Cases reported "Spinal Cord Diseases"

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1/15. Myelopathy secondary to spinal epidural abscess: case reports and a review.

    Spinal epidural abscess (SEA) is a rare disease with an unknown incidence rate. This paper will illustrate that early diagnosis and rehabilitation may result in improved outcomes for patients with neck or back pain presenting with neurological deficits. Three cases of SEA in individuals without the commonly acknowledged risk factors of intravenous drug abuse (IVDA), invasive procedures, or immunosuppression were seen at our institution during a 10-month period between October 1995 and July 1996. The patients presented with neck or thoracic back pain and progressive neurological deficits without a febrile illness. Predisposing factors were thought to be urinary tract infection with underlying untreated diabetes mellitus in the first case, a history of recurrent skin infection in the second, and alcoholism without a definite source of infection in the third. leukocytosis, elevated sedimentation rate, and confirmatory findings reported on magnetic resonance imaging (MRI) led to the diagnosis of SEA in all three cases. Immediate surgical drainage and decompression followed by proper antibiotic treatment and early aggressive rehabilitation led to good functional outcomes. All the individuals became independent in activities of daily living, wheelchair mobility, and bowel and bladder management. Two eventually became ambulatory.
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keywords = rare disease
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2/15. Rosai-Dorman disease causing cervical myelopathy.

    STUDY DESIGN: A case report of a rare disease entity and review of the literature. OBJECTIVES: To illustrate the occurrence of an unusual disease affecting the spine and spinal canal. SUMMARY OF BACKGROUND DATA: Rosai-Dorman disease is considered an idiopathic benign lymphoproliferative disease that typically occurs in the earlier decades of life. The usual manifestation is painless massive cervical lymphadenopathy, although involvement of many extra nodal sites is common. This case illustrates Rosai-Dorman disease occurring in an elderly man with myelopathy and mass lesions of the cervical, thoracic, and lumbar spinal canal without typical lymphadenopathy.- methods: Case report illustrating clinical presentation as well as radiographic and pathologic findings, including comparisons to cases previously reported. RESULTS: Surgical decompression with incomplete resection of the lesion was performed, providing diagnosis and treatment guidance. The patient experienced significant neurologic improvement of myelopathy. CONCLUSIONS: Recognizing clinical and laboratory features of this disease may permit earlier diagnosis and limit or avoid surgical intervention in some cases.
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keywords = rare disease
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3/15. Idiopathic spinal cord herniation: report of three cases and review of the literature.

    STUDY DESIGN: Three case reports and a literature review are presented. OBJECTIVE: To describe characteristic clinical and radiographic findings of idiopathic spinal cord herniation. SUMMARY OF BACKGROUND DATA: Idiopathic spinal cord herniation is a rare disease, with only 26 cases reported before the current study. methods: Three cases of idiopathic spinal cord herniation are reported, and previous reports on this subject are reviewed. RESULTS: The responsible regions were in the thoracic spine from T2 to T7. Symptoms were mainly unilateral muscle atrophy in the lower extremity and sensory disturbance below the thoracic level. These symptoms had been progressing gradually. magnetic resonance imaging demonstrated a unique feature: The spinal cord shifted anteriorly in a few segments. Computed tomographic myelogram showed another distinctive picture: There was no subarachnoid space anterior to the spinal cord. CONCLUSIONS: Because idiopathic spinal cord herniation is out of the concept of "compression myelopathy," this condition may be a pitfall in the diagnosis. Idiopathic spinal cord herniation should be recognized as one of the treatable causes for thoracic myelopathy.
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keywords = rare disease
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4/15. Thoracic spine arachnoid ossification with and without cord cavitation. Report of three cases.

