A case of sarcoidosis presenting as a non-specific intramedullary lesion

Neurosarcoidosis occurs in 5–15% of sarcoidosis cases. Approximately 50% of patients with neurosarcoidosis present with a neurological disease at the time sarcoidosis is first diagnosed. Spinal sarcoidosis is rare. We report the case of a 61-year-old man with a highly aspecific intramedullary lesion as the first manifestation of sarcoidosis. One year after the onset of neurological symptoms, the high levels of angiotensin-converting enzyme and the results of a total body gallium scan and bronchoalveolar lavage supported the diagnosis of sarcoidosis. Isolated single reports indicate that spinal neurosarcoidosis may be the initial manifestation of sarcoidosis. In our case, magnetic resonance imaging of the dorsal spine showed a largely aspecific lesion. Neurosarcoidosis should be considered in the differential diagnosis of intramedullary cord lesion with leptomeningeal enhancement; a systematic search for evidence of sarcoidosis should be mandatory in all cases for a correct diagnosis and early treatment.     

Medulloblastomas of the cerebellopontine angle.

It is unusual for a medulloblastoma to occur in the cerebellopontine angle (CPA). Four of the total 63 cases of medulloblastoma managed in our department between 1988 to 1998, presented with a mass in the cerebellopontine angle. There were two children amongst these four patients, aged eight and nine years. The other two patients were young adults. Three patients had primary CPA medulloblastoma, while the fourth case had a recurrence in this region, following the excision of the primary vermian tumour. All the four cases were managed by gross total microsurgical excision and craniospinal irradiation. One of the two children, developed subfrontal recurrence after 1 year and 8 months of initial treatment, while the other child developed a large recurrence at primary site, within three months of post-operative irradiation. However, the adult patient developed CPA recurrence and subsequent cervical intramedullary metastasis within two and a half years of diagnosis. These three cases succumbed to their disease, between three months to thirty months of their initial management. However the fourth patient, a 20 years female with desmoplastic variant, was well at two and a half years of follow up.     

Intramedullary spinal cord metastasis as initial presentation of systemic cancer--report of a rare case

We report the rare case of a 74-year-old man who was admitted to our hospital with rapid progression of tetraparesis, which was most apparent in the lower right limb, sensory disturbances from C3 to S1 on the left side and recent onset of constipation and urinary retention. There was no known history of cancer. As MRI of the neck disclosed a cervical intramedullary mass lesion at C 4/5 level suspicious for a primary glial tumour, the patient underwent surgery. After microsurgical excision the histological analysis of the lesion unexpectedly revealed an intramedullary spinal cord metastasis (ISCM) of a poorly differentiated carcinoma, immunohistochemically consistent with a bronchial carcinoma. As intramedullary spinal cord metastases are generally associated with poor survival, a palliative irradiation of the levels C1-6 was additionally performed. Unfortunately tetraparesis and numbness remained. The very rare occurrence of intramedullary spinal cord metastasis and the absence of pathognomonic symptoms often lead to a delay until an underlying malignancy is discovered. Although rare, intramedullary spinal cord metastasis should be considered as a differential diagnosis of a spinal intramedullary lesion. Surgery and radiation are both options in the controversially discussed treatment of ISCM.     

Exophytic intramedullary meningioma of the cervical spinal cord

Intramedullary spinal cord neoplasms are relatively uncommon. The most common intramedullary tumors are astrocytomas and ependymomas. Meningiomas can occur as an intradural tumor; however, they are typically in the extramedullary compartment. A 42-year-old male presented with progressive sensory loss in the upper extremities and lower extremity weakness. Pre-operative imaging suggested an intramedullary cervical lesion. To treat the progressive neurological abnormality, surgical resection was planned. At surgery, it was noted that the tumor originated in the cervical spinal cord and extended into the extramedullary region. Histology confirmed the lesion to be a meningioma. This meningioma variant has not previously been described. Spinal meningiomas may occur in locations other than intradural, extramedullary locations, and should be included in the differential diagnosis of intramedullary lesions. Intramedullary meningiomas can be successfully treated with surgery.     

