Cases reported • Central Nervous System Neoplasms; Neoplasms, Central Nervous System

11/110. cerebrospinal fluid oligoclonal IgG bands in patients with spinal arteriovenous malformation and structural central nervous system lesions.

OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, israel. patients: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, arnold-chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.

- - - - - - - - - -

ranking = 1
keywords = disorder
(Clic here for more details about this article)

12/110. Intravascular lymphomatosis: contribution of cerebral MRI findings to diagnosis.

Intravascular lymphomatosis (IL) is a rare variant of non-Hodgkin's lymphoma with an unusual predilection for the central nervous system (CNS). Most cases are not diagnosed until postmortem because of variable clinical presentation and nonspecific laboratory findings. Neuroimaging findings vary widely and range from diffuse involvement of the deep white matter to infarct-like lesions. Cerebral magnetic resonance imaging (MRI) may show parenchymal and meningeal gadolinium enhancement. The authors describe brain MRI findings of linear, punctate, and patchy enhancement suggestive of CNS IL in two patients confirmed by brain biopsy/histologic studies. High index of clinical suspicion and careful interpretation of MRI (including gadolinium contrast studies) may contribute to premortem diagnosis and early intervention of this often-missed disease.

- - - - - - - - - -

ranking = 8.4033346545118
keywords = disease
(Clic here for more details about this article)

13/110. Angiotropic lymphoma: proliferation of B cells in the capillaries of cutaneous angiomas.

We report a 52-year-old patient with cutaneous angiomas on the trunk in association with angiotropic lymphoma involving the nervous system. The lesional skin showed proliferation of atypical B cells in the capillaries. The diagnosis of angiotropic lymphoma with neurological deficits is often difficult before death. Thus, skin biopsy in this case proved to be diagnostic of the central nervous system disease without the risk of brain biopsy. The diagnosis of angiotropic lymphoma in the future may be achievable by biopsy of coincidental angiomas.

- - - - - - - - - -

ranking = 8.4033346545118
keywords = disease
(Clic here for more details about this article)

14/110. Non-Hodgkin's lymphoma of the uterus and CNS.

We report a case of a non-Hodgkin's lymphoma of the uterus and central nervous system in an 8-year-old female. The neurologic signs included blurred vision, neck stiffness, and walking difficulties but no abdominal problems. She deteriorated further, and repeated lumbar punctures revealed the presence of malignant cells in the cerebrospinal fluid. A repeated ultrasound scan of the abdomen demonstrated a markedly enlarged uterus. biopsy revealed B-cell non-Hodgkin's lymphoma. Treatment according to the berlin-Frankfurt-Munster protocol was initiated, but she developed hyperventilation syndrome and required mechanical ventilation. Her condition improved after 1 week but then deteriorated again, and despite additional chemotherapy she developed myelosuppression and septicemia with multiresistant klebsiella pneumoniae and eventually died 13 months after her first admission to the hospital. No clinical or laboratory signs of relapse were evident at the time of death.

- - - - - - - - - -

ranking = 8.7579712570279
keywords = syndrome
(Clic here for more details about this article)

15/110. Primary central nervous system lymphoma and subcutaneous metastases.

Primary central nervous system lymphoma (PCNSL) is a rare disease with a poor prognosis. It usually remains confined to central nervous system (CNS). Reports of metastases outside of the CNS are rare. We report a patient with well-documented PCNSL who responded to treatment, but subsequently developed a histologically confirmed subcutaneous metastasis to the left leg without local failure.

- - - - - - - - - -

ranking = 8.4033346545118
keywords = disease
(Clic here for more details about this article)

16/110. Seven cases of breast cancer recurrence limited to the central nervous system without other visceral metastases.

We report 7 rare cases of recurrent breast cancers who presented with central nervous system (CNS) metastases as the initial relapse site without any other organ metastases. The average age of the patients at surgery was 42.6 years old of age (median 45:range 32-60), and 6 of the 7 cases (86%) were premenopausal. The mean disease-free period was 25.7 months (median 22, range 2-60 months). The primary tumors were all invasive ductal carcinomas. The estrogen receptor and progesterone receptor status of the 3 tumors available for study were all negative. The metastatic CNS lesions included the cerebrum (4 cases), cerebellum, cervical spinal cord, and meninges. In 6 out of these 7 cases (86%), the CNS metastasis was the initial recurrent lesion. Multidisciplinary treatments including surgery, radiotherapy and systemic or intrathecal chemotherapy were given. Although the mean survival time from clinical manifestations of the metastases of the 4 deceased patients was 20 months (median 20.5; range 6-33), one patient treated with surgery and radiotherapy is been still alive18 years later. These cases were also notable for the fact that the only metastatic site was in the CNS only during the entire clinical course, except for 2 cases, one with ocular adnexa metastasis, and the other with cervical lymph node metastasis. Premenopausal patients with negative hormone receptor status are more likely to develop this type of recurrence, regardless of the histological type. It is necessary to pay attention to neurological symptoms and signs during follow-up of breast cancer patients.

- - - - - - - - - -

ranking = 8.4033346545118
keywords = disease
(Clic here for more details about this article)

17/110. CNS lipoma in patients with epidermal nevus syndrome.

