Posterior mediastinal neuroblastoma masked as flaccid paraparesis in a 3 year child.

Neuroblastoma is an embryonic tumor of the sympathetic nervous system originating from neural crest cell remnants, which may be found in the adrenal glands or sympathetic chain. We report a case of a three-year-old girl who presented with flaccid paraparesis, and acute respiratory distress that required mechanical ventilation after a multiple-day history of gradual-onset dyspnea and right upper-lung opacity that was initially misdiagnosed as pneumonia. Chest and spinal magnetic resonance imaging (MRI) revealed a tumor in the posterior mediastinum with intra-spinal canal extension. The patient underwent thoracotomy and surgical en bloc resection with spinal canal decompression. Histopathology revealed neuroblastoma. The postoperative period was uneventful. The patient was administered a course of chemotherapy but died due to advanced stage tumor, severe pancytopenia, and septicemia with renal failure. The aim of this report is to consider posterior mediastinal neuroblastoma in the differential diagnosis of dyspnea and acute lower limb weakness in young children.

Intradural Extramedullary Epidermoid Cyst at the Conus Medullaris Level with Thoracic Syringomyelia: A Case Report.

Spinal epidermoid cysts are benign tumors. Syringomyelia secondary to intramedullary tumors are frequently observed. However, the association between syringomyelia and spinal intradural extramedullary epidermoid cyst in the conus medullaris region is extremely rare. We present the case of a 3-year-old male who was admitted with paraparesis and urinary retention. Magnetic resonance imaging (MRI) of the spine demonstrated intradural extramedullary lesion, compatible with epidermoid cyst, that at the conus medullaris level and a large syringomyelia extending from T4 to L1 vertebrae. Total microsurgical excision of the cyst was performed. No additional drainage was carried out for the syringomyelic cavity. Histopathological examination verified the diagnosis of the epidermoid cyst. Total excision of the cyst and disappearance of the syringomyelia were observed on MRI at 15 days postoperatively. We have clarified the etiology, clinical, histopathological and radiological features, differential diagnosis, and treatment modalities of spinal epidermoid cysts. In addition, we have discussed the possible mechanisms of syringomyelia formation in spinal intradural lesions.

Natural Course of Myxopapillary Ependymoma: Unusual Case Report and Review of Literature.

BACKGROUND: Spinal cord ependymomas are the most frequent primary intramedullary tumors of the cord in middle age (40-60 years of age). Myxopapillary ependymomas are low-incidence tumors and occur in the cauda equina and conus medullaris. They are typically described as fleshy, sausage-shaped, vascular lesions. CASE DESCRIPTION: In this report, we present a 36-year-old woman who was seen in another hospital with complaints of back pain radiating into both legs 5 years ago. Magnetic resonance imaging (MRI) examinations revealed a lesion releated to the spinal cord. At that point, the patient refused the proposed surgical treatment. Five years later, the patient was referred to our clinic with advancement of the complaint to paraparesis. The new MRI showed an intradural mass at the level of T11 and S1. We performed a total intradural tumor resection. Histopathologic examination revealed a myxopapillary ependymoma. CONCLUSIONS: Myxopapillary ependymomas grow slowly and are well defined by MRI. Advancements in radiologic technology and surgical equipment have allowed this tumor type to be treated successfully. Unfortunately, if untreated, these lesions have poor prognosis and result in clinical symptoms such as paraparesis. Surgical treatment of these lesions using total resection was performed total resection and was associated with an excellent postoperative outcome. We highlight the natural course of myxopapillary ependymomas.

Histopathological proof of the pathogenicity of a rare GFAP mutation in a patient with flaccid paraparesis.

Infantile Alexander disease is a rare progressive leukodystrophy caused by autosomal dominant mutations in the (GFAP) gene typically presenting with psychomotor retardation, progressive macrocephaly and refractory epilepsy. Neuroradiological hallmarks are extensive white matter lesions with frontal preponderance as well as signal intensity changes of basal ganglia and medulla oblongata with variable contrast enhancement. Here, we report an atypical manifestation in a 21-month-old boy presenting with flaccid paraparesis and areflexia. Cognitive, visual as well as fine motor skills and muscular strength of the upper extremities were appropriate for age. Weight and height as well as head circumference were within normal range. Clinical or electroencephalographic signs of seizures were absent. Cranial MRI demonstrated bifrontal cystic tumorous lesions with partial contrast rims, as well as space-occupying focal lesions of the caudate nuclei. Spinal MRI revealed swelling of the lumbar and cervical spinal cord. CSF and blood chemistry showed normal results. Histopathology of a subcortical lesion showed large amounts of Rosenthal fibers and protein droplets characteristic of Alexander disease. Sequencing detected a heterozygous mutation of the GFAP gene (c.205G > A; p.(Glu69Lys)) that has been reported before as probably pathogenetic in another case of lower spinal involvement. This well documented case draws attention to atypical spinal manifestations of Alexander disease and gives histopathological proof of the pathogenetic role of a rare GFAP mutation with marked spinal involvement.

