Clinical Neuroscience

[Diffuse primary leptomeningeal gliomatosis in Von Recklinghausen' disease: presentation with ischemic myelopathy]

ISTVÁN Vajtai1, ÁDÁM Kuncz1, ZSUZSANNA Varga1, ERIKA Vörös1, MIHÁLY Bodosi1

MAY 20, 1996

Clinical Neuroscience - 1996;49(05-06)

[A case of diffuse primary leptomeningeal gliomatosis, complicated by ischemic myelopathy is presented. Symptoms developed acutely in a 13 year-old girl with neurofibromatosis type 1, previously manifesting only in kyphoscoliosis and typical melanotic skin dots. Autopsy revealed extensive involvement of the cerebrospinal leptomeninges by a low-grade (pilocytic) astrocytoma, with secondary thrombosis of the posterior spinal arteries. There was no evidence of parenchymal involvement by the tumour. Histopathologic features of primary glial neoplasms of the leptomeninges and major traits of their symptomatologic correlates are discussed. With regard to problems of diagnostic access to nervous system neoplasms associated with phacomatoses, hazards of prognostic evaluation are envisaged.]

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  1. Szent-Györgyi Albert Medical School, Szeged

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Clinical Neuroscience

[Somatic defects and pain manifested at adults' drawings of a man]

GRYNAEUS Tamás

[The outhor observed that “draw-a-man tests” of mentally retarded or demented adult patients may truly reflect their somatic defects or painful disorders. Drawings of mentally intact patients could also exhibit their somatic illness if it causes pain or the compensating mechanisms are insufficient. A brief discussion is given on the possible mechanisms, in connection with the body-schema.]

Clinical Neuroscience

[Topographic pattern of spike-waves in absence seizures]

CLEMENS Béla

[Topography of generalized ictal spike-wave patterns (absence seizures) have been investigated by voltage mapping analysis. The 31 patients investigated in this study belonged to a wide variezy of epileptic syndromes with absence seizures. Previously described different topographic patterns of the spike and the wave components have been confirmed in this study.]

Clinical Neuroscience

[The effect os levodopa in Parkinson's disease ]

MECHLER Ferenc, DIÓSZEGHY Péter, GLAUB Theodóra, HIDASI Eszter, JOSE Rosa

[The effect of carbidopa/levodopa on clinical signs and laboratory data was studied in 21 patients with Parkinson's disease during a follow-up period of 7-12 months. The Columbia and Webster scales were used for analysis. The improvement was significant already in the first weeks of the treatment. The drug did not influence the laboratory data and the side effects were found to be insignificant. The changes due to the drug were analysed by electrophysiological methods. Certain peak amplitudes and the area of visually evoked responses increased significantly during the treatment suggesting facilitation in the function of visual pathways. On stimulating the motor system by transcranial and spinal magnetic stimulation, the motor latencies and the duration of silent period showed normalization. ]

Clinical Neuroscience

[The prognostic relevance of apoptosis in medulloblastoma]

SCHUBERT EO Thomas, CERVOS-NAVARRO Jorge

[Apoptosis is known to be a phenomenon of prognostic significance in certain neoplasms. Several previous studies have indicated that the prognosis of desmoplastic medulloblastoma is better than that of classical medulloblastoma. We studied 5 desmoplastic and 5 classical medulloblastomas by in situ end labelling of DNA strand breaks to see whether there is any difference in amount or pattern of apoptosis in these subtypes, correlating to the different prognostic behaviour. We found no significant difference neither in amount nor in pattern of apoptosis between the two subtypes. Our results indicate that apoptosis may not be a relevant variable in the prognosis of medulloblastoma.]

