Spinal thoracic extradural angiolipoma manifesting as acute onset of paraparesis: Case report and review of literature.
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ABSTRACT:
SUBMITTER: Onishi FJ
PROVIDER: S-EPMC5525459 | biostudies-other | 2017
REPOSITORIES: biostudies-other
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Project description:Introduction and importanceBrown tumor (BT) is defined as osteolytic lesion of an underlying state of hyperparathyroidism. Hyperparathyroidism will activate osteoclasts which initiate active bone resorption foci of lytic-cysts with hemosiderin depositions that pigment it with its characteristic brown pathologic gross appearance. Devastating fractures and injuries can occur to affected bones and surrounding tissue that require emergent intervention and correction.Case presentationWe present a case of a medically free 31-year-old female patient, who presented complaining of unsteadiness and progressive lower limbs weakness over 40 days of duration. Subsequent lab tests showed elevated PTH levels, along with 3.5 × 1.8 cm heterogeneous soft tissue mass involving the right pedicle on T7 level compressing the corresponding level of the spinal cord. Surgical management aimed to decompress the spinal cord and to obtain a biopsy for histopathologic examination which revealed a brown tumor. Neck ultrasound and Sestamibi scan indicated the presence of hyperactive and hyperplastic parathyroid tissue most suggestive of parathyroid adenoma.Clinical discussionVarious presentations of Brown Tumor depend on the bone affected, despite the rarity of spinal involvement, yet expanding tumors can manifest either with back pain, radicular pain, paresthesia, weakness, paralysis, or incontinence. The highest incidence rates of spinal brown tumors affect adults over the age of 40. Management goals are to decompress the neuronal tissue emergently and to prevent further bony lytic deterioration.ConclusionThe objective of this study is to provide an overview of primary hyperparathyroidism-related spinal brown tumors, presentation, and summary of previously reported similar cases in the literature.
| S-EPMC7933711 | biostudies-literature
Project description:Background Meningiomas are mostly benign and slow-growing neoplasms of the central nervous system. Spinal meningiomas account for up to 45% of all intradural spinal tumors in adults and up to 25%–45% of all spinal tumors. Spinal extradural meningiomas are rare and may be easily confused with malignant neoplasms. Case description A 24-year-old woman was presented to our hospital with paraplegia and loss of sensation in the T7 dermatome and lower body. MRI findings showed T6-T7 right-sided intradural extramedullary and extradural lesion, measuring 1.4 cm × 1.5 cm × 3 cm, extending to the right foramen, compressing the spinal cord, and displacing it to the left. Hyperintense lesion on T2 and hypointense lesion on T1 were observed. The patient reported improvement after surgery and during follow-up. We recommend maximizing the decompression during surgery to achieve better clinical outcome. Extradural meningiomas represent 5% of all meningiomas; therefore, having an intradural on top of extradural meningioma with extraforaminal extensions makes this a unique and rare case. Conclusion Meningiomas can be easily missed in diagnosis depending on imaging and the pathognomonic pattern it represents, which can mimic other pathologies, such as schwannomas. Therefore, surgeons should always suspect their patient having a meningioma even if the pattern is not typical. Moreover, preoperative preparation, such as navigation and defect closure, must be taken in case it turns out be a meningioma instead of the presumed pathology.
| S-EPMC10150021 | biostudies-literature
Project description:BackgroundSpontaneous subdural hematoma rarely presents with a hypervascular or malignant tumor but even less frequently in a benign tumor like meningioma. We encountered a patient with acute subdural hematoma associated with benign meningioma. Here, we report this case along with a review of previous reports, especially focusing on their clinical features and possible bleeding mechanisms.Case presentationA 53-year-old Asian woman presented with severe headache and progressive neurologic deterioration due to cerebral edema. The patient was submitted to open surgery for evacuation of the subdural hematoma and concurrent tumor removal on the ipsilateral parietal convexity. A hypervascular, encapsulated mass was identified during surgery and completely removed including the adjacent dura mater (Simpson grade 0). The tumor was histologically confirmed as an angiomatous meningioma (World Health Organization grade I). Her clinical course was uneventful after surgery.ConclusionsAlthough meningiomas are commonly benign according to their histological traits, they can lead to spontaneous bleeding and cause neurologically unstable condition. Therefore, meningiomas need to be considered as a cause of spontaneous subdural hematoma if radiologically suspicious, which should be reflected by proper management for a positive outcome.