Medulloblastoma with mandibular metastasis after complete remission of the central nervous system lesions]

A 6-year-old boy presented with headache and vomiting. Brain and spinal MRI demonstrated a large mass in the cerebellar vermis and 4th ventricle and showed thick spinal subarachnoid dissemination. Suboccipital craniotomy was performed and the tumor was totally removed. The histological diagnosis was medulloblastoma. The patient subsequently received craniospinal irradiation, and also received systemic and intrathecal perfusion chemotherapy. Then complete remission was achieved 10 months after operation. Three years later, however, a swelling at the left mandibular angle appeared. A CT scan revealed osteosclerotic lesion. After biopsy was performed, the specimen was detected infiltration of medulloblastoma cells. Bone scintigram showed a single lesion and MRI of brain and spinal cord revealed no recurrence of the central nervous system lesions. He underwent local irradiation and systemic chemotherapy with ICE regimen. This is the rare case of extracranial metastasis following remission of medulloblastoma in childhood.     

Intramedullary subependymoma of the cervical spinal cord: a case report with immunohistochemical study

Spinal subependymomas, which have a relatively benign nature, are very rare tumors. It is difficult to distinguish spinal subependymomas from other intramedullary spinal tumors based on neuroradiological findings. A case of cervical intramedullary subependymoma in a 63-year-old female is reported. The diffused enlargement of the spinal cord at C2 level involved the lesion with isointensity on a T1-weighted MRI and relatively high intensity on a T2-weighted MRI. Enhancement in the small part of the tumor was observed on a T1-weighted MRI with gadolinium administration. The tumor occupied the left side of the spinal cord, and was totally removed through a laminoplasty of C2. Immunohistochemistry was useful for pathological diagnosis. The clinical feature of this patient is described with the review of literatures.     

Intramedullary spinal arteriovenous malformation in a boy of familial cerebral cavernous hemangioma

Object The coexistence of spinal arteriovenous malformation (AVM) and a familial cerebral cavernous hemangioma (CCH) is extremely rare. Methods A 9-year-old boy suddenly developed severe paraplegia and urinary dysfunction. Spinal magnetic resonance imaging (MRI) scan revealed a cervical and upper thoracic intramedullary lesion. Due to acute neurological dysfunction, the patient underwent emergency surgical exploration. An intramedullary vascular lesion was found and excised. Pathologically, AVM was noted. After the surgery, the boy was ambulatory with left lower limb stiffness. MRI scan of the brain revealed multiple cerebral cavernous hemangioma. Symptomatic multiple CCH in his mother and grandmother were also noted. Conclusions We concluded that the presence of spinal AVM should be suspected if the patient with familial CCH develops the signs of space-occupying lesion of the spinal cord, facilitating early diagnosis of the spinal AVM.     

Diagnostic and therapeutic strategy for confounding radiation myelitis

We report a case of confounding radiation myelitis to demonstrate the usefulness of surgical biopsy in ensuring the correct diagnosis and to avoid unnecessary treatment. The patient was a 40-year-old man with a history of epiglottis carcinoma and sarcoidosis. Six months after radiation therapy and chemotherapy for epiglottis carcinoma, he noticed paresthesia and dysesthesia in the left arm and leg. Two months after that, he complained of severe neck pain and rapidly progressing weakness in all extremities. MRI showed an enhanced intramedullary lesion with extensive edema in the cervical spinal cord. Radiation myelitis, intramedullary spinal tumor, and neurosarcoidosis were considered as differential diagnoses. Spinal cord biopsy with laminectomy was performed and radiation myelitis was diagnosed. After the surgery, the lesion was significantly decreased in size even though corticosteroid therapy was rapidly tapered. We emphasize that a spinal cord biopsy is indicated to obtain a pathological diagnosis and to make a clear treatment strategy for patients with associated diseases causing lesions of the spinal cord.     

The clinical experience of medulloblastoma treatment and the significance of time sequence for development of leptomeningeal metastasis

Objects  Among patients with medulloblastoma, an unrare aggressive central nervous system (CNS) tumor found mostly in the posterior fossa, drop metastasis from primary location to anatomically lower sites including whole spinal cord is known as a potential way for tumor to spread. There were already lots of extensive discussions regarding diagnosis and treatment for such common neurologic complication in systemic cancer; however, fewer were found about medulloblastoma. This study aimed to reexamine the possible known prognostic factors, especially drop metastasis, and defined the influence of them. Materials and methods  We retrospectively review a series of 36 patients who suffered from posterior fossa medulloblastoma and found leptomeningeal metastasis in 12 of them. Incidence, diagnosis, prognostic factors, and treatments for this common complication are discussed. The magnetic resonance images (MRI) of these patients were reviewed. The time sequence between primary surgical procedure and development of MRI evidences of drop metastasis was used for subgrouping. Log-rank test was used for survival analysis. Conclusions  By survival analysis, patients with late drop metastasis, defined as new radiologic evidence of leptomeningeal involvement after primary CNS procedures, were found to have significant survival difference to those with early metastasis defined as drop metastasis found at the time of diagnosis (log-rank test, p = 0.0047). Further stratification of patients within this group may help in evaluation of prognosis and development of different treatment strategies.     

