搜索结果：Journal of neurosurgery. Case lessons

Ambiguous destructive lumbosacral tumors with intractable pain and neurological compromise present a dilemma between timely decompression and oncological adequacy. The authors present a rare case of primary unclassified sarcoma of the sacrum to illustrate the implications of diagnostic uncertainty in surgical decision-making. A 59-year-old woman presented with excruciating low back pain and bilateral sciatica from a destructive lumbosacral mass spanning L5-S4 with circumferential cauda equina compression. CT-guided biopsy demonstrated an atypical epithelioid and spindle cell neoplasm, not otherwise classified. Given rapid neurological decline and refractory pain, she underwent preoperative embolization and urgent posterior decompression with multilevel laminectomies (L4-S4), transpedicular corpectomies (S1-3), and lumbopelvic fixation, followed by paraspinous flap closure. Frozen-section analysis favored a tenosynovial giant cell tumor (TGCT) to support a nerve root-sparing resection. Postoperative MRI showed small residual enhancement at S1-2. Final pathological analysis revealed an intermediate-grade unclassified sarcoma, and she subsequently underwent adjuvant radiation therapy. Systemic staging is ongoing. At the 6-month follow-up, she reported durable analgesia and function. Destructive lumbosacral tumors should be approached with an oncological mindset even when histology is uncertain. In urgent cases, timely decompression and functional preservation may outweigh radical resection, underscoring the value of adaptable, multidisciplinary management. https://thejns.org/doi/10.3171/CASE2646.

Neuromodulation, including deep brain stimulation (DBS), is a treatment option for patients with medically intractable epilepsy who do not meet candidacy for resective surgery. DBS of the anterior nucleus of the thalamus (ANT) has been FDA approved for focal epilepsy, and stimulation of the centromedian nucleus of the thalamus (CMT) is increasingly being explored for generalized epilepsies including juvenile myoclonic epilepsy (JME). The authors present the case of a patient with JME and medically intractable seizures who underwent bilateral, sequential ANT and CMT DBS implantation. This case describes a 51-year-old woman with medically intractable JME in whom adequate trials of more than 10 antiseizure medications and prior vagus nerve stimulation failed. She underwent bilateral ANT DBS, which reduced her generalized tonic-clonic seizures but did not improve her myoclonic jerks. Owing to persistent disability from daily myoclonus, she later underwent bilateral CMT DBS. Following CMT stimulation, she experienced rare myoclonic jerks. At 7 months postoperatively, her status remained Engel class IIb with sustained functional improvement. This case highlights the complementary roles of ANT and CMT stimulation in modulating distinct thalamocortical pathways. Although CMT DBS has been described in patients with generalized epilepsy, this case demonstrates a meaningful reduction specifically in disabling myoclonic jerks that previously resulted in falls and dropped objects. https://thejns.org/doi/10.3171/CASE251033.

Vestibular schwannomas (VSs) are common benign tumors of the cerebellopontine angle, often treated with surgery or stereotactic radiosurgery to preserve nerve function. Malignant transformation of VS (MTVS) is exceedingly rare and has been primarily associated with neurofibromatosis type 1 (NF1), neurofibromatosis type 2-related schwannomatosis (NF2-SWN), or prior radiation exposure. However, cases of MTVS occurring without these risk factors are rare and remain poorly understood. A 58-year-old woman without neurofibromatosis underwent subtotal VS resection in 2019, with stable residual tumor and no adjuvant treatment. In 2023, she developed acute neurological decline, and imaging suggested malignant transformation or a new tumor. Surgery confirmed a high-grade neoplasm, likely leiomyosarcoma or malignant peripheral nerve sheath tumor, with genetic analysis revealing no NF1 or NF2-SWN mutations. She received adjuvant stereotactic radiation therapy, and at the 3-month follow-up, imaging showed stable residual disease. However, she continued to experience persistent neurological deficits, including facial weakness, ataxia, and intention tremor, with a Karnofsky Performance Scale score of 60%. MTVS is extremely rare without neurofibromatosis or prior radiation therapy, potentially driven by surgical inflammation or unidentified genetic alterations. While increased surveillance after subtotal resection may be considered, more research is needed to identify predictive markers and guide clinical management. https://thejns.org/doi/10.3171/CASE251003.