Epidermal nevus syndrome (ENS) is a congenital neurocutaneous disorder characterized by linear nevus with a significant involvement of the nervous, ophthalmological and skeletal systems. Clinical manifestations of ENS include neurological features such as mental retardation, seizures, and movement disorders which are caused by a wide range of neuropathological lesions. We describe three patients with ENS, all of whom had in addition to the characteristic features of ENS intracranial and/or intraspinal lipomas. In one patient the lipoma extended from the thoracal vertebra 8 to the 4th ventricle; in the second patient it was localized on T9, and in the third patient an intracranial lipoma was located at the right cerebellopontine angle. The intraspinal lipomas caused a significant spastic movement disorder. So far, CNS lipomas have not been described as typical neuropathological findings in ENS. The differential diagnosis to encephalocraniocutaneous lipomatosis with the typical finding of CNS lipoma is discussed.

- - - - - - - - - -

ranking = 46.78985628514
keywords = syndrome, disorder
(Clic here for more details about this article)

18/110. Clinicopathological characterization of an hiv-2-infected individual with two clonally unrelated primary lymphomas.

Human immunodeficiency virus 2 (hiv-2) is endemic in West africa and is a causative agent of the acquired immunodeficiency syndrome. Only a small number of hiv-2-infected patients have been described in detail. Non-Hodgkin's lymphoma (NHL) is the second most common neoplasm occurring in hiv-1-infected patients, but its incidence seems to be lower in hiv-2-infected individuals. We report an hiv-2-infected patient from Cape Verde (West africa) with separate and distinct systemic and primary central nervous system large B-cell lymphomas and review the findings of cases of hiv-2-associated lymphomas reported in the literature. Different clonal rearrangements of the immunoglobulin heavy chain gene could be detected in the two lymphomas of our patient by polymerase chain reaction and sequence analysis. These data indicate the presence of two clonally unrelated large B-cell lymphomas in the same patient, which is an unusual finding. Neither Epstein-Barr virus nor human herpesvirus 8 could be detected in the tumor tissues or the cerebrospinal fluid. hiv-2 infection should be considered in patients with NHL, especially in those from West africa.

- - - - - - - - - -

ranking = 8.7579712570279
keywords = syndrome
(Clic here for more details about this article)

19/110. Cryptic von hippel-lindau disease: germline mutations in patients with haemangioblastoma only.

OBJECTIVES: central nervous system haemangioblastoma (HAB) is a major feature of von Hippel-Lindau (VHL) disease, and it is estimated that about 30% of HAB patients have VHL disease. Consequently, it is widely recommended that sporadic HAB patients are screened for clinical and radiological features of VHL disease because of the risk of multiple tumours. We investigated the frequency of VHL germline mutations in patients with HAB only with no clinical or radiological evidence of VHL disease to define the role of molecular genetic analysis in the management of such patients. methods: Eighty four patients with a single HAB (23 Dutch, 61 UK) and four with multiple HAB (two Dutch, two UK) were studied by direct sequencing of the coding region and quantitative Southern blotting. RESULTS: A VHL germline mutation was found in three of 69 (4.3%) single HAB patients aged 50 years or less (three of 84 (3.6%) total single HAB patients). A germline VHL mutation was detected in a 44 year old woman with a solitary cerebellar HAB, as well as in four clinically unaffected close relatives, and in two single HAB cases presenting at the ages of 29 and 36 years. Germline VHL mutations were detected in two of four cases with multiple HAB. CONCLUSIONS: Early detection of VHL disease is important to reduce morbidity and mortality and therefore we recommend that, in addition to conventional clinical and radiological investigations, VHL gene mutation analysis should be offered to all HAB patients younger than 50 years. HAB patients aged >50 years will have a lower a priori risk of VHL disease and further data are required to evaluate the role of routine molecular genetic investigations in late onset HAB cases. The failure to detect germline VHL mutations in some patients with multiple HAB may indicate the presence of somatic mosaicism or additional HAB susceptibility genes.

- - - - - - - - - -

ranking = 84.033346545118
keywords = disease
(Clic here for more details about this article)

20/110. radiation therapy approach in gliomatosis cerebri--case reports and literature review.

Gliomatosis cerebri is defined as a remarkably diffuse glioma, characterized by widespread infiltration of the central nervous system. Clinicopathologic characteristics and imaging findings have been published but valid classification remains controversial. Few reports exist regarding therapeutic options in gliomatosis cerebri. Here we review data on 17 patients treated with radiation therapy extracted from the literature, in which we focus our attention on available details of irradiation and clinical outcome and present the results of three additional patients treated at our two institutions. Radiologic-pathologic correlation in gliomatosis cerebri indicates that tumor delineation should be based on T2-weighted MRI. radiation therapy in gliomatosis cerebri is associated with a temporary improvement in or stabilization of clinical symptoms in the majority of cases. Duration of improvement was > or = 6 months in 50% of treated patients. survival from onset of symptoms was 23.8 months (range 8-42). Considerable variation in the natural course of the disease precludes conclusions regarding the impact of radiation therapy on survival.

- - - - - - - - - -

ranking = 8.4033346545118
keywords = disease
(Clic here for more details about this article)