Neurocutaneous melanosis with hydrocephalus and syringomyelia

1985 ◽  
Vol 62 (1) ◽  
pp. 148-152 ◽  
Author(s):  
Barry J. Leaney ◽  
Peter W. Rowe ◽  
Geoffrey L. Klug
Keyword(s):  
Clinical Manifestations ◽  
Malignant Change ◽  
Unique Case ◽  
Content Type ◽  
Neurocutaneous Melanosis ◽  
Fine Print

✓ The authors report the investigation, treatment, and progress of a unique case of neurocutaneous melanosis with syringomyelia. The suspected etiology, the pathology, and the variable clinical manifestations of this rare phakomatosis are recounted. The potential for malignant change and the possible mechanisms of syrinx development in this syndrome are discussed.

Posterior petrous meningiomas: 82 cases

2005 ◽  
Vol 102 (2) ◽  
pp. 284-289 ◽  
Author(s):  
Zhe Bao Wu ◽  
Chun Jiang Yu ◽  
Shu Sen Guan
Keyword(s):  
Facial Nerve ◽  
Clinical Manifestations ◽  
Petrous Bone ◽  
Type I ◽  
Type Ii ◽  
Posterior Surface ◽  
Total Resection ◽  
Content Type ◽  
Functional Preservation ◽  
Fine Print

Object. The aim of this study was to discuss posterior petrous meningiomas—their classification, clinical manifestations, surgical treatments, and patient outcomes. Methods. A retrospective analysis was performed in 82 patients with posterior petrous meningiomas for microsurgery. According to the anatomical relationship with the posterior surface of the petrous bone and with special reference to the internal auditory canal (IAC), posterior petrous meningiomas were classified into three types: Type I, located laterally to the IAC (28 cases); Type II, located medially to the IAC, which might extend to the cavernous sinus and clivus (32 cases); and Type III, extensively attached to the posterior surface of the petrous bone, which might envelop the seventh and eighth cranial nerves (22 cases). Sixty-eight (83%) of 82 cases involved total resection. The rate of anatomical preservation of facial nerve was 97.5%, whereas the functional preservation rate was 81%. The rate of hearing preservation was 67%. All Type I tumors were completely resected, and the rate of anatomical preservation of facial nerve was 100% and functional preservation was 93%. Regarding Type II lesions, 75% of 32 cases involved total resection; the rate of anatomical preservation of facial nerve was 97% and functional preservation was 75%. For Type III lesions, 73% of 22 cases were totally resected. The rate of anatomical preservation of facial nerve in patients with this tumor type was 95%, whereas functional preservation was 73%. Conclusions. Clinical manifestations and surgical prognoses are different among the various types of posterior petrous meningiomas. It is more difficult for Types II and III tumors to be resected radically than Type I lesions, and postoperative functional outcomes are significantly worse accordingly. The primary principles in dealing with this disease entity include preservation of vital vascular and central nervous system structures and total resection of the tumor as much as possible.

Intradural cervical chordoma without bone involvement

1995 ◽  
Vol 82 (4) ◽  
pp. 650-653 ◽  
Author(s):  
Rui M. Vaz ◽  
Josué C. Pereira ◽  
Umbelina Ramos ◽  
Celso R. Cruz
Keyword(s):  
Bone Involvement ◽  
Unique Case ◽  
Content Type ◽  
Radiological Evidence ◽  
Cervical Chordoma ◽  
Fine Print

✓ The authors report a unique case of cervical chordoma in a 37-year-old woman without radiological evidence of bone involvement that was found to be totally intradural at surgery. Chordomas that are entirely extraosseous and intradural are extremely rare and in most of the cases described are located near the clivus. This is the first reported case of an intraspinal intradural chordoma.

Intraventricular fat from a ruptured sacral dermoid cyst: clinical, radiographic, and pathological correlation

1993 ◽  
Vol 78 (4) ◽  
pp. 666-668 ◽  
Author(s):  
Tim A. Scearce ◽  
Cheng-Mei Shaw ◽  
Andrew D. Bronstein ◽  
Phillip D. Swanson
Keyword(s):  
Dermoid Cyst ◽  
Subarachnoid Space ◽  
Unique Case ◽  
Content Type ◽  
Pathological Correlation ◽  
Subarachnoid Spaces ◽  
Fine Print

✓ The authors report a unique case of a dermoid cyst that ruptured into the lumbosacral subarachnoid space following trauma, resulting in dissemination of cyst contents into the ventricles and cerebrospinal subarachnoid spaces. An intraspinous source should be considered when intraventricular fat is identified without a clear intracranial source.

