Clinical Neuroscience

[Surgical outcomes of brain aneurysms ]

OROSZ Éva1, VAJDA János1, JUHÁSZ János1, SLOWIK Felicia1

MAY 01, 1986

Clinical Neuroscience - 1986;39(05)

[The authors evaluate the records of patients operated on for cerebral aneurysms at the National Institute of Neurosurgery over a period of 8 years (1977-1984) using computer processing. The overall mortality of 894 operations is 15%. They examined the role of patient age, sex, preoperative status, aneurysm location, time between bleeding and surgery, and surgical complications, as well as spasm on angiography, in the outcome and evaluated the sectional findings of patients who died. Special attention was paid to the evaluation of late ischaemic complications. The most important factor for surgical outcome appears to be the patient's preoperative condition and the appropriate timing of surgery. ]

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Clinical Neuroscience

The independence of cycloid psychosis from schizophrenia and affective psychosis

CARLO Perris

A short survey of the evidence supporting the independence of cycloid psychotic disorders from both schizophrenic and manic-depressive disorders is presented. Converging results from different quarters support the view that the concept of cycloid psychosis has represented a kind of blind spot in the search of a solution to a correct identification of psychotic conditions which do not fit into current conceptions of schizophrenia and of bipolar manic-depressive psychosis. A warning is also given against the identification of cycloid psychosis with schizoaffective psychosis and the risk of letting the former follow the nosological uncertainties of the latter concept.

Clinical Neuroscience

[Agoraphobic and panic neurotic patients management of respiratory control]

KOPP Mária, MIHÁLY Katalin, TRINGER Katalin, VADÁSZ Péter

[We report on a domestic adaptation of the cognitive-behavioural therapy for panic syndrome and agoraphobia with panic attacks described by Salkovskis and Clark (1984), based on the treatment of fourteen patients. During the six-week treatment period, the number and intensity of panic attacks and the level of anxiety reflecting their subjective state were significantly reduced. At the beginning of treatment, an abnormal breathing pattern was observed in the group of patients, and changes in autonomic parameters were well monitored during treatment. Among the questionnaires translated for the diagnostic study, the Anxiety and Panic Attack Diary seems to be a good measure for monitoring the effect of treatment, and the Hyperventilation Symptom List and the Marks and Mathews Fear Questionnaire may be useful tools for grouping patients. ]

Clinical Neuroscience

[Aseptic sinus thrombosis with a predominantly benign course]

SÁTORI Mária, GÁCS Gyula

[The authors studied 7 cases of sinus thrombosis among 1390 patients in a 2-year study of a neurology department, of whom only 1 patient died. A significant proportion of patients had a coagulopathy leading to thrombosis. The authors attempt to outline the symptom clusters and unusual symptom associations that may underlie suspected benign sinus thrombosis. It is likely that fatal sinus thromboses due to various coagulation disorders may be preceded by mild attacks with a warning course similar to TIAs that predict ischaemic events. ]

Clinical Neuroscience

[The dexamethasone suppression test in psychiatric practice ]

SZÁDÓCZKY Erika, ARATÓ Mihály, RIHMER Zoltán, MOUSSONG-KOVÁCS Erzsébet

[The authors examined dexamethasone suppression test results in 186 patients diagnosed according to DSM III criteria and treated in an open psychiatric ward. The test was found to have a relatively high sensitivity (61%) and a relatively low specificity (64%) for major depression. Abnormal test results above those reported in healthy individuals were observed in a group of patients with dysthymia and panic. A close association of these two categories with major affective disorder is assumed.]

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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

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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.

Clinical Neuroscience

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

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Clinical Neuroscience

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Lege Artis Medicinae

[The author’s response to the comment on “Exploratory study of outcomes of blood sample mass examinations by rank correlations”]

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.