A thoracic vertebral brown tumor presenting with paraparesis in a patient with end-stage renal disease.

Vertebral brown tumors are rare, non-neoplastic bone lesions that occur in the setting of hyperparathyroidism. There are differences in the management of them in the literature. Because brown tumors usually resolve after a parathyroidectomy. We present a case of a thoracic vertebral brown tumor with paraparesis.

Warfarin-associated Intraspinal Hematoma.

Intracerebral hemorrhage is a well-known complication resulting from warfarin use; however, warfarin-associated intraspinal hematoma is very rare. Warfarin-associated intraspinal hematoma may exhibit delayed progression, and patients may present with atypical symptoms, occasionally resulting in delayed diagnosis. We report the case of a 65-year-old man who visited our emergency department (ED) with acute urinary retention. He had been previously diagnosed with non-valvular atrial fibrillation, arterial hypertension, and benign prostatic hyperplasia, and he used warfarin for the prevention of systemic embolism. The patient was initially diagnosed with worsening of the prostatic hyperplasia. After 2 days, he revisited the ED with painless paraparesis. Magnetic resonance imaging of the thoracic spine revealed an intraspinal hematoma at Th7-8, and blood coagulation tests indicated a prothrombin time-international normalized ratio of 3.33. Despite attempts to reverse the effects of warfarin with vitamin K administration, the paraparesis progressed to paraplegia, necessitating urgent surgical removal of the hematoma. Partial recovery of motor function was evident after surgery. From the present case, we learned that intraspinal hematoma should be included in the differential diagnosis of patients using warfarin who present with acute urinary retention. Although there are no evidence-based treatment guidelines for warfarin-associated intraspinal hematoma, surgical treatment may be warranted for those who exhibit neurological deterioration.

Thoracic Hemangioma From Rib Presenting as Compressive Paraparesis in a Young Adult: A Treatment Dilemma.

STUDY DESIGN: A case report. OBJECTIVE: To describe the presentation of compressive paraparesis as a result of thoracic rib hemangioma in a young adult and its nonsurgical management. SUMMARY OF BACKGROUND DATA: Hemangiomas are rare bone tumors and those arising from rib are rarer. Only about 50 such cases have been reported in literature so far. METHODS: A 21-year-old male student, presented to us with a 6-week history of progressive weakness in both lower limbs and loss of bowel bladder control. Patient gave history of being operated for left periscapular tumor treated with wide excision and proven with biopsy to be a hemangioendothelioma (benign but locally aggressive hemangioma variant) a year ago. RESULTS: New radiograph of the chest showed an expansile lesion of left fifth rib and magnetic resonance image showed a tumor of left dorsal thoracic wall with AV malformation causing compressive thoracic myelopathy at T5 level vertebrae. We planned for immediate decompression surgery for spine along with excision of tumor with the help of a thoracic surgeon. However, on preoperative digital subtraction angiography, the tumor was found to be highly vascular with high risk of intraoperative bleeding and morbidity. So, the plan was revised and the patient underwent digital subtraction angiography, followed by embolization by an expert interventional neurosurgeon. The patient showed signs of recovery within a week. Lower limb power improved from grade 2 to 3/5 to grade 4 to 4+/5. The patient became ambulatory with single stick at 3-month follow-up; he was a nonwalker to start with. At 2 years plus follow-up, the patient fully recovered and walks without stick. CONCLUSION: This unique case brings to light the dilemma a spine surgeon sometimes faces. A case that warranted immediate surgical intervention based on clinical findings was treated with interventional fibrin glue embolizations with excellent results. LEVEL OF EVIDENCE: N/A.

Neurological deterioration induced by sitting in patients after cervicothoracic posterior decompression with instrumented fusion surgery for ossification of the longitudinal ligament: two cases reports.

