Clinical Neuroscience

[Rare symptom of cervical vertebral chordoma]

HAFFNER Zsolt1, DÖMÖTÖR László1, VACZÓ György1

DECEMBER 01, 1961

Clinical Neuroscience - 1961;14(12)

[A 60-year-old man with a cervical chordoma, the clinical picture of which was fluctuant and the diagnosis of which could only be clarified by histopathological examination. Contrary to previous literature, reactive marginal sclerosis was detected in vertebra C III, with the chordoma as the primary cause. This case confirms the phenomenon that the clinical picture of vertebral chordoma is atypical and thus difficult to diagnose clinically. ]

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  1. Fővárosi István Kórház Ideggyógyászati, Kórbonctani és Röntgenosztálya

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Clinical Neuroscience

Entwicklung – Reaktion — Prozess

NIKOLA Schipkowensky

In dieser Begriffsbestimmung wird die Untrennbarkeit des somatischen und psy chischen Lebens in den Vordergrund gestellt, was allerdings nicht besagen will, sie seien identisch. Schon dadurch ist aber unterstrichen, dass die Materie das Primäre ist, der Geist — das Sekundäre ; denn Form ohne Inhalt ist undenkbar. Das Bewusstsein, als höchste Form menschlichen Daseins, darf man nicht als ein blosses Epiphänomen seines Organs hinstellen. Die psychische Tätigkeit wird zwar vom Grosshirn geleistet, sie ist aber in ihren Inhalten und in ihrer logischen Gliederung von der objektiven Wirklichkeit deter miniert, deren subjektive Abbildung sie darstellt. Die Definition verweist ferner auf den biologischen Ursprung der menschlichen Persönlichkeit, deren weitere Entwicklung auf der Verbindung von unbedingten Reflexen mit der Er arbeitung kompliziertester dynamischer Stereotypen zeitlicher Verknüpfungen beruht. Schliesslich setzt diese Definition auch die Untrennbarkeit von Psychi atrie und Neurologie voraus : Sie sind einheitliche Glieder der einen medizi nischen Wissenschaft.

Clinical Neuroscience

[On surgical indications for metastases of spinal carcinoma]

PÁSZTOR Emil, NAGY Vilmos

[Based on our experience with 40 patients with spinal metastases, we do not refrain from operating on single nodal spinal metastases and paraplegia alone is not a contraindication. The indication for surgery and the expected results depend mainly on the histological and biological characteristics of the primary tumour, the course of the disease and the general physical condition of the patient. Based on the catamnestic evaluation of operated and non-operated patients, surgery can be recommended. Since the severity of compression or paresis is significant in the postoperative outcome, early surgery is desirable. ]

Clinical Neuroscience

[Multiple sclerosis and generalised reticulosarcoma combined case]

PAPP Mátyás, KIRÁLY Ferenc

[A 63-year-old patient had multiple sclerosis for about 20 years, which progressed in relapses. 8 months before his death, he developed generalized lymph node enlargement. Autopsy and histopathological examination showed generalized reticulosarcoma throughout the body and diffuse soft tissue and perivascular tumour proliferation in the central nervous system. With minor exceptions, the pia-glia membrane inhibited spread into the neural tissue everywhere except in the corpus pinealea, which may be attributed to the specific biological behaviour of the corpus pinealea in the face of pathological processes. In the central nervous system, in addition to the tumoral proliferation occupying the perivascular spaces, we found multinodular marrow neoplasms, which correspond in their location and histological features to old multiple sclerosis foci. On the basis of the histological picture and the clinical course, the described case must be considered as an accidental encounter of two separate diseases. ]

Clinical Neuroscience

[Spiractin electroencephalography tests]

SOMOGYI István

[Studies with Spiractin have shown that: 1. in addition to its effect on the respiratory center, it has an excitatory effect on the diffuse activating system of the brainstem. 2. it can be used as a provoking agent in electroencephalography of patients with epilepsy (especially in children). 3. It may also occasionally cause generalized seizures, which should be kept in mind in cases of epilepsy.]

Clinical Neuroscience

[Report on the EEG training course in Marseille ]

WALSO Róbert

[The author reports on the EEG course in Marseille, organized by the International Federation of Societies for EEG and Clin. Neurophysiology" in Marseille from 28 August to 2 September 1961.]

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

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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.

Journal of Nursing Theory and Practice

[Assessing Quality of life of Women with Cervical Cancer After Treatment]

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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.