Clinical Neuroscience

[Sarcoma of the femoral nerve causing symptoms of "mononeuritis" ]

RÁPOLTY Zsuzsa1

SEPTEMBER 01, 1967

Clinical Neuroscience - 1967;20(09)

[A case of a 37-year-old female patient. A relatively small tumour, histologically consistent with a sarcoma, causing symptoms of femoral nerve dropout, was removed by complete transection of the nerve trunk. Surgery was performed 3 months after the first symptoms appeared. The patient has been symptom-free for 16 months so far. In our case, unilateral progressive lesions of the femoral nerve of unclear origin should be considered as tumours and surgical exploration should be considered in case of suspicion.]

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  1. Budapesti Orvostudományi Egyetem I. Neurológiai és Psychiatriai Klinikája

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Clinical Neuroscience

[Effect of anxiety on Achilles tendon reflex semi-relaxation time]

FEJÉR Arthur

[In states of anxiety, the duration of the f. r. i. of the Achilles reflex decreases, although the decrease rarely reaches the extent of the decrease in f. r. i. induced by hyperthyroidism. Both placebo injections and exam situations cause a decrease in f.r.i., but the latter effect is several times greater than the former. In a patient in a state of anxiety, 10 mg. Frenolone i.m. 1 hour after the sedative effect, a significant increase in f.r. i. is observed. This effect is significantly greater than that observed in normal controls after administration of the same dose of the drug. In hyperthyroid patients, the f.r. i. of 10 mg. Frenolone hardly alters the effect. The different behaviour of normal controls and hyperthyroid subjects from anxious patients is presumably due to the fact that Frenolone only favourably affects the central arousal associated with anxiety. During psychopharmacon therapy of anxious neurotic and psychotic patients, a gradual elongation of f.r. i. is observed as the patients calm down. This provides an opportunity to objectively monitor the effect of the therapy. ]

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[Valium (Diazepam) effects on epileptic crises and certain epileptic mechanisms]

HALÁSZ P., MOLNÁR Gy., HIDASI J.

[In addition to the generally observed good effect, we observed one case in which Valium worsened the epileptic mechanism (case 6). This observation seems to contradict the many reports in the literature that Valium has a good effect in petit mal and spike-wave mechanism. However, a distinction should be made between the different spike-wave mechanisms. In our other patient (case 3) we also observed a beneficial effect of Valium in spike-wave mechanism. However, this latter case was a spike-wave variant, slow spike-wave pattern. However, our case 6 showed a classic 3 c/s spike-wave pattern with clinical absances. It is not clear from the literature whether Valium is effective in all 3 c/s spike-wave mechanisms. Further experience in this direction is needed. Apart from the neuroleptic effect, no other "side effects" were observed. ]

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[Data for the Autogen Training methodology and indicative questions]

KORONKAI Bertalan, HORVÁTH Szabolcs

[The authors outline the main factors involved in the development, practice and spread of AT. They briefly describe Schultz's classical method. They describe in more detail the methodology they developed, which they applied to groups of 6-10 patients and in which they attach great importance to suggestive support. They share their experience with AT treatment, which they find useful for AT indicatio: all personality types except H and I are suitable for learning the exercises. Of the psychic symptoms, mood disorders, feelings of insecurity, inner tension and anxiety in particular improved, and of the autonomic disorders, sweating, tremor, hypertension, enteral disturbances, insomnia and cephalgia responded most favourably. Based on their experience, they discuss the current and perspectivicus possibilities of AT in their department. In their opinion, a gradual diffusion of AT treatment can be expected due to its advantages over other psychotherapeutic methodologies and for the practical and theoretical reasons discussed. ]

Clinical Neuroscience

[Persistent expansive-productive confabulatory image after contusio cerebri]

POHL Ödön, HAITS Géza

[The authors describe a 35-year-old male patient who developed a specific expansive confabulosis after contusio cerebri, which persisted and even progressed after the other symptoms of amnestic syndrome had resolved. The patient was re-examined 21 months after his first admission: the confabulosis persisted and was embedded in the symptoms of personality development suggestive of post-traumatic encephalopathy. The authors refer to their previous communication describing the medical history of four of their patients. Their new case analysis confirms their earlier findings and provides a catamnestic data set for this rare form of posttraumatic confabulosis. ]

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[Diffuse demyelinating giant cell reticulomascoma metastases ]

ÉDER Mária

[In a circumscribed case of metastatic reticulum sarcoma, severe oedema and demyelination were observed in both hemispheres. In the clinical picture, local symptoms attributable to the direct effect of the tumour were accompanied by bilateral hemisphaerial white matter lesions. In all cases of brain tumours, but especially in sarcoma metastases, bilateral symptoms should be considered not only bilateral tumour formation but also bilateral white matter oedema-necrosis.]

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Clinical Neuroscience

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Clinical Neuroscience

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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.