Intramedullary Sarcoidosis Presenting with Delayed Spinal Cord Swelling after Cervical Laminoplasty for Compressive Cervical Myelopathy

Sarcoidosis is a systemic disease of unknown etiology that may affect any organ in the body. The nervous system is involved in 5-16% of cases of sarcoidosis. Here, we report a case of intramedullary sarcoidosis presenting with delayed spinal cord swelling after laminoplasty for the treatment of compressive cervical myelopathy. A 56-year-old woman was admitted to our hospital complaining of upper extremity pain and gait disturbance. The patient had undergone laminoplasty for compressive cervical myelopathy 3 months previously. Follow-up magnetic resonance imaging revealed a large solitary intramedullary lesion with associated extensive cord swelling, signal changes, and heterogeneous enhancement of spinal cord from C2 to C7. Spinal cord biopsy revealed non-necrotizing granulomas with signs of chronic inflammation. The final diagnosis of sarcoidosis was based upon laboratory data, imaging findings, histological findings, and the exclusion of other diagnoses. Awareness of such presentations and a high degree of suspicion of sarcoidosis may help arrive at the correct diagnosis.     

Intramedullary spinal teratoma with spina bifida

Spinal intramedullary teratoma is a rare tumor. We report a case of intramedullary teratoma of the conus medullaris with spina bifida. A 5-year-old boy was admitted to our hospital for removal of a lumbosacral tumor sited in an intramedullary location at the conus medullaris, which was totally resected with neuroendoscopic assistance. The pathological diagnosis was mature teratoma consisting of three germ cell layers. The pathogenesis of spinal intramedullary teratoma is discussed with special reference to spina bifida. Received: 21 July 1999 Revised: 21 December 1999     

Spinal intramedullary tuberculosis: A series of 15 cases

Objectives

Spinal intramedullary tuberculosis is a rare disease. This study aims to acquaint readers with its clinicoradiological features and emphasizes the importance of early treatment in intramedullary spinal tuberculosis.

Materials and methods

Retrospective analysis was conducted from 1985 to 2006 over a period of 21 years and data were retrieved from patient records at our institute (single centre study). Clinicoradiological and pathological data were reviewed along with final outcome at discharge and analyzed. Results: Fifteen patients were analyzed. Mean age of presentation was 31 years (range: 18–45 years), with average duration at presentation being 11 months (2–24 months). Common locations: dorsal region: 7 cases, cervical: 5 cases, cervicodorsal: 2 cases and dorsolumbar region: 1 case. Sensori-motor involvement was noted in fourteen patients. Bowel and bladder involvement was seen in ten patients while one patient had respiratory distress. Only 40% of patients had secondary involvement of spine while the rest of the cases were having primary spinal intramedullary tuberculosis. Three patients had previous history of tubercular meningitis, while one patient had old pulmonary tuberculosis. There were one case each of cervical node involvement and intracranial granuloma. Twelve patients underwent surgery while others were conservatively managed, all patients received antitubercular therapy for 18 months.Nine of the twelve operated patients showed improvement in motor power, while two of the conservatively managed patients improved. Patients presenting late had a poorer outcome.

Conclusion

Spinal intramedullary tuberculosis is a non-malignant, treatable lesion giving a good outcome on management. Surgically managed patients showed a better outcome.     

Dorsally exophytic cervicomedullary glioblastoma

Spinal glioblastoma multiforme is a rare entity comprising 1.5% of all spinal cord tumors. We report a 57-year-old man presenting with a 1.5 month history of left sided radiculopathy, lower cranial nerve weakness and difficulty in walking. MRI of the brain and spine showed an exophytic intramedullary lesion extending from the cervicomedullary junction to the lower margin of C4. To our knowledge, we report the first patient with dorsally exophytic cervicomedullary and cervical intramedullary glioblastoma multiforme.     