    Thoracic spine arachnoid ossification is a relatively rare disease that affects mainly women and causes sensory, motor, and sphinctal symptoms associated with inferior limb pain. Based on three cases, the authors comment on pathogenic and surgery-related aspects of the disease. The patient in Case 1 was followed over the course of 23 years. Spinal cavitation is highlighted in Case 2, and yellow, gross, half-ring ossification is described in Case 3. calcium deposits usually occur in the middle and lower thoracic spine where the majority of trabeculated arachnoid cells are located. Operative treatment does not interrupt the ossification process, which continues over time, causing progressive deterioration in the patient. Spinal cavitation can occur due to spinal cord tethering, stretching, and central cord edema formation, accompanied by cerebrospinal fluid blockage and pulse pressure changes. The results of surgical intervention are poor, offering short-term recovery with later deterioration. Multiple pathogenic factors are involved in this clinical syndrome including metabolic changes.
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5/15. Re-aggravation of myelopathy due to intramedullary lesion with spinal cord enlargement after posterior decompression for cervical spondylotic myelopathy: serial magnetic resonance evaluation.

    STUDY DESIGN: A case report. OBJECTIVES: To report a case of swelling of the spinal cord and an intramedullary lesion occurring after expansive laminoplasty for cervical spondylotic myelopathy. SETTING: A university hospital in japan. methods: Clinical evaluation, radiography, MR imaging. RESULTS: A 65-year-old man with a cervical spondylotic myelopathy in whom symptoms were improved immediately after expansive laminoplasty, but became aggravated 2 weeks later. Magnetic resonance images demonstrated swelling of the spinal cord and an intramedullary lesion that extended from the medulla oblongata to C7. Nine months after surgery, the lesion was reduced to C2-6, but neurological deterioration had not improved. Six years after surgery, the patient remains confined to bed. CONCLUSION: patients with such disease conditions are rare, and it is difficult to predict postoperative swelling of the spinal cord before surgery. spine surgeons should be aware of such rare disease conditions involving the spinal cord.
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ranking = 1
keywords = rare disease
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6/15. Spontaneous spinal epidural hematoma: an uncommon presentation of a rare disease.

    INTRODUCTION: Spontaneous spinal epidural hematoma is rare in children. The presenting symptoms are usually pain, either local or radicular, followed by progressive bilateral weakness, and sensory loss hours and even days later. In the absence of significant precipitating factors such as severe trauma or previously known coagulopathies the diagnosis is usually delayed, and it is not until the full picture of severe cord compression is developed, that MRI is done and the diagnosis is finally made. CASE REPORT: We describe a case of 10-year-old girl who presented with pain and pure brachial plexus radiculopathy as the only clinical manifestations of spinal epidural hematoma. CONCLUSION: A high index of suspicion can lead to the correct diagnosis even before the development of full cord compression and thus improve the overall prognosis.
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ranking = 4
keywords = rare disease
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7/15. Intradural extramedullary mass formation in spinal cord sarcoidosis: case report and literature review.

    STUDY DESIGN: A case of myelopathy caused by intradural extramedullary spinal cord sarcoidosis. OBJECTIVES: To report a rare case of mass formation in spinal cord sarcoidosis and discuss the diagnostic approach and therapeutic management of this condition. SUMMARY OF BACKGROUND DATA: spinal cord sarcoidosis is a rare condition that is difficult to diagnose, and intradural extramedullary mass formation is even rarer. MATERIALS AND methods: A 42-year-old woman first noticed numbness in her fingers, and these symptoms gradually spread to her hands and feet. MRI revealed an intradural extramedullary mass, as confirmed by an isointensity T1 and low-intensity T2 signal, and also enhanced by Gd-DTPA. The authors suspected the mass to be a meningioma and performed surgery to resect it. RESULTS: The mass was observed through the dura, and it was twined with the rootlet. After surgery, the patient experienced temporary paralysis of her right upper extremity, followed rapidly by almost complete neurologic recovery. Bilateral hilar lymphadenopathy was noted and this was diagnosed histologically as lung sarcoidosis. One year after surgery, a complete AV block occurred because of heart sarcoidosis and a pacemaker was inserted. CONCLUSIONS: spinal cord sarcoidosis is rare disease and is difficult to diagnose, but when a mass is present in the spinal canal, it is important to remain open to the possibility of spinal cord sarcoidosis. Besides if it is diagnosed as spinal cord sarcoidosis, it is also important to remain systemic sarcoidosis before, during, and after surgery to check general conditions and to guide appropriate treatment.
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ranking = 1
keywords = rare disease
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8/15. Case of idiopathic thoracic spinal cord herniation with a chronic history: a case report and review of the literature.

