Multiple intramedullary neurinomas of the spinal cord. Case report.
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Biomedical subjects
Publications and source records attributed to G Iraci.
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The case is presented of a child with early manifestations of Hand-Schüller-Christian disease limited to exophthalmos, and rapid final progression towards a clinical picture dominated by intracranial hypertension and cerebellar involvement, caused by massive histiocytic infiltration of the cerebellum, combined with discrete subdural deposits of xanthomatous tissue and minor deposits in other parts of the central nervous system and other organs.
Six patients (3 couples of brothers) with adhesive arachnoiditis of the opto-chiasmatic cistern, surgically verified, are presented. Only one couple of brothers offered a possible family history of a similar condition in one maternal uncle. The visual damage had almost constantly a sudden onset and was predominantly of the axial type. No gross pathology, other than the arachnoiditic involvement of the chiasmal cistern and alterations of the anterior optic pathways, was found at surgery. Pneumocisternoencephalographic findings did not prove constantly reliable for a preoperative diagnosis of the condition when compared with surgical pathology. The overall results of the neurosurgical operations (exploration of the chiasm and removal of the adhesions) can be considered as favorable and this type of treatment is once again recommended as the therapy of choice without undue delay when general or local medical treatment proves of no avail after a reasonable length of time.
Cysts of Rathke's cleft are considered as benign lesions, a practically permanent cure of which can be achieved by aspiration of the fluid content and ample opening of the capsule. The case of a patient who suffered from a recurrence of the cyst, 13 years after a first operation carried out according to such surgical criteria, is reported in order to signal the actual possibility of recurrence of such lesions.
Three cases of an infrequent cerebral vascular malformation, venous angioma, are reported. The first 2 of these cases, with the lesion in a supratentorial location, exhibited exophthalmos as a presenting symptom, together with palpebral or intraobital angiomatous formations, and also impairment of visual function. The first patient was operated upon and the "venous angioma" could be confirmed histologically. An angiographic follow-up of the same patient at an 18 year interval was also possible. The diagnostic work-up to be performed in cases of exophthalmos is discussed and the need for angiography is stressed.
A patient, previously operated upon for a supra-and retrosellar arachnoid cyst, had again to undergo surgery for a recurrence and died in the early postoperative course. The gross and microscopic pathologic evidence obtained through necropsy is described: it seems to confirm the inflammatory nature of the arachnoid cyst, thus legitimating the term cystic optochiasmatic arachnoiditis used for the description of the same case in a previous report.
A case of ectopic pinealoma is reported. It presented with the clinical triad of visual disturbances of the chiasmatic type, diabetes insipidus, hypothalamic-hypophyseal impairment, and with enlargement of the sella turcica on roentgenographic examination. The patient is alive and well eight years after incomplete removal followed by radiation treatment. It is felt that this is the correct treatment for such lesions, rather than radiation treatment alone as apparently favoured in other reports. The diagnostic and therapeutic points supporting such an opinion are discussed.
Adhesive arachnoiditis is an occasional finding during neurosurgical operations for pituitary adenoma, previously treated by radiation therapy. A case where an arachnoidal process was found in combination with an eosinophilic pituitary adenoma, which had never been treated by irradiation, is reported. A 44-year-old woman at the time of surgery with scarce endocrinologic symptoms had suffered visual loss from 2 episodes, 18 years apart. Analysis of her symptoms, neuro-ophthalmologic findings and neurodadiologic features suggest that her visual damage was due to an optochiasmatic arachnoiditis, rather than to the tumor itself.
In a series of 80 patients with surgically proven optochiasmatic arachnoiditis, only one case with an exclusively cystic form of the disease was found and is reported here. Certain changes, within a relatively short (6 months) period of time, of the neuro-ophthalmologic and neuroradiologic features are described in detail and discussed.
Seven cases of adhesive arachnoiditis of the optochiasmatic cistern, verified surgically, with a history of head injury are presented. Neuroophthalmologic findings consisted of impairment of visual acuity and funduscopic atrophy rather than papilledema. The visual fields showed a damage of the periaxial rather than the axial type. Neuroradiologic examinations did not consistently give the findings considered classical of optochiasmatic arachnoiditis. Surgical lysis of the adhesions has given an improvement of the visual function, or at least an arrest in its impairment, in almost all cases. It is thought that these cases still present some valid indications for neurosurgical exploration of the optochiasmatic region.
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