Intraventricular neurocysticercosis mimicking colloid cyst

2002 ◽  
Vol 97 (1) ◽  
pp. 208-210 ◽  
Author(s):  
Arti Gupta ◽  
Surya Prakash Rao Nadimpalli ◽  
Robert P. Cavallino
Keyword(s):  
Magnetic Resonance Imaging ◽  
Differential Diagnosis ◽  
Magnetic Resonance ◽  
Colloid Cyst ◽  
High Signal ◽  
Resonance Imaging ◽  
Unique Case ◽  
Content Type ◽  
Weighted Magnetic Resonance Imaging ◽  
Fine Print

✓ The authors recently encountered a unique case of anterior third ventricular neurocysticercosis in which the cyst exhibited an unusually high signal on T1-weighted magnetic resonance imaging. The lesion's signal intensity and location made differentiation from colloid cyst difficult. Intraventricular neurocysticercosis should be included in the differential diagnosis of a colloid cyst.

Benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children

1989 ◽  
Vol 70 (4) ◽  
pp. 568-572 ◽  
Author(s):  
Jean-François Hirsch ◽  
Christian Sainte Rose ◽  
Alain Pierre-Kahn ◽  
André Pfister ◽  
Elizabeth Hoppe-Hirsch
Keyword(s):  
Postoperative Radiotherapy ◽  
Clinical Manifestations ◽  
Postoperative Mortality ◽  
Cerebral Hemispheres ◽  
Resonance Imaging ◽  
Tumor Removal ◽  
Complex Partial Seizures ◽  
Content Type ◽  
Pediatric Service ◽  
Fine Print

✓ The authors review 42 consecutive cases of benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children undergoing surgery in the pediatric service of the Hôpital des Enfants Malades between 1975 and 1987. Epilepsy was the presenting sign in 76% of the children and remained the only clinical sign at diagnosis in 62%. Partial or complex partial seizures were observed in half of the cases, but other seizure types were also frequent. Diagnosis of the tumor as the etiological agent rested upon the results of computerized tomography or magnetic resonance imaging. Postoperative mortality (5%) and morbidity (16%) rates were low. The postoperative intelligence quotient was above 80 in 71% of the patients, and 77% of the children had no major problem in school. Although only two of the 42 patients were given postoperative radiotherapy, there were no recurrences in 82% of the survivors. The actuarial probability of nonrecurrence of the tumor was 95% at 5 years. Three patients with recurrent tumor underwent further surgery and are, at the present time, recurrence-free. The incidence of epilepsy fell from 76% before surgery to 19% after removal of the tumor alone; therefore, intraoperative electrocorticography and resection of the electrically abnormal cortex at the time of the first surgical procedure do not appear necessary. It is possible that tumor removal restores a mechanism that limits the spread of seizures and, thus, the clinical manifestations of epilepsy. Benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children should not be treated with adjuvant radiotherapy, at least initially.

The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma

1999 ◽  
Vol 91 (2) ◽  
pp. 212-220 ◽  
Author(s):  
Kazunori Arita ◽  
Fusao Ikawa ◽  
Kaoru Kurisu ◽  
Masayuki Sumida ◽  
Kunyu Harada ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Magnetic Resonance ◽  
Precocious Puberty ◽  
Third Ventricle ◽  
Clinical Manifestations ◽  
Hypothalamic Hamartoma ◽  
Content Type ◽  
The Third ◽  
The Relationship ◽  
Fine Print

Object. Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship.Methods. The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the “parahypothalamic type,” in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the “intrahypothalamic type,” in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle.Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone—releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed.This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty.Conclusions. Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.

Intracranial metastatic parathyroid carcinoma

2004 ◽  
Vol 101 (6) ◽  
pp. 1065-1069 ◽  
Author(s):  
Michael Kern ◽  
Gabriel Lee ◽  
Peter Robbins ◽  
Michael Bynevelt ◽  
Peter Watson
Keyword(s):  
Parathyroid Carcinoma ◽  
English Literature ◽  
Unique Case ◽  
Intracranial Metastasis ◽  
Content Type ◽  
Fine Print

✓ Parathyroid carcinoma (PTC) is a rare pathological entity, with fewer than 400 reported cases. Intracranial metastasis of a PTC is exceptional; only five other cases have been identified in the English literature. The authors present a unique case of a patient with a solitary intracerebral metastasis of a PTC in an individual presumed to have hereditary hyperparathyroidism who had concomitant papillary and follicular carcinomas of the thyroid. The literature relevant to the management of these rare lesions is reviewed and discussed.