    Idiopathic spinal cord herniation is a rare disease that presents with slowly progressive myelopathy. This article describes the clinical findings of a patient with a chronic history. A 68-year-old woman initially presented at the age of 32 years with left leg weakness. After slowly progressive neurological deterioration over 34 years, she became completely paraplegic. At the age of 66 years, magnetic resonance imaging resulted in a diagnosis of idiopathic spinal cord herniation at the T6/7 level. Surgery was performed to reduce aching of the lower extremities. The spinal cord was released from the dural defect through a T5-T8 laminectomy. The dural defect was enlarged by resecting its periphery to prevent reherniation of the spinal cord. After the surgery, pain in the lower extremities resolved and her motor function slightly improved. Although operative treatment is naturally recommended at the early stage of this disease, our case suggests that some symptoms can be resolved by surgical treatment despite prolonged, severe preoperative symptoms.
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ranking = 1
keywords = rare disease
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9/15. Lumbar intramedullary spinal schistosomiasis presenting as progressive paraparesis: case report.

    OBJECTIVE AND IMPORTANCE: blood fluke infection or schistosomiasis affects up to 300 million people worldwide. Although infection of the hepatic and urogenital systems commonly occurs, central nervous system involvement is rare. When presenting in the spinal cord, schistosomiasis can be difficult to diagnose because it can present as mass lesion or transverse myelitis. We describe a patient with lumbar intramedullary spinal schistosomiasis who presented to a tertiary medical center in the united states and discuss the diagnosis and treatment of this rare disease. CLINICAL PRESENTATION: A 25-year-old Brazilian man presented with progressive, subacute, bilateral lower extremity motor and sensory deficits and disturbances in bladder function. magnetic resonance imaging revealed a diffusely enhancing mass expanding the region of the conus medullaris. INTERVENTION: Laboratory investigation disclosed serum and cerebrospinal fluid eosinophilia. Ultimately, cerebrospinal fluid serology demonstrated the presence of schistosoma mansoni. The patient was treated with praziquantel and steroids and made a marked recovery. CONCLUSION: The clinical and radiographic manifestations of spinal schistosomiasis can mimic those of intra-axial spinal tumors and transverse myelitis. To avoid unnecessary surgery or delay in treatment, the clinician must have knowledge of this type of presentation. The increasing volume of international travel and high prevalence of the disease worldwide increases the possibility that the practicing neurosurgeon in the united states may encounter this rare but treatable disease.
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ranking = 1
keywords = rare disease
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10/15. An intradural extramedullary tuberculoma of the spinal cord in a non-hiv-infected patient: case report and review of the literature.

    Spinal tuberculomas are extrapulmonary manifestations of tuberculosis involving the central nervous system. They are characterized as extradural, intradural extramedullary, or intradural, according to their location. Intradural extramedullary tuberculomas are extremely rare. There have been only 24 case reports found in English language literature. Our case is the only documented intradural extramedullary tuberculoma in a non-hiv-infected patient in north america. A literature review using a medline search from 1966 to the present is performed to characterize the clinical spectrum of the three types of tuberculomas and review the diagnosis and management of this potentially curable disease. An analysis of the cases of intradural extramedullary tuberculomas published since 1984 is performed to highlight the unique characteristics of this rare disease.
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ranking = 1
keywords = rare disease
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