BACKGROUND: We report on Japanese patients who showed neurological deterioration induced by sitting after cervicothoracic posterior decompression with instrumented fusion, but showed immediate neurological recovery after bed rest. CASE PRESENTATION: Patients showed incomplete paraparesis caused by the ossification of the posterior longitudinal ligament at uppermost thoracic spine. Cervicothoracic posterior decompression with instrumented fusion was performed. Postoperatively, the patients showed partial paraparesis when they were sitting. They showed rapid recovery from lower extremity paralysis upon lying down. After strict bed rest for one month, those patients showed no apparent development of paralysis during sitting. CONCLUSION: In patients with postoperative residual anterior spinal cord compression, micromotion might exacerbate neurological symptoms.

Thoracic disk herniation with paraparesis treated with transthoracic microdiskectomy in a 14-year-old girl.

Herniated thoracic intervertebral disk causing spinal cord compression with paraparesis is uncommon in adults and rare in children. This article describes a case of pediatric thoracic disk herniation with paraparesis treated surgically.A 14-year-old girl presented with a 4-month history of diffuse back pain and sudden onset paraparesis. Motor strength was 4/5 in both legs, and she had lost the ability to ambulate. Magnetic resonance imaging revealed spinal cord compression due to a herniated intervertebral disk at T5-T6. Computed tomography scan after myelogram demonstrated anterior dural sac compression at T5-T6 but no intervertebral disk calcification. She underwent transthoracic microdiskectomy. The herniated disk was removed, and the thoracic spinal cord was decompressed. No fusion was performed after microdiskectomy. The postoperative course was uncomplicated, and neurologic deficit resolved within 2 weeks postoperatively. The patient was pain free with no neurologic deficit at 24-month follow-up, and computed tomography scan showed remodeling of the T5 and T6 vertebral bodies.Most cases of thoracic disk herniation are asymptomatic. If no compression of the spinal cord exists, the natural history of the disease justifies conservative management. Although the treatment of choice is conservative, surgery is required in patients who develop progressive neurologic deficit or severe radicular pain. Transthoracic microdiskectomy without fusion is considered a treatment in similar cases.

Sodium 4-phenylbutyrate protects against spinal cord ischemia by inhibition of endoplasmic reticulum stress.

OBJECTIVE: Delayed paraplegia after operation on the thoracoabdominal aorta is considered to be related to vulnerability of motor neurons to ischemia. Previous studies have demonstrated the relationship between neuronal vulnerability and endoplasmic reticulum (ER) stress after transient ischemia in the spinal cord. The aim of this study was to investigate whether sodium 4-phenylbutyrate (PBA), a chemical chaperone that reduces the load of mutant or unfolded proteins retained in the ER during cellular stress, can protect against ischemic spinal cord damage. METHODS: Spinal cord ischemia was induced in rabbits by direct aortic cross-clamping (below the renal artery and above the bifurcation) for 15 minutes at normothermia. Group A (n = 6) was a sham operation control group. In group B (n = 6) and group C (n = 6), vehicle or 15 mg/kg/h of sodium 4-PBA was infused intravenously, respectively, from 30 minutes before the induction of ischemia until 30 minutes after reperfusion. Neurologic function was assessed at 8 hours, and 2 and 7 days after reperfusion with a Tarlov score. Histologic changes were studied with hematoxylin-eosin staining. Immunohistochemistry analysis for ER stress-related molecules, including caspase12 and GRP78 were examined. RESULTS: The mean Tarlov scores were 4.0 in every group at 8 hours, but were 4.0, 2.5, and 3.9 at 2 days; and 4.0, 0.7, and 4.0 at 7 days in groups A, B, and C, respectively. The numbers of intact motor neurons at 7 days after reperfusion were 47.4, 21.5, and 44.9 in groups A, B, and C, respectively. There was no significant difference in terms of viable neurons between groups A and C. Caspase12 and GRP78 immunoreactivities were induced in motor neurons in group B, whereas they were not observed in groups A and C. CONCLUSION: Reduction in ER stress-induced spinal cord injury was achieved by the administration of 4-PBA. 4-PBA may be a strong candidate for use as a therapeutic agent in the treatment of ischemic spinal cord injury.

Spontaneous spinal epidural haematoma in a geriatric patient on aspirin.