1H magnetic resonance spectroscopy and diffusion weighted imaging findings of medulloblastoma in 3.0T MRI A retrospective analysis of 17 cases

1H magnetic resonance spectroscopy and diffusion weighted imaging features of the cerebellar vermis in 17 medulloblastoma patients were retrospectively analyzed, and 17 healthy volunteers were selected as controls. 1H magnetic resonance spectroscopy showed that in all 17 medulloblastoma patients, N-acetyl aspartate and creatine peaks were significantly decreased, the choline peak was significantly increased, and there was evidence of a myo-inositol peak. Further, 11 patients showed a low taurine peak at 3.4 ppm, five patients showed a lipid peak at 0.9-1.3 ppm, and three patients showed a negative lactic acid peak at 1.33 ppm. Compared with the control group, the ratios of N-acetyl aspartate/choline and N-acetyl aspartate/creatine were significantly decreased, and the ratio of choline/creatine was increased, in medulloblastoma patients. Diffusion weighted imaging displayed hyperintensity and decreased apparent diffusion coefficient in medulloblastoma patients. These findings indicate that 1H magnetic resonance spectroscopy and diffusion weighted imaging are useful for qualitative diagnosis of medulloblastoma.     

Thoracic exophytic ependymoma masquerading as a benign extra-axial tumor

Spinal tumors are conventionally differentiated based on location in relation to the spinal cord. Benign spinal tumors such as schwannomas and meningiomas are typically extra-axial (intradural extramedullary) lesions, whereas more aggressive primary spinal tumors such as ependymomas are typically intramedullary masses. Rarely, ependymomas can have both intramedullary and extramedullary components (typically referred to as exophytic ependymomas). We report a case of a spinal exophytic ependymoma that radiographically masqueraded as a benign intradural extramedullary lesion causing cord compression and neurologic deficit in a 47-year-old man. The diagnosis of exophytic ependymoma was made intra-operatively, with resultant gross total resection of the extramedullary portion and subtotal resection of the intramedullary portion. Histopathological examination confirmed ependymoma with World Health Organization grade II/IV. Pre-operative suspicion of an exophytic ependymoma influences operative planning and clinical management. We review the literature and discuss clinical management strategies for these interesting spinal tumors.     

Myélopathie sarcoïdosique associée à un canal cervical rétréci : aspects IRM

Introduction

Spinal neurosarcoidosis is rare and exceptionally inaugural.

Observation

A 49-year-old African woman developed a progressive left Brown-Sequard syndrome. Magnetic resonance imaging (MRI) scans of the cervical spinal cord revealed an intramedullary lesion from C2 to T1 with intense pial enhancement after administration of contrast material associated with cervical spondylosis. The diagnostic of sarcoidosis was confirmed by liver biopsy which demonstrated noncaseating granulomas.

Conclusions

MRI features of spinal neurosarcoidosis were reviewed by the authors with focus on differential diagnosis.     

Intramedullary spinal cord metastasis as the first manifestation of a renal carcinoma

We present the case of a 69 year-old male without known antecedente who presented a clinical loss of distal force in his lower limbs during the last two months. Magnetic resonance imaging (MRI) showed an intramedullary spinal cord mass at the level of the medullaris cone. The patient was operated on; the histological diagnosis was clear cells carcinoma suggestive of metastasis from an unsuspected renal tumor that was later confirmed with an abdominal CT. When the patient's state is good, surgery can correct the neurological deficit produced by an intramedullary spinal cord lesion. The neurological state of our patient improved after the intervention, and 14 months after surgery, he has no neurological deficit in the lower limbs.     

Adult medulloblastoma and the “dural-tail” sign: Rare mimic of a posterior petrous meningioma

The authors describe clinical and radiological features of a 32-year-old male patient with a posterior fossa lesion. The radiological characteristics resembled an extra-axial lesion; a meningioma, with attachment to the tentorium and petrous bone. The histological diagnosis was classical medulloblastoma. We review literature of this atypical presentation of medulloblastoma and “dural-tail” sign, which can be associated with other benign or malignant lesions. The role of magnetic resonance spectroscopy in the diagnosis of primitive neuroectodermal tumors is discussed.     

Isolated spinal neurocysticercosis with clinical pleomorphism

Neurocysticercosis, involvement of the central nervous system by taenia solium, is one of the most common parasitic diseases worldwide. However, isolated spinal involvement by neurocysticercosis, either intramedullary or extramedullary, is uncommon. The authors report a case of C1-C2 intradural extramedullary neurocysticercosis in a young male that presented initially with signs of raised intracranial pressure followed by high cervical myelopathy. Transventricular migration of the neurocysticercal cyst along the cerebrospinal pathways leading to clinical pleomorphism as described by the authors can perplex the best of clinicians leading to a missed diagnosis as in the present case. Diagnosis was confirmed after surgical excision and the patient is doing well at six months follow up with no neurological deficits. Spinal neurocysticercosis should be considered in the differential diagnosis in high-risk populations with new symptoms suggestive of a spinal mass lesion.