| S-EPMC8244191 | biostudies-literature
Project description:PurposeThe aim of this publication is to present a case of idiopathic spinal cord herniation (ISCH) associated with a transdural disk herniation, demonstrate an operative technique used to treat this condition and provide an updated review the literature.Background contextISCH is an infrequent condition that can cause progressive myelopathy leading to severe neurological dysfunction. This condition is characterized by ventral displacement of the spinal cord across a defect in the dura, either congenital or acquired, resulting in vascular compromise and adhesion that subsequently causes injury to the spinal cord. We present the management of such a patient, in addition to a review of the literature regarding management of ISCH.MethodsThis patient underwent surgery using the dural graft sling technique for repair of the dural defect and restoration of normal spinal cord position within the thecal sac. A review of the literature revealed a total of 171 patients supplemented by our 1 patient, which were then analyzed.ResultsThe majority of patients, treated with a variety of surgical techniques, experienced improvements in symptomatology. Our patient experienced significant improvement in symptomatology.ConclusionsAlthough ISCH is a rare clinical condition that causes myelopathy, patients managed with surgery generally, though not universally, have a favorable neurological outcome. The associated surgical technique video demonstrates the dural sling technique for the treatment of this rare disorder.
| S-EPMC7337104 | biostudies-literature
Project description:Background and importanceIdiopathic spinal cord herniation (ISCH) is an uncommon condition located predominantly in the thoracic spine and often associated with a remote history of a major traumatic injury. ISCH has an incompletely described presentation and unknown etiology. There is no consensus on the treatment algorithm and surgical technique, and there are few data on clinical outcomes.Clinical presentationIn this case series and technical report, we describe the atypical myelopathy presentation, remote history of traumatic injury, radiographic progression, treatment, and outcomes of 5 patients treated at Washington University for symptomatic ISCH. A video showing surgical repair is presented. In contrast to classic compressive myelopathy symptomatology, ISCH patients presented with an atypical myelopathy, characterized by asymmetric motor and sensory deficits and early-onset urinary incontinence. Clinical deterioration correlated with progressive spinal cord displacement and herniation observed on yearly spinal imaging in a patient imaged serially because of multiple sclerosis. Finally, compared with compressive myelopathy in the thoracic spine, surgical treatment of ISCH led to rapid improvement despite a long duration of symptoms.ConclusionSymptomatic ISCH presents with atypical myelopathy and slow temporal progression and can be successfully managed with surgical repair.
| S-EPMC4134727 | biostudies-literature
Project description:RATIONALE:Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease that is commonly characterized by massive painless cervical lymphadenopathy and systemic manifestations. Isolated extranodal involvement, especially spinal involvement, is extremely rare. PATIENT CONCERNS:A 28-year-old man presented with intermittent dorsodynia and bilateral lower-limb weakness and numbness. A magnetic resonance scan (MRI) showed an extradural lesion of the T6-T9 thoracic spine that lead to cord compression. DIAGNOSES:Histopathological findings showed distinctive emperipolesis and immunohistochemistry results that were positive for cluster of differentiation CD68 and S100. Therefore, we diagnosed the Rosai-Dorfman disease. INTERVENTIONS:we performed a nearly total surgical resection and a limited T6-T9 laminectomy. OUTCOMES:Postoperatively, the patient's symptoms were partially relieved and experienced no recurrence during the 6-month follow-up. The preoperative diagnosis of isolated spinal RDD still remains challenging. LESSONS:Thus, we should consider RDD in the differential diagnosis of the central nervous system. Besides surgical resection, the treatment also included radiation, chemotherapy or monoclonal antibodies. However, the optimal treatment remains controversial. Therefore, we should exert all our energies on the exploration of etiology and adjuvant therapy for this disease.