Clinical Neuroscience

[Thrombolytic therapy in acute ischemic stroke (preliminary report)]

HAFFNER Zsolt, HORVÁTH Erzsébet, HORVÁTH Éva, PAPP Gabriella, EGERVÁRI Ágnes, LIPÓTH Sarolta, CSÁNYI Attila, TUKA Andrea

[Intravenous stretiokinase infusion therapy was performed on four patients in acute occulsive cerebrovascular disease (hemorrhage was rxcluded by CT scan). The thrombolytic therapy was started not later than six hours after the onset of the first symptoms. Kabikinase (1 250 000 IU) was administered in intravenous infudion during two hours. After thrombolysis no prolonged anticoagulant therapy was applied. Improvement of the neurological symptoms was impressive and convincing: in two cases immadiate. Ino one case we could be observed during the treatment (2 hours) nor after it. The possibility of thromolytic therapy in acute occulsiv stroke should be take into consideration. So far as we are aware this is the first time that thromolysis is occulsive stroke according to a protocol like this has been used in Hungary.]

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Clinical Neuroscience

Fatal outcome of cervical myelopathy caused by fibrocartilaginous embolism. Rare cause of spinal vascular damage

FOLYOVICH András, HAVAS László, VADÁSZ Gizella, FEHÉR Ágnes, VADASDI Károly, SZABÓ Zsolt, TÓTH Kornélia, BÉRES-MOLNÁR Anna Katlin, TOLDI Gergely

Fibrocartilaginous embolism is a rare cause of ischemic myelopathy. Authors report a case of a 39-year-old woman with progressive tetraparesis and severe autonomic dysfunction. Despite of the detailed examinations, the definite diagnosis was verified by autopsy. The patient was admitted because of progressive pain and numbness of the upper extremities and tetraparesis. Hypotonic muscles of the lower extremities with mild tetraparesis were observed. Magnetic resonance imaging showed an intramedullary lesion at the level of the cervical V-VII vertebral. Patient’s tetraparesis worsened gradually to plegia with urinary retention. Expansive, rapidly progressing multiple decubiti developed, which were resistant to therapy. In spite of the complex therapy, the patient died. No internal disease was found to explain the death by autopsy. Multiple subacute infarctions of the cervical myelon (involving the lateral columns as well) in the territory of the anterior spinal artery were verified by neuropathological examination. The occluded vessels were filled by a material containing cartilaginous cells, while signs of atherosclerosis or thrombosis were not present. Cartilaginous embolism of spinal arteries was diagnosed.

Lege Artis Medicinae

[NEUROFIBROMATOSIS, MALIGNANT MELANOMA AND HYPERTHYREOIDISM IN A HCV POSITIVE PATIENT]

FOLHOFFER Anikó, HORVÁTH Andrea, CSÁK Tímea, NÉBENFÜHRER László, TELKES Márta, IVÁNYI András, SZALAY Ferenc

[INTRODUCTION - Similar occirrence of neurofibromatosis and malignant melanoma is rare. We report a patient with neurofibromatosis, cutaneous melanoma, hyperthyreoidism and HCV positivity. CASE REPORT - A 43-year-old woman has been under care for neurofibromatosis for 16 years when she presented with increased serum ?- glutamyltransferase, alkaline phosphatase activity and anti-HCV antibody positivity at regular checkup. A pigmented lesion removed from her back histologically proved to be cutan melanoma. Interferon treatment was applied. She lost 8 kilograms in half a year which was caused not by the tumor progression, but hyperthyreoidism. A rapid clinical and laboratory improvement was observed for thyreostatic treatment and she regained her bodyweight. One year later she presented with a cough caused by pulmonary tumor. The tumor was surgically removed and histologically diagnosed as metastasis of melanoma. Cytostatic treatment was applied and she became asymptomatic. Four years after the diagnosis of melanoma she died of apoplexy. During the autopsy there was no sign of either melanoma or liver disease. CONCLUSIONS - The careful investigation of skin should be emphasised even in case of long established neurofibromatosis. The presented case shows an association of malignant melanoma and neurofibromatosis. In the background of loss of bodyweight even in a patient with history of malignant disease other causes should also be searched such as hyperthyreoidism especially during interferon therapy.]

Clinical Neuroscience

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.