Hemorrhage from moyamoya-like vessels associated with a cerebral arteriovenous malformation

2004 ◽  
Vol 101 (5) ◽  
pp. 869-871 ◽  
Author(s):  
Daniel R. Fassett ◽  
Peter E. Schloesser ◽  
William T. Couldwell
Keyword(s):  
Arteriovenous Malformation ◽  
Parietal Lobe ◽  
Severe Headache ◽  
Cerebral Arteriovenous Malformation ◽  
Neurological Deficits ◽  
Unique Case ◽  
Radiographic Findings ◽  
Content Type ◽  
Neurologically Intact ◽  
Fine Print

✓ The authors describe a case of subarachnoid hemorrhage from moyamoya-like vessels associated with an arteriovenous malformation (AVM) in a 44-year-old Hispanic man who presented with severe headache. The AVM was located in the left parietal lobe and the ipsilateral middle cerebral artery was occluded. Although the patient was initially neurologically intact, he began to experience neurological deficits from mild vasospasm, illustrating the sensitivity of the underperfused portion of brain surrounding an AVM. His neurological deficits improved with aggressive hydration and elevated blood pressure. After a 3-week period, the AVM was resected without complication and all of the patient's neurological deficits resolved. The authors review radiographic findings of this unique case.

Thoracic myelopathy caused by ossification of the ligamentum flavum: a report of 18 cases

2003 ◽  
Vol 99 (2) ◽  
pp. 157-161 ◽  
Author(s):  
Karim Ben Hamouda ◽  
Hafedh Jemel ◽  
Slim Haouet ◽  
Moncef Khaldi
Keyword(s):  
North Africa ◽  
Endochondral Ossification ◽  
Ligamentum Flavum ◽  
Clinical Manifestations ◽  
Magnetic Resonance Images ◽  
Thoracic Myelopathy ◽  
Content Type ◽  
Mode Of Development ◽  
Symptoms And Signs ◽  
Fine Print

Object. Thoracic myelopathy caused by ossification of the ligamentum flavum (OLF) is a rare entity, most evident in Japan. The authors studied the clinical manifestations, radiological aspects, surgical treatment, and pathogenesis of this disease. Methods. Eighteen patients with OLF-induced thoracic myelopathy underwent laminectomy. The severity of myelopathy varied. Complete paraplegia was seen in three cases. Compression of the upper and middle third of the thoracic spine was evident in six cases and of the lower third in 12 cases. Multilevel OLF was demonstrated in 13 cases. In most cases, the ossified ligamentum flavum appears as a V-shaped lesion on computerized tomography and magnetic resonance images. In all patients the diameter of the posterior spinal canal, already narrowed, was further exacerbated by the OLF. Laminectomy was limited to the levels of compression, and the ligamentum flavum was resected in all cases. The symptoms and signs improved in 13 cases and stabilized in four cases. In one case symptoms recurred as a result of ossified lesions forming at other sites. Histological examination showed that the mode of development of the ossified ligaments was endochondral ossification. Conclusions. Reports of OLF-induced myelopathy are rare and mainly described in Japan. The incidence also seems high in North Africa. An early laminectomy limited to the level of compression is recommended. Ossified ligamentum flavum is different from the calcification of the ligamentum flavum, which is due to crystal deposits.

Hereditary neurocutaneous angiomatosis

1996 ◽  
Vol 85 (6) ◽  
pp. 1135-1142 ◽  
Author(s):  
Richard Leblanc ◽  
Denis Melanson ◽  
Ralph D. Wilkinson
Keyword(s):  
Cerebral Hemorrhage ◽  
Venous Malformation ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Neurological Deficits ◽  
Content Type ◽  
Male Patients ◽  
The Brain ◽  
Fine Print ◽  
Variable Penetrance

✓ The authors report the coexistence of vascular nevi (hemangiomas and arteriovenous malformations (AVMs) of the skin) with AVMs and venous malformations of the brain in male siblings from two related but nonconsanguineous families of three generations. The proband, his siblings, parents, aunts, uncles, and cousins were examined, underwent magnetic resonance (MR) imaging and MR angiography, and when appropriate, cerebral angiography. A father had vascular nevi and a mother, his sister, had an azygos anterior cerebral artery. No other cutaneous or cerebrovascular malformations were present in the parents. Each of the two families had two boys and one girl, 9 to 18 years of age. All the children had vascular nevi and all of the boys had coexisting cerebrovascular malformations: AVMs in three, and a venous malformation in another. One boy had three cerebral AVMs. Two boys had a cerebral hemorrhage, and one also had focal motor seizures. The skin lesions were not those of the Sturge-Weber-Dimitri, Rendu-Osler-Weber, or Wyburn—Mason syndromes. The association of cutaneous and cerebrovascular malformations was seen only in males in these families, but females have also been reported in the literature. The results obtained in these families and three other families reported from Western and Central Europe indicate that the association of cerebral and cutaneous vascular hamartomas constitutes a distinct, hereditary clinicopathological entity with autosomal dominant inheritance and variable penetrance. The clinical manifestations of this syndrome are visible, painful vascular nevi, epilepsy, cerebral hemorrhage, and focal neurological deficits. The preponderance of male patients with the full expression of the syndrome suggests a possible hormonal influence on the expression of the gene.

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