Clinical Neuroscience

[Vascular mechanisms in the pathological variations of subacute progressive panencephalitis]


OCTOBER 01, 1959

Clinical Neuroscience - 1959;12(10)

[On the basis of 15 clinically and pathologically reviewed cases, the author considers that there is no firm basis for distinguishing between Dawson's inclussios, Pette-Döring's pan-, and van Bogaert's subacute sclerotoid leukoencephalitis and therefore proposes the name subacute progressive panencephalitis. The disease (group) has a wide spectrum of pathological variation and is not considered to be a direct consequence of the underlying inflammatory process, but a product of complicating anoxic-vascular disorders. By presenting 3 cases, he demonstrates anoxic-vascular effects ranging from a few days of cortical erythema and transudative damage to several years to months of ulegyria. With respect to the formal genesis of anoxia-vascular injury, he considers the complex interplay of cardiac, circulatory, respiratory, and local intracranial cavity vascular tamponade, which may be compounded by cytotoxic effects due to liver dysfunction.]


  1. Országos Ideg-és Elmegyógyintézet



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Clinical Neuroscience

[Changes in protein fractions in blood after partial removal of the cerebral cortex and lesion of certain areas of the central nervous system (hypothalmus, formatio reticularis)]

MAROS Tibor, KOVÁCS Endre, MÓDY Jenő, LÁZÁR László

[The authors have observed how damage to different parts of the central nervous system causes changes in the protein fractions in the blood. Their experimental studies in 57 dogs show that partial removal of the cerebral cortex produces only temporary changes in this respect. These transient changes are not specific but are rather a consequence of the general trauma associated with surgery. Injury to the hypothalamus results in permanent shifts in alpha and beta globulins, while electrical disruption of the formatio reticularis adjacent to the raphe mesencephali produces absolute and relative decreases in albumin and significant increases in alpha 2 and gamma globulin fractions. The authors conclude that there are centres and pathways in the hypothalamus and along the brainstem formatio reticularis that control protein metabolism, the closer delineation of which requires further research. ]

Clinical Neuroscience

[Right complete hemihypertrophy with schizophrenia associated with schizophrenia]

ADORJÁNI Ferenc, BŐHM Tivadar

[Hemihypertrophy (hh.) is the enlargement of one half of the body relative to the other half. In complete hh., this enlargement extends from the skull to the toes and includes bones and soft tissues. The first clinical study of hh. was published by Wagner (37) in 1839, followed by Friedberg (6) in 1867 who described crossed hypertrophy (h.). Gesell (9, 10) reviewed the literature of cases published up to that time in 1921 and 1927, followed by Lenstrup (20), Wakefield and Hines (38), Petre (26), Schwartzmann and colleagues (36), E. Toussaint Aragon (1), Sayer and Fatherre (32), Rugel (29), Ward and Lerner (39) described their patients. According to Sylver and Gruskay (34), no fewer than 130 cases were reported in the literature up to 1957. ]

Clinical Neuroscience

[The role of cash benefits for people with mental illness therapeutic employment of mental mental patients]

TÖRÖK István, LESCH Gyula

[It is a long-established fact that reasonable employment is beneficial for the mentally ill. Institutions with a long tradition, such as the Soviet institutions or the Polish Berenice, the East German Goerden, the West German Gütersloh and Lengerich, the Czechoslovak institutions and many others, have developed therapeutic occupations and systems based on experience. Here, unfortunately, therapeutic employment is still in its infancy. But it would also be of benefit to the national economy. ]

Clinical Neuroscience

[Book review]


[The author reviews Klinische Psychotherapie innerer Krankheiten.]

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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

Atypical presentation of late-onset Sandhoff disease: a case report

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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

[Christmas inventory]

BRYS Zoltán

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Clinical Neuroscience

Neuroscience highlights: The mirror inside our brain

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Over the second half of the 19th century, numerous theories arose concerning mechanisms involved in understanding of action, imitative learning, language development and theory of mind. These explorations gained new momentum with the discovery of the so called “mirror neurons”. Rizzolatti’s work inspired large groups of scientists seeking explanation in a new and hitherto unexplored area of how we perceive and understand the actions and intentions of others, how we learn through imitation to help our own survival, and what mechanisms have helped us to develop a unique human trait, language. Numerous studies have addressed these questions over the years, gathering information about mirror neurons themselves, their subtypes, the different brain areas involved in the mirror neuron system, their role in the above mentioned mechanisms, and the varying consequences of their dysfunction in human life. In this short review, we summarize the most important theories and discoveries that argue for the existence of the mirror neuron system, and its essential function in normal human life or some pathological conditions.

Clinical Neuroscience

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

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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.