Intracerebral Whipple disease: unusual location and bone destruction

2002 ◽  
Vol 97 (4) ◽  
pp. 988-991 ◽  
Author(s):  
Ziya Akar ◽  
Necmettin Tanriover ◽  
Saffet Tüzgen ◽  
Bülent Canbaz ◽  
Haldun Erman ◽  
...  
Keyword(s):  
Chronic Diarrhea ◽  
Bone Destruction ◽  
Cns Involvement ◽  
Content Type ◽  
First Case ◽  
Whipple Disease ◽  
The Central Nervous System ◽  
Mass Lesions ◽  
Systemic Bacterial Infection

✓ Whipple disease is a rare systemic bacterial infection characterized by migratory polyarthralgia and chronic diarrhea. In 5 to 20% of patients with Whipple disease, the infection may present initially with or eventually develop symptoms related to the central nervous system (CNS). Although CNS involvement is a known feature of systemic Whipple disease, intracerebral mass lesions are uncommon. Mass lesions in these cases are typically deep seated and multifocal. Corticosubcortical regions are unusual sites of CNS involvement in cases of Whipple disease. In the present paper, the authors describe the first case of Whipple disease to feature a single corticosubcortical solid frontoparietal mass lesion that displayed homogeneous contrast enhancement on neuroimaging and was associated with bone destruction of the calvaria. Although CNS involvement has been observed in the form of deep-seated mass lesions in cases of systemic Whipple disease, unusual manifestations should be kept in mind during diagnosis and follow-up review in these patients.

Whipple disease confined to the central nervous system presenting as a solitary frontal tumor

2004 ◽  
Vol 101 (2) ◽  
pp. 336-339 ◽  
Author(s):  
Mario Löhr ◽  
Werner Stenzel ◽  
Georg Plum ◽  
Wulf-Peter Gross ◽  
Martina Deckert ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Gastrointestinal Symptoms ◽  
Low Grade ◽  
Cns Involvement ◽  
Content Type ◽  
Distinctive Features ◽  
Whipple Disease ◽  
The Central Nervous System ◽  
Histopathological Investigation

✓ Whipple disease is a rare infection caused by the bacterium Tropheryma whippelii. Patients usually present with gastrointestinal symptoms or migratory arthralgias. Although symptomatic central nervous system (CNS) involvement frequently occurs, Whipple disease confined to the CNS is rare. The authors present the case of a 40-year-old man who was surgically treated for a symptomatic left frontal tumor that had the neuroimaging features of a low-grade glioma (LGG). A histopathological investigation revealed a perivascular accentuated inflammation with macrophages harboring PAS-positive diastase-resistant rods, which are distinctive features of cerebral Whipple disease. The patient received cotrimoxazole for 1 year postoperatively and recovered well. This case is exceptional because it represents an isolated cerebral manifestation of Whipple disease that presented as a solitary frontal tumor, thus raising the differential diagnosis of LGG. A review of diagnostic and therapeutic options in suspected cases is presented.

Atlantoaxial instability in Dyggve-Melchior-Clausen syndrome

2002 ◽  
Vol 96 (1) ◽  
pp. 112-117 ◽  
Author(s):  
Frank Kandziora ◽  
Luitgard Neumann ◽  
Klaus John Schnake ◽  
Cyrus Khodadadyan-Klostermann ◽  
Stefan Rehart ◽  
...  
Keyword(s):  
Screw Fixation ◽  
Cord Compression ◽  
Atlantoaxial Instability ◽  
Posterior Fixation ◽  
Content Type ◽  
First Case ◽  
Solid Fusion ◽  
Specific Care ◽  
Preventable Complication