Spinal hemorrhagic disorders are relatively uncommon. Hematomas occurring outside the spinal canal, particularly around the spinal nerve root, are especially rare. To date, reported cases have been limited to a single case associated with a nerve root block procedure and 9 cases of Tarlov cyst hemorrhage. A 35-year-old woman presented with acute right L5 radiculopathy and extensor hallucis longus weakness. MRI demonstrated a septated cystic lesion in the right L5-S1 extraforaminal region adjacent to the L5 nerve root. Fluoroscopy-guided aspiration yielded a dark red liquefied hematoma with immediate symptom relief and radiographic reduction. The patient experienced recurrence 10 days later with worsened weakness; repeat aspiration of xanthochromic fluid resulted in rapid recovery. No recurrence was observed during 3 years of follow-up. This appears to be the first reported case of spontaneous perineural hematoma involving a spinal nerve root. Acute unilateral lower limb radicular pain is commonly attributed to lumbar disc herniation. However, MRI may reveal an atypical lesion adjacent to the nerve root. Perineural hematoma should be included in the differential diagnosis of acute radiculopathy. Fluoroscopy-guided needle aspiration may offer a minimally invasive treatment option. Early MRI evaluation is important for accurate diagnosis and timely management. https://thejns.org/doi/10.3171/CASE26413.

Spontaneous intracranial hypotension (SIH) is an underrecognized yet increasingly diagnosed cause of orthostatic headaches. Reduction of intracranial CSF volume and pressure produces characteristic imaging findings such as diffuse pachymeningeal enhancement and cerebellar tonsillar descent, mimicking a Chiari malformation type I. Distinguishing SIH from other causes of hindbrain herniation is critical for prompt recognition and targeted treatment to resolve symptoms and radiological abnormalities. A 55-year-old male patient presented with a history of persisting postural headaches, back pain, and sensory loss. MRI of the spine demonstrated a subtle ventral epidural fluid collection extending along the cervical and thoracic spine. Intraoperative digital subtraction myelography confirmed a CSF leak site at the T3-4 level due to a dural defect. Under the same anesthetic, a posterior T3-4 laminectomy and intradural repair of the ventral dural defect were performed. The patient recovered with complete resolution of symptoms and no neurological deficits or weakness. Postoperative MRI showed resolution. Identifying a pathological defect can be challenging in SIH, especially in chronic cases that mimic Chiari malformation type I due to tonsillar decent. The authors illustrate the clinical, radiological, and surgical framework utilized at their institution for managing a chronic SIH case due to a ventral dural defect. https://thejns.org/doi/10.3171/CASE26369.

Blister aneurysms (BAs) constitute an uncommon and distinct subtype of intracranial aneurysm. Owing to their inconspicuous morphology and nonbranching location, they are often not apparent on conventional angiographic studies. The pathophysiology of BAs remains incompletely elucidated, with arterial dissection considered a principal pathogenic mechanism. A 49-year-old woman presented with a spontaneous subarachnoid hemorrhage of unknown etiology. Initial lumen-based imaging was nondiagnostic. Vessel wall MRI (VW-MRI) discerned a small hemispheric protrusion without a definable neck, arising from the left A3 segment. Postcontrast imaging demonstrated aneurysmal wall enhancement, a feature correlated with recent rupture in the context of a subarachnoid hemorrhage, implicating this lesion as the presumed hemorrhagic source. Concomitantly, eccentric mural thickening with intrinsic T1 and T2 hyperintensity was evident along the parent artery, supportive of intramural hematoma and underlying arterial dissection. This case emphasizes the adjunctive value of VW-MRI in the diagnostic workup of suspected BAs. By targeting the arterial wall beyond the luminal contour, VW-MRI enhances lesion visibility and delineates associated parent artery abnormalities. https://thejns.org/doi/10.3171/CASE26248.

