Clinical Neuroscience

[Clinically observed retroolivarial softening]

SOÓS Imre1

DECEMBER 19, 1951

Clinical Neuroscience - 1951;4(04)

[Five clinically observed cases of retroolivar ankylosis with typhoidal history and symptomatic pathology were described. The pathology is characterized by an ictus-like onset, nodal cerebellar symptoms, crossed anaesthesia, and lacunar lesions of the bulbar nuclei on the same side as signs of damage to the posterior landscape of the olive. In our cases, by following the behaviour of sensory disturbances during the course of the disease, a certain regularity was observed, in that the initial sensory loss was replaced over time by hypersensitivity or central sensory disturbances (dysaesthesias, contraer heat sensations). Blood pressure was persistently subnormal in all our patients. Of our five patients, three had obstruction of the art. fossae. lat. bulbi described by Foix, and two had typus Wallenberg's syndrome. This fact speaks in favour of the high relative frequency of retroolivar tissue softening.]

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  1. Budapesti Tudományegyetem Elme- és Idegkórtani Klinikája

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Clinical Neuroscience

[Parietal syndromes (Gerstmann's syndrome, attitudinal diseases) in the light of conditioned reflexology]

JUBA Adolf

[Anomia, parietal agraphia and alexia, and finally ujjagnosia were found in three circumscribed skull lesions of the left parietal landscape (the fossa in case 1 affecting the junction of the gyr. angularis, gyr. supramarginalis and the peduncular segment of the 1st temporal gyrus, in observations 2 and 3 the gyr. angularis and partly the occipital cortex). The anomia can be interpreted, following the Pavlovian doctrines, in terms of increased iraddition of the stimulus, reduced concentration, and ultimately degradation of differential inhibition: patients vocalise a series of similar words but do not always reach the adaequate word trace. A similar mechanism is at work in the development of agraphia and alexia, exacerbated by the limitation of dynamic stereotypy to capture the word composed of letters. Parietal foci thus limit speech in its entirety and the pathology of the lesion cannot be seen as a negative of the normal functioning of the site. Finger agnosia in case 1 presented as a simple finger anomaly; in cases 2 and 3, where the occipital cortex was also damaged, the disorder corresponded to Gerstmann's true finger agnosia. Here, a multilayered inhibition of the conditional connections is expected, in which the relaxation of the kinaesthesia-optic junction plays a major role. In a fourth brain lesion in j.o., the foci were located in the more superior lip of the posterior segment of the right interparietal sulcus. In the aura of the epileptic seizures, there was a rotating dizziness, with an onset of contralateral adversion, all of which could be evaluated as focal symptoms. Macropsia developed during the aura and can be understood as a distortion of the optic and oculomotor connections (Pavlov), which indicate the size of objects, and developed in response to the pathological stimulus.]

Clinical Neuroscience

[Data on cerebral histological changes and their origin after CO intoxication]

FAZEKAS I. Gyula, GERÉB Tibor

[The brain of a 33-year-old man who died 3 months after CO poisoning was examined histologically with the following results: 1. There was a high degree of fatty degeneration of the cortical and medullary nuclei and endothelial fatty degeneration of both the cortical and medullary vessels with perivascular granule cell accumulation. No calcification of blood vessels was observed. 2. In addition, a marked proliferation of microglia, hypertrophy and hyperplasia of macroglia, and the transformation of microglia and oliodendroglia into granule cells were observed, accompanied by generalized watery phenomena. 3. In addition to lens opacities on both sides, diffuse patchy demyelination was found in the white matter of the cerebrum, with ring-like sparing of the vascular surroundings. Both demyelination and rings of sparing, as well as a high degree of fat deposition (fat-filled granular cells) were observed in other intact-appearing brain areas. 4 Our case suggests that white matter disease can be diffuse and extensive even in the presence of negative or insignificant lesions in the medulla. 5. We consider the anoxaemic state to be a primary factor in the pathomechanism of CO poisoning. In addition, however, there is no doubt that CO itself plays a role in the development of brain tissue lesions through its direct tissue paralysis effect. 6. Based on the histological picture of our case and recent physiological observations, we do not exclude Hallervorden's view that oedema may also play a role in the development of white matter lesions, probably by obstructing cerebral blood flow and thus increasing anoxaemia by increasing intracranial and cerebral pressure, which leads to the development of pathological lesions. The perivascular sparing rings are explained by the fact that the blood vessels in the immediate vicinity still receive enough oxygen from the circulating blood to prevent severe destruction of their tissue elements, but that the more distant tissue elements no longer receive sufficient oxygen and that the anoxic condition leads to the destruction of these tissue elements.]

Clinical Neuroscience

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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.

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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.

Clinical Neuroscience

[Is the implementation of Vojta therapy associated with faster gross motor development in children with cerebral palsy? ]

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Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

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Clinical Neuroscience

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

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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.