological consequences of electrocution are reviewed, and the pathology and pathomechanism of electrocution are discussed. The development of Panse's syndrome is associated with the specific vascularity of the spinal cord, based on the conclusions drawn from their case. We would like to thank Ernő Guzsal, assistant professor, for the preparation of the microphotographs. ]

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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

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

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN

TUTAR Kaya Nurhan, EYIGÜRBÜZ Tuğba, YILDIRIM Zerrin, KALE Nilufer

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report – A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro­logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.

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.