✓ Dyggve-Melchior-Clausen (DMC) syndrome is a very rare disease. Only 58 cases have been reported in the literature. The syndrome is probably an autosomal recessive inherited disorder, one that is characterized by mental retardation, the short-spine type of dwarfism, and skeletal abnormalities, especially of the spine, hands, and pelvis. Atlantoaxial instability— induced spinal cord compression is a serious and preventable complication. The purpose of this report is to describe the first case of DMC syndrome in which anterior transarticular atlantoaxial screw fixation was used to treat atlantoaxial instability. The authors report on a 17-year-old man with DMC syndrome and concomitant severe atlantoaxial instability. Computerized tomography scanning and magnetic resonance angiography demonstrated an irregular course of the vertebral artery (VA) at C-2, which made a posterior fixation procedure impossible. Additionally, transoral fusion was impossible because the patient was unable to open his mouth sufficiently. Therefore, the patient underwent anterior transarticular screw fixation. Follow-up examination 36 weeks after surgery showed solid fusion without implant failure. In conclusion, treatment of atlantoaxial instability in DMC syndrome must be considered. Specific care must be taken to determine the course of the VA. If posterior and transoral fusion are impossible, anterior transarticular atlantoaxial screw fixation might be the only alternative.

The influence of transoral odontoid resection on stability of the craniovertebral junction

1992 ◽  
Vol 77 (4) ◽  
pp. 525-530 ◽  
Author(s):  
Curtis A. Dickman ◽  
Jacqueline Locantro ◽  
Richard G. Fessler
Keyword(s):  
Rheumatoid Arthritis ◽  
Bone Destruction ◽  
Craniovertebral Junction ◽  
Transoral Surgery ◽  
Posterior Decompression ◽  
Radiographic Evidence ◽  
Content Type ◽  
Surgical Interventions ◽  
Fine Print

✓ Twenty-seven cases of craniovertebral junction compression treated with transoral surgery were reviewed to assess the influences of pathological processes and surgical interventions on spinal stability. All patients presented with signs and symptoms of spinal-cord or brain-stem dysfunction. Pathology included rheumatoid arthritis in 11 patients, congenital osseous malformations in 11, spinal fractures in two, plasmacytoma in one, osteomyelitis in one, and a gunshot injury in one. Instability was defined as clear radiographic evidence of mobile subluxation in conjunction with clinical assessment. Of 19 patients (70%) requiring internal fixation, nine underwent upper cervical fusion and 10 had occipitocervical fusion. When instability occurred, all subluxations were at the C1–2 level. There were no occipitoatlantal subluxations. Eight patients (30%) had preoperative instability of the craniovertebral junction due solely to their pathology, 11 patients (40%) suffered instability after transoral surgery, and eight (30%) were without clinical or radiographic evidence of instability (mean follow-up period 14 months). Craniovertebral junction instability predominated among patients with rheumatoid arthritis: 91% required fusion and 45% presented with pre-existing instability. Among individuals with congenital osseous malformations, 45% required fusion and only one patient (9%) had pre-existing instability. Patients who required subsequent posterior decompression of a Chiari malformation were at risk for developing instability; three of four became unstable after posterior decompression. Transoral resection of the dens, the anterior arch of C-1, and the lower clivus does not fully destabilize the spine; however, this operation may potentiate incipient pathological instability. The primary determinants of instability are the extent of pathological bone destruction, ligamentous weakening, and operative bone removal. Long-term follow-up monitoring is needed after transoral surgery to detect cases of late instability.

Cerebral hemangioma with glial neoplasia (angioglioma?)

1982 ◽  
Vol 56 (3) ◽  
pp. 430-434 ◽  
Author(s):  
Edwin G. Fischer ◽  
Ana Sotrel ◽  
Keasley Welch
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
The Other ◽  
Content Type ◽  
Growth Reference ◽  
Biological Potential ◽  
Cavernous Hemangiomas ◽  
The Central Nervous System ◽  
Mass Lesions ◽  
Fine Print

✓ Two patients are reported who had intracerebral mass lesions composed of hemangioma and glial neoplasm. After excision, one recurred as an oligodendroglioma, and the remnant of the other remained static over a 5-year period. These lesions may represent a subgroup of cerebral hemangiomas that have the biological potential for future glial neoplastic growth. Reference is made to experimental work with polyoma virus which can induce cavernous hemangiomas in the central nervous system in mice, and which is a papovavirus. Other papovaviruses can induce ependymomas in hamsters.