Bow hunter's syndrome (BHS), or rotational vertebral artery (VA) occlusion, is a rare cause of vertebrobasilar insufficiency resulting from dynamic compression of the VA during head rotation. Classically described at the atlantoaxial level with rotational occlusion, subaxial and nonrotational variants exist and can remain underdiagnosed as standard vascular imaging often fails to reveal positional occlusion. A 54-year-old man with no vascular risk factors presented with recurrent posterior circulation strokes despite medical management. Conventional imaging identified a left VA occlusion but failed to reveal the cause. Dynamic digital subtraction angiography revealed paradoxical reopening of the left VA with neck extension, confirming BHS at the C6-7 level. The patient underwent direct decompression and anterior cervical decompression and fusion, resulting in restoration of VA flow and clinical stabilization. This case highlights an underrecognized diagnostic pitfall of BHS through a presentation where static imaging suggested a fixed VA occlusion. Nonrotational variants at a subaxial level may mask positional occlusion. Dynamic imaging should be pursued in patients with recurrent posterior circulation infarcts despite seemingly explanatory static image findings. Recognition of these atypical patterns can help timely intervention to achieve durable prevention of further ischemic events. https://thejns.org/doi/10.3171/CASE2666.

In moyamoya disease, fragile collateral networks such as periventricular anastomoses are associated with an increased risk of hemorrhage, particularly in the posterior circulation. Peripheral aneurysms arising from these vessels are rare, and those involving the artery of Percheron are exceptionally uncommon. A 55-year-old patient with a history of thalamic hemorrhage presented with headache and impaired consciousness. CT revealed a right thalamic hemorrhage with intraventricular extension and acute hydrocephalus. Emergency ventricular drainage was performed. Subsequent angiography identified a ruptured peripheral aneurysm arising from the artery of Percheron originating from the left P1 segment, along with right-dominant moyamoya vascular architecture. The aneurysm was successfully treated with N-butyl cyanoacrylate embolization to prevent rebleeding. Due to insufficient clearance of intraventricular hematoma, endoscopic evacuation was performed. The patient experienced no rebleeding, and follow-up angiography confirmed durable obliteration of the aneurysm. Peripheral aneurysms arising from the artery of Percheron in moyamoya disease are extremely rare but should be considered in cases of deep hemorrhage with intraventricular extension. Prompt endovascular embolization using liquid embolic agents, combined with endoscopic hematoma evacuation, may provide an effective and minimally invasive treatment strategy. https://thejns.org/doi/10.3171/CASE26356.

Primary intradural intramedullary melanocytomas of the spinal cord are exceedingly rare neoplasms. Their optimal treatment remains ill-defined because their imaging characteristics, molecular profile, and prognosis are poorly characterized. The authors first present the case of a 71-year-old male with low back pain, lower extremity weakness, sensory loss, and urinary incontinence who was found to have an enhancing conus mass requiring T11-L1 laminectomy and subtotal tumor resection. The tumor was positive for BAP1 inactivation and GNAQ mutations. The patient demonstrated partial neurological improvement, with residual motor deficits, and was subsequently counseled regarding radiation therapy. Then the authors present the case of a 65-year-old male with back pain, gait instability, and chronic progressive numbness of the torso and lower extremities who was found to have an intradural intramedullary mass for which T4-9 laminectomy and T5-8 laminoplasty with gross-total resection were performed. After recurrence, a revision T4-9 laminectomy was performed with incomplete tumor resection, and the patient underwent adjuvant radiotherapy. These cases demonstrate that molecular characterization, such as BAP1 inactivation or GNAQ mutation, offers valuable prognostic insights and may inform the need for adjuvant therapies including chemotherapy, radiotherapy, or close surveillance. https://thejns.org/doi/10.3171/CASE25946.