| S-EPMC6200482 | biostudies-other
Project description:Diagnosis of pediatric intellectual disability (ID) can be difficult because it is due to a vast number of established and novel causes. Here, we described a full-term female infant affected by Kleefstra syndrome-2 presenting with neurodevelopmental disorder, a history of hypotonia and minor face anomalies. A systematic literature review was also performed. The patient was a 6-year-old Caucasian female. In the family history there was no intellectual disability or genetic conditions. Auxological parameters at birth were adequate for gestational age. Clinical evaluation at 6 months revealed hypotonia and, successively, delay in the acquisition of the stages of psychomotor development. Auditory, visual, somatosensory, and motor-evoked potentials were normal. A brain MRI, performed at 9 months, showed minimal gliotic changes in bilateral occipital periventricular white matter. Neuropsychiatric control, performed at 5 years, established a definitive diagnosis of childhood autism and developmental delay. Molecular analysis of the exome revealed a novel KMT2C missense variant: c.9244C > T (p.Pro3082Ser) at a heterozygous state, giving her a diagnosis of Kleefstra syndrome 2. Parents did not show the variant. Literature review (four retrieved eligible studies, 10 patients) showed that all individuals had mild, moderate, or severe ID; language and motor delay; and autism. Short stature, microcephaly, childhood hypotonia and plagiocephaly were also present. Conclusion. Kleefstra syndrome 2 is a difficult diagnosis of a rare condition with a high clinical phenotypic heterogeneity. This study suggests that it must be taken in account in the work-up of an orphan diagnosis of intellectual disability and/or autism spectrum disorder.
| S-EPMC8954887 | biostudies-literature
Project description:BackgroundClassic cerebrotendinous xanthomatosis (CTX; OMIM #213700) manifests with chronic diarrhea, juvenile cataracts, tendon xanthomas and neurological symptoms. It is due to biallelic inactivation of CYP27A1 wich leads to cholestanol accumulation in the central nervous system, eyes and tendons. Less commonly, the disease can present in young adults as spastic paraparesis in the absence of xanthomas.Case presentationWe report a 38-year old woman who presented with chronic diarrhea and progressive spastic paraparesis in her twenties. Brain magnetic resonance imaging (MRI) showed cerebral atrophy with diffuse periventricular white matter hyperintensities. Spinal MRI was normal. CYP27A1 gene sequencing confirmed the diagnosis of CTX. Chenodeoxycholic acid (CDCA) treatment was introduced with remission of diarrhea. Unfortunately, the treatment had to be discontinued several times and the patient developed psychosis and an severe ataxospastic gait. Spinal MRI revealed new linear hyperintensities of the corticospinal and gracile tracts. Thirty-three spinal CTX patients were identified by searching in Pubmed, EMBASE™ and Web of Science databases. All patients presented pyramidal signs and 48% had dorsal column signs. Juvenile cataracts were described in 78% of patients, chronic diarrhea in 65%, and tendon xanthomas in 31%. Disease improvement or stabilization with chenodeoxycholic acid was observed in 69% of patients. A higher prevalence of the Arg395Cys allele was observed in patients with spinal CTX as compared to CTX in general (ᵡ2; p < 0.00001).ConclusionsThe diagnosis of spinal CTX can be easily missed or delayed in absence of xanthomas. There is a higher prevalence of the Arg395Cys allele in spinal CTX as compared to classic childhood-onset CTX. CDCA treatment seems to stabilize or improve clinical symptoms in most patients. However, as seen in our patient and in two previously reported cases, sudden interruption of CDCA may lead to irreversible neurological complications.
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Project description: Not available
| S-EPMC9171304 | biostudies-literature
Project description:Spinal epidermoid tumors are rare, benign tumors that are either acquired from trauma, surgery, or lumbar puncture or arise as congenital lesions, particularly spinal dysraphisms. We report a case of a massive spinal epidermoid tumor and review the literature with a focus on the surgical outcomes. A 71-year-old female patient presented after a fall with subsequent symptoms of severe back and hip pain, as well as loss of motor strength in the left leg. Her magnetic resonance imaging demonstrated a T2/short tau inversion recovery hyperintense mass extending from the level of the T10-11 disc caudally through S2. A biopsy was recommended to determine whether the tumor was radio- or chemo-sensitive. The patient underwent a L4 laminectomy and a pearly-white tumor was encountered, with a subsequent biopsy confirming it to be an epidermoid tumor. The following conclusions can be drawn from a review of the literature. Spinal epidermoid tumors are more common in women and tend to present in younger patients (median age of 23). The majority of patients had acquired lesions (46%). In terms of surgical outcomes for adherent tumors, gross total resection was found to provide optimal outcomes, with 90% of patients improving clinically after surgery.
| S-EPMC6104727 | biostudies-literature