Transpedicular instrumentation as an adjunct in the treatment of thoracolumbar and lumbar spine tuberculosis with early stage bone destruction

1999 ◽  
Vol 91 (2) ◽  
pp. 163-169 ◽  
Author(s):  
Tao-Chen Lee ◽  
Kang Lu ◽  
Lin-Cheng Yang ◽  
Hsuan-Ying Huang ◽  
Cheng-Loong Liang
Keyword(s):  
Spinal Tuberculosis ◽  
Bone Destruction ◽  
Duration Of Symptoms ◽  
Content Type ◽  
Excellent Outcome ◽  
Transpedicular Instrumentation ◽  
Vertebral Bodies ◽  
Lumbar Spinal ◽  
Fine Print

Object. Because modern imaging techniques now allow for early diagnosis of spinal tuberculosis, more conservative management options are possible. The authors evaluated the effectiveness of transpedicular instrumentation for treatment of thoracolumbar and lumbar spinal tuberculosis in patients with mild bone destruction and the main symptom of “instability catch” (a sudden painful “snap” that occurs when one extends from a forward bent to an upright position). Methods. Eighteen patients (nine men and nine women, age range 49–71 years) with spinal tuberculosis were treated with transpedicular instrumentation that was supplemented with posterolateral fusion and chemotherapy. All patients were wheelchair dependent or bed-ridden due to severe instability catch, with a mean symptom duration of 2.5 months (range 1–6 months). Two contiguous vertebrae were involved in 17 patients, and a single vertebrae was involved in one. In five patients mild neurological deficits (Frankel Grade D) were present. During surgery, the screws were implanted into the two nonaffected pedicles nearest the lesion to stabilize the involved segments. No attempt at radical debridement or neural decompression was undertaken. The follow-up period ranged from 21 to 40 months. Postoperatively the instability catch was relieved within 10 days (excellent outcome) and within 1 month (good outcome) in seven and eight patients, respectively, and within 3 months (fair outcome) in two; in the remaining patient, the symptom did not resolve (poor outcome). A short duration of symptoms (generally < 3 months) and bone destruction of less than 50% in the involved vertebral bodies were observed in patients who made a good or excellent outcome. During the follow-up period, good maintenance of spinal alignment, stabilization of the involved segment, and resolution of the inflammatory process were shown; however, there was no strong evidence that fusion had occurred at the bony defect. Patients in whom a fair outcome was achieved experienced a longer duration of symptoms, and in each, one vertebral body with greater than 50% bone destruction was demonstrated. However, good maintenance of spinal alignment was also shown during the follow-up period. The patient whose outcome was poor had the longest history (6 months) of symptoms and the most extensive involvement of the spine (> 50% destruction of two adjacent lumbar vertebral bodies). Postoperatively, implant failure occurred and the patient developed a wound infection. Conclusions. Transpedicular instrumentation provides rapid relief of instability catch and prevents late angular deformity in patients with thoracolumbar and lumbar spinal tuberculosis in whom limited (< 50%) bone destruction of the involved vertebral bodies has been shown and whose main symptom is instability catch.

Treatment of colloid cysts of the third ventricle by stereotaxic microsurgical laser craniotomy

1989 ◽  
Vol 70 (4) ◽  
pp. 525-529 ◽  
Author(s):  
Chad D. Abernathey ◽  
Dudley H. Davis ◽  
Patrick J. Kelly
Keyword(s):  
Third Ventricle ◽  
Total Removal ◽  
Total Resection ◽  
Content Type ◽  
The Third ◽  
Cyst Aspiration ◽  
Colloid Cysts ◽  
Mass Lesions ◽  
Fine Print