Purely cystic meningiomas are extremely rare extra-axial neoplasms that can mimic aggressive malignancies, like glioblastoma multiforme (GBM), because radiologically and intraoperatively they lack a visible solid component and demonstrate postcontrast enhancement and significant vasogenic edema. The authors present a case of a purely cystic intra-axial meningioma mimicking GBM with an accompanying systematic review of the literature. An 84-year-old female presented with expressive dysphasia. MRI revealed a 3-cm inhomogeneously enhancing intra-axial left temporal lobe mass with apparent MRI presentation of central necrosis, suggesting a high-grade glioma. Intraoperatively, the lesion appeared vascular and infiltrative like a GBM; however, histopathological and immunohistochemical analyses (somatostatin receptor 2 antigen positive, progesterone receptor positive, glial fibrillary acidic protein negative) confirmed an angiomatous meningioma (WHO grade 1). Postoperatively, the patient's symptoms resolved, and 10 years of annual follow-up MRI studies confirmed no recurrence. Only 3 other purely cystic meningioma cases (extra-axial) were identified in the literature. Cystic meningiomas are rarely GBM "imitators." Surgeons should consider cystic meningioma when classic radiological hallmarks are absent. Despite an aggressive imaging profile, these tumors are biologically and clinically benign. Gross-total resection remains the gold-standard treatment, offering excellent long-term prognosis and the potential for a permanent cure. This case highlights the need for histological tissue diagnosis before final treatment plans are considered. https://thejns.org/doi/10.3171/CASE26438.

Intracranial pressure monitors (IPMs) are commonly placed in patients with severe traumatic brain injury to diagnose and guide treatment for intracranial hypertension. Complications of IPM placement include infection, hemorrhage, and malposition. While rare case reports describe ruptured iatrogenic pseudoaneurysms associated with IPM, the incidence and implications of unruptured pseudoaneurysms are poorly understood. A 19-year-old female sustained a severe traumatic brain injury, leading to IPM placement. After a prolonged hospital course, delayed noninvasive imaging to characterize potential cerebrovascular injury raised concern for a carotid-cavernous fistula. Digital subtraction angiography (DSA) ruled out this pathology but revealed an unruptured distal right anterior cerebral artery pseudoaneurysm at the site of the previously placed IPM, which was later successfully treated with endovascular embolization after short-interval repeat DSA. While pseudoaneurysm formation is a rarely described complication of ICM placement, they are associated with a high rate of rupture (60%) and, subsequently, high mortality (20%). Thus, a high index of suspicion of potential pseudoaneurysm rupture should be maintained in the setting of a delayed rapid decline in neurological examination or delayed intraparenchymal hemorrhage after IPM placement. Urgent evaluation using CT angiography and/or catheter angiography should be considered in addition to standard noncontrast imaging. https://thejns.org/doi/10.3171/CASE251022.

Cerebral hyperperfusion after reconstructive surgery can develop intracerebral hemorrhage. However, there is no previous report of cerebellar hyperperfusion after surgical treatment for dural arteriovenous fistula (DAVF). In this report, the authors describe a case of cerebellar hyperperfusion following endovascular obliteration of DAVF. A man in his 70s had a progressing cognitive disturbance. Preoperative angiography showed a high-grade DAVF at the right sigmoid sinus with occlusion of the ipsilateral internal jugular vein. Since the superior sagittal sinus (SSS) was also occluded and the left transverse sinus (TS) exhibited severe stenosis, the bilateral cerebellum exhibited severe venous congestion. Preoperative brain perfusion SPECT showed severely reduced cerebrovascular reactivity to acetazolamide in the bilateral cerebellum. Transvenous transluminal angioplasty of the left TS and SSS and coil embolization of the right sigmoid sinus were performed. While the patient did not develop new neurological deficits, postoperative brain perfusion SPECT showed increased cerebral blood flow in the bilateral cerebellum up to 210% compared to that before the endovascular treatment. Blood pressure was strictly controlled, and then cerebellar hyperperfusion recovered on postoperative day 7. Cerebellar hyperperfusion can develop after surgical treatment for DAVF. Therefore, careful evaluation of preoperative hemodynamics, even in the posterior fossa, may be important. https://thejns.org/doi/10.3171/CASE26342.

