Clinical Neuroscience

[Endoneural collagenosis in orbital neurofibroma]

HARASZTI Antal1

NOVEMBER 01, 1960

Clinical Neuroscience - 1960;13(11)

[1. We described an orbital neurofibroma with the tissue pattern of hypertrophic neuritis. 2. In our case, neurofibroma was supported by the fact that neurofibroma had been previously removed from the same region, that the images resembling onion sections were localized only to some nerve fibres and that the connective tissue of the perineurium was also increased, and that clinical examination did not find lesions in other nerves. 3 We agree with Krücke's view that concentric fibre sclerosis is a secondary phenomenon and a consequence of serous infiltration. In addition, the two processes were observed side by side. 4. concentric fibrous sclerosis is not specific for hypertrophic neuritis but also occurs in neurofibromas. ]

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  1. Debreceni Orvostudományi Egyetem Kórbonctani Intézete

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Clinical Neuroscience

[Data for arthrolysis syringomyelica]

IVÁN László

[In reporting our case of rare and severe arthrolysis syringomyelica, we aimed to contribute to the literature on syringomyelia and to the practical diagnostic and theoretical problems that arise. ]

Clinical Neuroscience

[Epileptiform EEG manifestations in schizoform psychoses]

MAGYAR István, WALSA Róbert

[Authors use clinical and EEG phenomena of 16 patients to study the problem of the relationship between epilepsy and schizoform psychosis. After a careful analysis of all the data, 11 cases were diagnosed with epilepsy, 4 with schizophrenic psychosis and 1 with a coexistence of epilepsy and schizophrenia. They conclude that the two pathologies (a) are likely to develop from the same pathological basis in cases with structural lesions of the subcortical (temporo-limbic or related) area, (b) in some rare cases, the two pathologies may co-exist, (c) they draw attention to the non-epileptic "pseudo-paroxysms" that often colour schizophrenic psychosis, (d) they stress that a definitive opinion which will determine the correct pathology and the therapy to be followed - can only be made on a case-by-case basis, after careful analysis of the phenomena. ]

Clinical Neuroscience

[Report on my study trip to Poland]

ORTHMAYR Alajos

[Author reports on his study trip to Poland.]

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[Transthyretin familial amyloid polyneuropathy - three Hungarian cases with rare mutations (His88Arg and Phe33Leu)]

CSILLIK Anita, POZSONYI Zoltán, SOÓS Krisztina, BALOGH István, BODÓ Imre, ARÁNYI Zsuzsanna

[Introduction - Transthyretin familial amyloid polyneuropathy is a rare autosomal dominant progressive systemic disesase of adults caused by endoneural amyloid deposition due to point mutations of the transthyretin gene. It is the most severe form among hereditary polyneuropathies, being fatal within 10 years if left untreated. The disease is underdiagnosed, the late onset forms (above the age of 50) being probably more widespread than previously thought. Early diagnosis is essential as the early introduction of causal therapy (tafamidis) slows progression and prolongs survival. Patients - We report here three non-related Hungarian cases of transthyretin familial amyloid polyneuropathy with non- Val30Met mutations (His88Arg in two cases, Phe33Leu in one case). They were all characterized by late-onset, progressive, length-dependent, axonal, sensorimotor polyneuropathy and the simultaneous presentation of severe restrictive cardiomyopathy. In all three cases, clinical and electrophysiological signs of myopathy were also present, suggesting the involvement of skeletal muscles as well. In two cases, high resolution ultrasound of the peripheral nerves was also performed, which showed segmental structural alterations (change or loss of fascicular structure) and some increase of echogenicity of the interfascicular epineurium, without substantial enlargement of the nerves. Conclusion - In Hungary, mainly the rare, non-Val30Met mutation forms of transthyretin familial amyloid polyneuropathy are encountered, as in our cases. As opposed to the Val30Met forms, these mutations are characterized by late onset and simultaneous presentation of severe cardiomyopathy. Our report highlights the importance of considering transthyretin familial amyloid polyneuropathy in the differential diagnosis of late-onset, progressive, axonal polyneuropathies of unknown etiology, particularly if associated with cardiac disease.]

Clinical Neuroscience

Secretory meningioma with bone infiltration and orbital spreading

KÁLOVITS Ferenc, TAKÁTS Lajos, SOMOGYI Katalin, GARZULY Ferenc, TOMPA Márton, KÁLMÁN Bernadette

Secretory meningioma is a rare form of meningiomas which differentiates from the meningothelial subtype. It is characterized by significant peritumor edema and distinct immunohistochemical and molecular genetic profiles. We present a middle aged female patient with secretory meningioma infiltrating the orbital bone from the primary cranial base location and causing exophthalmos, features rarely described with this tumor. Surgical resection was challenging because of the associated brain swelling and rich vascularization of the tumor. Imaging and immunohistochemical studies revealed characteristic hallmarks of secretory meningioma. While histologically it was a benign tumor, due to the orbital bone and soft tissue infiltration, postoperative management of neurological sequelae was challenging. This case highlights distinctive clinical, imaging and histological features along with individual characteristics of a rare form of meningiomas.