✓ The therapeutic strategies employed in the management of anterior third-ventricular mass lesions remain controversial. Resection by conventional craniotomy, whether via a transcallosal or transcortical approach, carries well-known risks and limitations. Alternatively, in this region traditional stereotaxy has been relegated to use with biopsy only or cyst aspiration procedures. Combining aspects of both conventional and stereotaxic techniques has allowed total removal of 12 colloid cysts in six women and six men ranging in age from 25 to 71 years. No mortality and minimal morbidity have been associated with the procedures. There has been no evidence of recurrence in an average follow-up period of 19 months. By coupling the benefits of stereotaxic precision and localization to the microsurgical management of colloid cysts, several rewards have been realized: 1) only a limited cortical dissection is needed; 2) the hazards of callosal or forniceal injury can be avoided; 3) the lesion is easily localized regardless of ventricular size; 4) hemostasis can be readily achieved with bipolar cautery or defocused laser power; and 5) most importantly, a total resection is possible with little risk to the patient. Stereotaxic microsurgical laser craniotomy provides a new option for the management of colloid cysts and other anterior third-ventricular lesions.

scholarly journals Brainstem angiocentric glioma: report of 2 cases

2017 ◽  
Vol 20 (4) ◽  
pp. 347-351 ◽  
Author(s):  
Kristin J. Weaver ◽  
Lexi M. Crawford ◽  
Jeffrey A. Bennett ◽  
Marie L. Rivera-Zengotita ◽  
David W. Pincus
Keyword(s):  
Cerebral Hemispheres ◽  
Third Ventriculostomy ◽  
Pathological Features ◽  
First Case ◽  
Close Observation ◽  
Pontine Lesion ◽  
The Central Nervous System ◽  
Angiocentric Glioma ◽  
Initial Description

Angiocentric glioma is a rare tumor that was recognized by the WHO Classification of Tumours of the Central Nervous System as a distinct clinicopathological entity in 2007. Since this initial description, the vast majority of cases of angiocentric glioma reported in the literature have involved tumors of the cerebral hemispheres. To date, only 1 case of angiocentric glioma arising from the posterior midbrain has been reported. The authors present the cases of 2 pediatric patients who were found to have brainstem angiocentric gliomas. The clinical course, radiological and pathological features, treatment, and follow-up are described. The first case is one of a 5-year-old girl who presented with double vision, headache, and nausea and was found to have a midbrain lesion with pathological features consistent with angiocentric glioma. She was treated with resection and endoscopic third ventriculostomy (ETV), followed by close observation and serial neuroimaging. The second case is one of a 6-year-old boy who presented with progressive mouth drooping and problems with balance. He was found to have a pontine lesion with pathological features consistent with angiocentric glioma. This patient was treated with ETV, followed by close observation and serial neuroimaging. This report includes 6 and 1.5 years of follow-up of the patients, respectively. While there are limited data regarding the prognosis or long-term management of patients with brainstem angiocentric gliomas, the cases described in this report suggest an indolent course for this tumor, similar to the course of angiocentric gliomas located in the cerebral hemispheres.

Subdural sarcoma associated with chronic subdural hematoma

1997 ◽  
Vol 86 (3) ◽  
pp. 553-557 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Michel Zerah ◽  
Michel Carteret ◽  
François Doz ◽  
Laurent Vinikoff ◽  
...  
Keyword(s):  
Subdural Hematoma ◽  
Fluid Collection ◽  
Chronic Subdural Hematoma ◽  
Subsequent Development ◽  
Good Resolution ◽  
Content Type ◽  
First Case ◽  
Contrast Enhanced ◽  
Fluid Collections

✓ The authors report the cases of two children who presented in the first months of life with progressive macrocrania related to chronic pericerebral fluid collection. This condition resolved spontaneously without treatment after a few months in the first case, whereas it required several aspirations of blood-stained fluid via the fontanel in the second case. Both patients developed normally without evidence of disease in the earliest years of life and presented at the ages of 3 1/2 and 4 1/2 years, respectively, with symptoms and signs of rapidly progressing intracranial hypertension. In both cases contrast-enhanced computerized tomography and magnetic resonance imaging revealed masses in the subdural space of the skull base and the cranial vault associated with significant subdural fluid collections. In the first case the lesion was misdiagnosed in the initial phase and treated, by means of multiple craniotomies, as an organized subdural hematoma. After a diagnosis of liposarcoma had been made, the patient was treated with chemotherapy, which resulted in a good resolution of the lesions at 3-month follow-up review. In the second case a biopsy allowed the diagnosis of fibrohistiocytic sarcoma and the patient was treated with chemotherapy. The authors review the literature of the few reported cases and discuss the possible pathophysiological association between pericerebral fluid collection and the subsequent development of a subdural sarcoma.

