Clinical Neuroscience

[Lethal mumps encephalitis in a six-year-old child]

NAGY József1, JURÁSZ Gabriella1

JUNE 01, 1966

Clinical Neuroscience - 1966;19(06)

[The authors describe a case of mumpssencephalitis with lethal outcome in a 6-year-old child, together with detailed brain histology findings. The clinical picture was characterized by the absence of meningeal symptoms, the predominance of disturbance of consciousness and the late onset of neurological laesio. The parainfectious type of encephalitis is localized mainly in the immediate vicinity of ventricles III and IV, which explains the peculiarities of the clinical course. The parotid and submandibular salivary glands and interstitial inflammation of the testis are sub-symptoms of mumps and also prove the mumps origin of the encephalitis. ]

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  1. Győr-Sopron megyei Tanács Kórház Kórbonctani osztálya és Gyermekosztálya

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Further articles in this publication

Clinical Neuroscience

[Experimental porphyrism and its relevance to human disease ]

LEHOCZKY Tibor, SÓS József, SELMECY László, HALASY Margit

[Authors have induced experimental porphyria in white rats. 13 animals with allyl isopropyl acetamide carbamide (Sedormid), 6 with griseofulvin (Likuden). This is the first successful experiment in the rat, having only been done in mice. The combined results of the two experiments: in all cases (19) very severe lesions were observed in the spinal cord and cortical neurons; spongiotic degeneration was observed in the white matter of the spinal cord of 16 animals, in the hippocampus and brainstem of 4 animals; in 2 cases demyelination was seen in the Burdach tracts of the spinal cord; finally, small haemorrhages were observed in the spinal grey horns of 3 animals. These experiments have demonstrated that the clinical signs described in experimental porphyria have a solid neurological basis. Their results are consistent with the neuropathological lesions reported in human disease, and thus their experiments are experimental evidence of human clinicopathological findings. ]

Clinical Neuroscience

[Evaluation of the treatment of depression (comparative, retrospective analysis)]

VARGA Ervin, JULES Angst, MICHAEL Shpherd

[We compared 3 common somatic treatments for depressive disorders [ES, Imipramine, Monoamine Oxidase Inhibitors (MAOIs)].]

Clinical Neuroscience

[Congenital absence of cerebellum]

VERES János, BODÁNSZKY Hedvig

[In conclusion, it is a metameric developmental disorder, of which cerebellar agenesis is an extremely rare feature. Among the factors leading to intrauterine harm, heredity, X-ray, radium harm, mechanical impact and viral diseases cannot be proven. ]

Clinical Neuroscience

[Addendum to the pathology of symptomatic narcolepsy]

POHL Ödön

[The author describes the case of a 52-year-old man who had a history of typhoid narcoleptic seizures with hypnagogic hallucinations for a year. The pathological findings showed a picture of an abortive, indeterminate polyoencephalitis with the focus of the inflammatory lesion located in the area paramediana posterior to the grey matter of the periventricular grey matter of the thalamus.]

Clinical Neuroscience

[Curing phantom pain with postcentral topectomy]

DEÁK György, TÓTH Szabolcs

[The authors describe the course of their 2 patients with phantom limb pain treated with postcentral topectomy. One of their patients has been pain-free for 5 years and, to their knowledge, this is the second longest pain-free period after topectomy in the literature. The worsening of their other patient after a temporary improvement was partly related to inflammation in the stump after surgery - as a peripheral excitation - and partly to the late surgery of a patient who was alcoholic and consumed alkaloids. Based on the literature and on their own studies of patients, they argue that the essence of phantom pain is a disturbance of the body pattern that is more crucial than peripheral pathological stimuli, and that is an unusual and unpleasant experience. They recommend early cortical or subcortical surgery to eliminate the pathological body schema in cases of ineffective conservative or peripheral surgical treatment. ]

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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

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

[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.

Lege Artis Medicinae

[The Great Acceleration]

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[Silent spring]

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[8th of August was this year's Overconsumption Day; it is a model calculation of when humanity will use up the total global biocapacity for a given year. The data series and calculation methods are available for anyone to download from the Global Footprint Network website. We...]