Clinical Neuroscience

Vitreous humor diffusion measurements from diffusionweighted imaging in idiopathic intracranial hypertension

CAGLI Bekir, TUNCEL Alpaslan Sedat, YILMAZ Erdem, TEKATAS Aslan, ERMIS Veli

Background - Idiopathic intracranial hypertension is a disease with uncertain etiology. It is not caused by an intracranial mass lesion or hydrocephalus and is characterized by abnormal elevation of intracranial pressure and normal composition of the cerebrospinal fluid. The orbita and intracranial area are closely related anatomically. Elevated intracranial pressure can be transmitted to the orbita through the cerebrospinal fluid around the optic nerve sheath changes at the vitreous humor on diffusion-weighted imaging have not been systemically studied in idiopathic intracranial hypertension. Purpose - The purpose of this study was to investigate diffusion changes in the vitreous humor in patients with intracranial hypertension. Methods - In this retrospective study, 25 patients with papilledema and who had been definitively diagnosed with idiopathic intracranial hypertension and 20 control participants were evaluated. Control subjects and patients were scanned with a 1.5 Tesla magnetic resonance imaging. Apparent diffusion coefficient maps were obtained from diffusion-weighted imaging with a b value of 1000 s/mm2 and apparent diffusion coefficient values were automatically calculated. These images were obtained by a radiologist who was blinded to the details of the study for center of each vitreous humor and the body of lateral ventricle. The mean apparent diffusion coefficient values of each vitreous humor and the body of the lateral ventricle were calculated for each group (control group and patients) and quantitative comparisons were performed. Results - There were no statistically significant differences in mean apparent diffusion coefficient values of the right vitreous humor, left vitreous humor and the body of the lateral ventricle between the patients with idiopathic intracranial hypertension and the control group (p=0.766, p=0.864, p=0.576, respectively). Discussion - Vitreous humor is a closed system and has no direct relationship with the cerebrospinal fluid or cerebral tissue and although morphological changes occur in the orbital structures, including the optic disk and optic nerve in idiopathic intracranial hypertension, the indirect effects of these changes on the vitreous humor may be too subtle to measure. Conclusion - We did not find a significant difference in the mean apparent diffusion coefficient value of the vitreous humor between the patients with idiopathic intracranial hypertension and the control group. However, future studies will be necessary to determine if changes in the vitreous humor can be used to diagnose intracranial hypertension.

Clinical Neuroscience

[Meningioma patients with some electrographic characteristics]

TOMKA Imre, BAUMANN Piroska

[Authors present EEG data from 122 meningioma patients. Of these, all but 6 were diagnosed by surgery or dissection. Locally, significant nodal activity was mainly provided by tumours with frontal location, followed by those with olfactory, occipital and temporal localisations. In contrast, the EEG abnormalities of centro-parietal, parasagittal meningiomas are mild but often diffuse and bilateral. No correlation was found between the background activity of electrical activity and the increase in brain pressure expressed by congestion in the orbital floor. Nor can the degree of relaxation of cerebral electrical activitas be used to infer surgical prognosis. Localisation diagnosis based on EEG was completely or approximately accurate in 75% of cases. However, in 14 cases the EEG opinion was insufficient or negative. The majority of these cases were centro-parietal parasagittal, posterior scalar or deep tumours. In 74% of patients with nodal activity before surgery, a significant reduction of nodal lesions was observed on the catamnestic curve, but complete negativity was observed in only a few cases. In contrast, excitatory or epileptiform activitas after surgery became appreciably more pronounced with a significant reduction of the slow foci in the majority of cases. ]

Clinical Neuroscience

[Ocular fundus lesions in cases of panencephalitis nodosa]

SZABADI Elemér, OLÁH Imre

[In 130 cases of panencephalitis nodosa, abnormal orbital lesions were found in 61 patients (47%), 39 (76.5%) of 51 cases in the Neurology Clinic. In 79 and 76% of the fundus lesions, respectively, the papilla and 21-24% of the extrapapillary lesions were visible. In 14 cases, yellowish-white dots similar to old-age lipoid deposits were observed near the aequator. No parallel between the course of panencephalitis and the fundus image can be drawn on the basis of previous studies.]