Spontaneous spinal epidural haematoma (SSEH) is a rare cause of spinal cord compression in adults, especially in the elderly. We report an independent 88-year-old female, on aspirin only for chronic atrial fibrillation, who presented with a 12-hour history of acute lumbar back pain, urinary incontinence and progressive bilateral lower limb paresis. Examination revealed saddle anaesthesia and reduced anal tone. Urgent spinal MRI demonstrated an epidural haematoma extending from T7 to L5. The patient made a poor initial post-operative recovery, but four months later had begun to mobilise independently after intensive physiotherapy. The case highlights the significance of clinical suspicion, especially in those patients on anti-platelet therapy, rapid spinal radiography and emergent decompressive surgery in SSEH patients, as well as the importance of ongoing rehabilitation in restoring neurological function.

Spinal intradural extramedullary hydatidosis: report of three cases.

OBJECTIVE: Spinal hydatid cyst is a serious form of hydatid disease affecting fewer than 1% of all patients with hydatid disease. We report 3 healthy patients who presented with progressive paraparesis attributed to a histologically proven intradural hydatid cyst. METHODS: There were 2 children (1 boy, 1 girl) and 1 adult with a mean age of 12 years. The median follow-up duration was 16 months. Spinal magnetic resonance imaging was performed in the 3 patients, and an anatomic and topographical diagnosis of the intradural hydatid cyst was made. RESULTS: Magnetic resonance imaging scans revealed cystic lesions with peripheral contrast enhancement. Surgery was performed through laminectomy, complete resection was achieved, and antihelminthic treatment with albendazole 10 mg/kg-1 per day for 6 months was included in the postoperative treatment. The patients improved after surgery with normal motor function. CONCLUSION: This localization is rare and serious, but its prognosis is excellent if diagnosis is made early enough and surgery is performed in time to prevent cyst rupture.

Multiple revisions of a L2 burst fracture in a suicide jumper: a retrospective analysis of what went wrong.

A 35-year-old female patient sustained three contiguous vertebral fractures at the thoracolumbar junction while jumping off the third floor in a suicide attempt. Initial fracture treatment occurred in the setting of a multiple injury scenario. While the Th12 and the L1 vertebral fractures were considered stable, the L2 fracture exhibited a complete burst configuration with 80% canal compromise due to a posterior wall fragment causing paraplegia. A posterior pedicle screw stabilisation with indirect fracture reduction was carried out initially from T12 to L3. At 1 year follow-up the patient presented to us for new onset radiculopathy L2, and loss of correction. A circumferential revision surgery with an expandable cage was carried out to restore the anterior and posterior columns. Unfortunately again loss of reduction with kyphosis occurred, this time at the upper instrumented vertebra, which made another revision necessary. In this situation a longer construct was chosen using a combined approach and a Mesh cage. This later procedure was complicated by a postoperative paraparesis believed to be vascular in origin. Six months later a further complication involving MSSA deep wound infection required a series of irrigation debridement for healing. At the 2.5 years follow up the spine was stable and the patient had a neurologic recovery allowing her to ambulate with crutches. This Grand Round Case raises the question on the initial management of multiply injured patients with spine fracture, the classification of these fractures, the optimal initial internal fixation, the need for complementary anterior column reconstruction and the strategy when all these fails.

Intratumoral hemorrhage of spinal schwannoma of the cauda equina manifesting as acute paraparesis--case report.

A 64-year-old man presented with spinal schwannoma of the cauda equina at the thoraco-lumbar junction manifesting as acute paraparesis and urinary incontinence after intratumoral hemorrhage. Surgical resection of the tumor resulted in significant neurological recovery. T(2)(*)-weighted gradient-echo magnetic resonance imaging is very useful to help establish the preoperative diagnosis, leading to early surgical intervention and better outcome after surgery.

Malignant intracranial meningioma with spinal metastasis--case report.

A 48-year-old woman presented with a left cerebellopontine angle mass. Over a 93-month period, the patient underwent seven surgeries, two radiosurgeries, and one external beam radiotherapy. The tumor was histologically benign at the first operation, but exhibited unusually aggressive behavior after failed radiosurgery and demonstrated clinical characteristics of malignancy such as spinal metastasis. The patient underwent gamma knife radiosurgery (GKR) for recurrence after the first operation, despite the tumor being located in a resectable region. The tumor did not respond. Six months after the sixth surgery, the patient presented with progressive lower extremity paraparesis and sensory disturbance below the T11 dermatome. Magnetic resonance imaging revealed multiple intradural mass lesions located at the T2, T11-T12, and L2 levels. She died 4 months after the diagnosis of spinal metastases. Retrospectively, we speculate that if a tumor is located in a resectable region and Simpson grade I or II tumor resection is possible, direct surgery may be a safer option than GKR.