Distraction fractures are unstable spinal injuries characterized by a horizontal fracture line through the vertebra, often occurring following deceleration mechanisms. Surgical management typically involves fixation and fusion to restore sagittal alignment. Minimally invasive approaches, including percutaneous pedicle screw fixation, have demonstrated reduced operative morbidity while achieving deformity correction comparable to that of open techniques. However, accurate rod contouring in percutaneous procedures remains technically challenging. A 61-year-old male presented with a three-column unstable T7 distraction fracture in the setting of diffuse idiopathic skeletal hyperostosis following a rear-end motor vehicle accident at low to moderate speeds. A T4-10 percutaneous pedicle screw fixation for fracture reduction and stabilization was performed. Preoperative CT images were formatted and printed at a 1:1 scale on a sheet of paper; cut along the fracture line, taped together to reduce the fracture, and photocopied; sterilely bagged; and used intraoperatively to guide rod bending prior to placement. Using a 1:1 ratio paper printout of the patient's preoperative CT scan as an anatomical template, the authors appropriately performed intraoperative rod bending without additional adjustment of rod contour. This method offers a practical, cost-effective solution to improve the precision and efficiency of rod bending in percutaneous spinal fusion. https://thejns.org/doi/10.3171/CASE26257.

While osteochondromas are the most common benign bone tumors, spinal involvement remains uncommon. In patients with hereditary multiple exostosis (HME), spinal osteochondromas predominantly affect posterior elements. Anterior vertebral body involvement is unusual, and anterior C3 osteochondroma with parapharyngeal extension in an adult has not been previously reported in the literature. Management of complex anterior cervical tumors near critical neurovascular structures presents significant surgical challenges. The authors present the first documented case of anterior C3 vertebral body osteochondroma with parapharyngeal space extension in a 20-year-old patient with HME presenting with progressive dysphagia. Preoperative imaging and advanced surgical planning utilizing patient-specific 3D-printed models and immersive virtual reality (VR) enabled detailed visualization of tumor anatomy. Intraoperative fluoroscopic volumetric navigation was used to guide precise osteotomy cuts in proximity to the vertebral artery. Complete tumor resection was achieved without neurological or vascular complications and without breach of the upper aerodigestive tract, with complete resolution of dysphagia and no recurrence at the 3-month follow-up. Advanced preoperative planning using 3D printing and VR provide significant value for rare and anatomically complex spinal pathology, especially when multiple surgical services are involved. The addition of intraoperative volumetric navigation enabled safe resection of the tumor adjacent to critical neurovascular structures. https://thejns.org/doi/10.3171/CASE26134.

Intracranial epidermoid cysts are rare congenital lesions accounting for approximately 1% of intracranial tumors. Petrous apex involvement is uncommon and surgically challenging because of the lesion's proximity to critical neurovascular structures. Reports describing minimally invasive endoscope-assisted approaches in this location remain limited. A 32-year-old man presented with progressive right-sided otalgia, pulsatile tinnitus, and hearing loss. Imaging demonstrated a destructive petrous temporal bone lesion with atypical diffusion characteristics, initially suspected to represent a jugular foramen tumor. The patient underwent a minimally invasive endoscope-assisted presigmoid burr hole approach. Intraoperatively, the lesion demonstrated the characteristic pearly appearance of an epidermoid cyst and was resected piecemeal under microscopic and endoscopic visualization. Endoscopic assistance improved visualization of deep and angled extensions beyond the microscopic field. Postoperatively, the patient had preserved facial nerve function, stable hearing, and an uneventful recovery. Histopathological examination confirmed an epidermoid cyst. Petrous apex epidermoid cysts require balancing maximal safe resection with preservation of neurological function. This case highlights the potential utility of a minimally invasive endoscope-assisted approach for improving visualization within constrained skull base corridors while minimizing operative morbidity. Long-term radiological surveillance remains important when complete capsule removal is not safely feasible. https://thejns.org/doi/10.3171/CASE26361.

Elective endovascular neurosurgical procedures are increasingly performed in ambulatory neurosurgery centers, enabled by advances in catheter technology, safety of conscious sedation, and refined patient selection. Although complication rates are low, rapid evaluation of postprocedural neurological deficits remains critical. Conventional MRI is often impractical in outpatient or procedural settings, whereas ultra-low-field portable MRI (ULF-pMRI) systems (such as Swoop) allow bedside imaging with favorable diagnostic performance. Two women in their 60s developed acute neurological deficits at an ambulatory neurosurgery center (ANSC) after diagnostic cerebral angiography in one case and elective internal carotid artery flow diversion in the other. In both cases, repeat angiography showed patent vessels. Bedside ULF-pMRI with diffusion-weighted and fluid-attenuated inversion recovery sequences demonstrated no acute infarction or hemorrhage. Symptoms fully resolved with supportive management. The availability of rapid portable MRI helped both patients avoid hospital admission, allowing same-day discharge without recurrent neurological symptoms. In these 2 cases, ULF-pMRI demonstrated timely, informative neuroimaging in an ANSC setting. When paired with clinical improvement, bedside MRI safely guided disposition decisions, helped avoid unnecessary transfers, and supported patient safety as outpatient neurointervention expands. https://thejns.org/doi/10.3171/CASE26245.