Sinus skeletonization: a treatment for dural arteriovenous malformation of the tentorial apex

1996 ◽  
Vol 84 (3) ◽  
pp. 514-517 ◽  
Author(s):  
César P. Lucas ◽  
Evandro de Oliveira ◽  
Helder Tedeschi ◽  
Mario Siqueira ◽  
Mario Lourenzi ◽  
...  
Keyword(s):  
Surgical Technique ◽  
Arteriovenous Malformation ◽  
Endovascular Embolization ◽  
Dural Arteriovenous Malformation ◽  
Content Type ◽  
Suboccipital Craniotomy ◽  
First Case ◽  
Transverse Sinuses ◽  
Fine Print

✓ Two cases of dural arteriovenous malformation of the tentorial apex are presented. Both were treated surgically by means of a sinus skeletonization technique. The operative technique included a combined bioccipital and median suboccipital craniotomy in which the posterior third of the superior sagittal and the straight and bilateral transverse sinuses were skeletonized by incising the falx and the tentorium along the sinuses. Endovascular embolization was used prior to the surgical approach in one case. Clinical and angiographic cure was achieved in both patients, with a follow up of 4 years in the first case and 1 year in the second one. The surgical technique is described in detail.

scholarly journals Clinicopathological Characteristics and Immunohistochemistry of Intracranial Hemangiopericytoma: Case Report and Literature Review

2021 ◽  
Author(s):  
Shikun Yang ◽  
Junbo Lian ◽  
Wenxuan Huang ◽  
Yang Liu ◽  
Shuangjie Huo ◽  
...  
Keyword(s):  
Clinicopathological Characteristics ◽  
The Body ◽  
Lower Limbs ◽  
Pathological Examination ◽  
Immunohistochemical Markers ◽  
First Case ◽  
Multiple Metastases ◽  
Long Term Follow Up ◽  
The Central Nervous System

Abstract BackgroundHemangiopericytoma was first named as a soft tissue tumor in 1942. It is a group of tumors composed of short spindle cells with pericyte growth. It is rare in clinical practice and accounts for 0.4 of all tumors originating in the central nervous system 0.4%, with a high degree of recurrence and the potential for metastasis outside the central nervous system.Case presentationWe report 4 patients, including 1 male and 3 females. All patients had headache, dizziness, and fatigue. The first and fourth patients showed acute exacerbation, paroxysmal limb twitching, flexion of both upper limbs, straightening of both lower limbs, unconsciousness, unresponsiveness, and upper eyeballs. Turning and closing of the teeth, 1 out of 2 patients was accompanied by a tongue bite. All 4 patients underwent imaging examinations. Considering the possibility of meningiomas and gliomas, all 4 patients underwent surgical resection and were followed up many times after the operation. The first case was followed up for 68 months and passed away due to recurrence without treatment. The second case was followed up for 50 months and relapsed at 42 months after the operation. It relapsed again 18 months after the operation and passed away with multiple metastases throughout the body; the third patient died in time The 4 patients were followed up for 32 months and 9 months respectively, and there is no recurrence at present.ConclusionsMeningeal HPC is a rare clinical tumor. 4 cases were followed up for 9-68 months, 2 cases recurred after surgery, and 1 case had multiple metastases throughout the body. It is not easy to distinguish HPC from meningioma on imaging. The diagnosis still depends on pathological examination, combined with the combined diagnosis of STAT6, CD34 and ALDHIA1 immunohistochemical markers, which can effectively improve the diagnosis and differential diagnosis of the disease. Because HPC is prone to recurrence and metastasis, Long-term follow-up and timely follow-up are still needed after operation.

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