Epidural masses in patients with metastatic spinal cord compression are generally interpreted as direct tumor extension into the spinal canal. However, not all such lesions necessarily represent fixed tumor tissue. Regression of a ventral epidural mass after stabilization alone in cervical spinal metastasis is rarely described. An 82-year-old man presented with tetraparesis after a fall, preceded by weeks of gait unsteadiness and repeated falls. Imaging revealed a destructive C7 lesion with a ventral epidural mass causing severe spinal cord compression. Systemic evaluation suggested metastatic prostate cancer, with prostatic enlargement, a rib lesion suspicious for metastasis, and a serum prostate-specific antigen level greater than 10,000 ng/mL. Because of mechanical instability and cord compression, the patient underwent C4-6 laminectomy and posterior instrumented fixation from C4 to T2. Histopathological examination of tissue obtained from the right C7 pedicle during surgery confirmed metastatic prostate adenocarcinoma. MRI obtained 1 month after surgery demonstrated complete spontaneous resolution of the ventral epidural mass despite the absence of prior systemic therapy. Not all epidural compressive lesions associated with cervical spinal metastasis necessarily represent progressive tumor extension alone. In patients with marked instability, a component of the lesion may be mechanically reversible and regress after stabilization. https://thejns.org/doi/10.3171/CASE26340.

Neurofibromatosis type 1 (NF1) is a genetic disorder occasionally associated with vascular abnormalities, including arterial fragility and spontaneous rupture. Cervical hemorrhage related to NF1 is rare, yet potentially life-threatening, and its management presents significant challenges. Here, the authors report a rare case of NF1 complicated by recurrent spontaneous cervical hemorrhage requiring repeated endovascular embolization. A 67-year-old man with NF1 presented with acute cervical swelling without a history of trauma. CT revealed a large cervical hematoma causing tracheal deviation, and CT angiography showed active extravasation from a branch of the thyrocervical artery. Emergency endovascular embolization using n-butyl-2-cyanoacrylate achieved hemostasis. The following day, recurrent hemorrhage developed from branches of the transverse cervical and internal thoracic arteries, necessitating repeat endovascular embolization. After the second intervention, no further bleeding occurred, and the patient recovered with supportive care. In patients with NF1, cervical hemorrhage may recur early from a vascular territory distinct from the initially treated lesion. Even after a technically successful embolization, close early surveillance is required. https://thejns.org/doi/10.3171/CASE26354.

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated condition associated with a range of neurological sequelae, including dystonia. Deep brain stimulation (DBS) has been established as an effective therapy for various forms of primary and secondary dystonia; however, its use in dystonia secondary to ADEM remains scarcely reported. Here, the authors present a case of dystonia following an episode of ADEM in childhood unresponsive to bilateral DBS of the globus pallidus internus (GPi). The authors report the case of a 12-year-old patient presenting with medication-refractory generalized dystonia secondary to infantile ADEM. Following onset of ADEM at 22 months of age, the patient began exhibiting motor skill regression and progressive generalized dystonia. The dystonia remained refractory to numerous medications, including anticonvulsant, dopaminergic, anticholinergic, and muscle relaxant agents. The decision was made to perform bilateral GPi DBS. The Burke-Fahn-Marsden Dystonia Rating Scale-Motor was used to assess treatment outcomes before and after surgery. At 12 months postoperation, no significant improvements or adverse effects were observed. Age of onset and duration of dystonic symptoms must be considered when evaluating pediatric patients for DBS treatment of dystonia secondary to ADEM. https://thejns.org/doi/10.3171/CASE25979.