Journal of neurosurgery. Case lessons最新文献

Background: Maximal safe resection of ependymoma should be attempted whenever feasible. To clarify the potential and limits of surgery in recurrent pediatric ependymoma, the authors report a posterior fossa anaplastic ependymoma in a boy who ultimately underwent 18 resections.

Observations: After initial multimodal therapy, resection with no detectable contrast-enhancing tumor on imaging and/or no visually apparent residual tumor intraoperatively was achieved at each operation from the 5th through the 16th, yielding 8 years 8 months of surgery-only local control. At the 17th surgery, a small remnant invading the right dorsolateral medulla was unavoidable; 7 months later, an 18th procedure achieved only partial resection and was followed by reirradiation. Recurrences arose from varying ependymal sites along the floor of the fourth ventricle rather than a single fixed locus, and a normal-appearing ependymal lining re-formed after sequential resections. Across all 18 operations, no infectious complications occurred. The tumor later progressed to circumferential brainstem involvement, and the patient died of respiratory failure 16 years 8 months after initial therapy.

Lessons: Repeated resections achieving no radiographic and intraoperative residual tumor may contribute to long-term survival in ependymoma; however, durable survival appears to depend on the persistence of locally resectable relapse. https://thejns.org/doi/10.3171/CASE25834.

Background: Cerebral venous sinus thrombosis (CVST) can cause vision loss from intracranial hypertension. Standard anticoagulation may be inadequate in severe cases. The EKOS Endovascular System enables ultrasound-assisted thrombolysis and may provide a targeted therapeutic alternative.

Observations: Three patients with CVST and severe visual dysfunction underwent EKOS after conventional therapy failed. Two patients, aged 32 and 42 years, treated within days of symptom onset, demonstrated marked visual recovery and resolution of papilledema following 24-hour thrombolysis. A third patient, aged 20 years, presented late and achieved papilledema resolution but limited visual recovery, emphasizing the importance of early treatment. All procedures were technically successful, well tolerated, and complication free.

Lessons: EKOS thrombolysis may represent a safe, effective option for CVST with vision-threatening intracranial hypertension, particularly after anticoagulation failure. Early intervention appears critical to optimize outcomes. Larger studies are warranted to confirm the safety, efficacy, and optimal treatment window. https://thejns.org/doi/10.3171/CASE25364.

Background: Responsive neurostimulation (RNS) enables real-time electrographic monitoring and chronic assessment of seizure burden in two distinct brain regions. Laser interstitial thermal therapy (LITT) offers a minimally invasive treatment for mesial temporal epilepsy. Unilateral LITT that maintains functional RNS contralaterally poses significant challenges. As RNS use becomes more widespread, adjunctive seizure reduction therapies following implantation may become increasingly common. The authors present 2 cases illustrating a minimally invasive surgical approach.

Observations: Two patients with bitemporal RNS underwent long-term monitoring that revealed a significant unilateral predominance in seizure onset. LITT targeting the more active mesial temporal lobe while maintaining a functioning RNS contralaterally was recommended. Mesial temporal laser ablations were performed that accounted for existing RNS hardware and manufacturer-imposed MRI safety limitations. Both patients retained fully functional RNS systems postoperatively and experienced no complications. The laser ablations were technically successful with encouraging early seizure freedom outcomes. Long-term monitoring will determine sustained efficacy.

Lessons: LITT was safely performed in the prone position in 2 patients with preexisting RNS systems. Caution is required to avoid hardware damage or electrode overheating during MRI. While further study is needed to assess outcomes, this technique offers a promising option for patients requiring targeted ablation following extended RNS monitoring. https://thejns.org/doi/10.3171/CASE25434.

Background: Lymphocytic vasculitis (LV), a histopathological subtype of primary angiitis of the CNS, is a rare inflammatory disorder that often presents as mass-like lesions mimicking malignant brain tumors. Owing to its rarity, the characteristics of LV remain poorly understood.

Observations: The authors report the case of a young woman who presented with progressive headache. MRI revealed a solitary, ring-enhancing lesion with marked perifocal edema. Fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated relatively low uptake at the lesion margin, whereas fluciclovine-PET showed mild uptake. Given the high intracranial pressure and uncertain diagnosis, gross-total resection was performed. Histopathological analysis confirmed LV, characterized by perivascular lymphocytic infiltration, fibrin exudation, and the absence of neoplastic or demyelinating features. The patient did not receive additional immunosuppressive therapy and remained disease free for 12 months.

Lessons: To the best of the authors' knowledge, this is the first reported case of a solitary intracranial LV that was successfully managed with resection alone. This case suggests that resection may be a possible therapeutic option for select patients, particularly those with solitary lesions and negative autoimmune profiles. FDG-PET and fluciclovine-PET with MRI may provide supportive information for distinguishing LV from malignant neoplastic tumors. https://thejns.org/doi/10.3171/CASE25705.

Background: Diffuse midline glioma (DMG), a highly malignant CNS tumor, is classified as a World Health Organization grade 4 tumor. Its overall survival prognosis, even after standard treatment, is typically extremely poor. Photodynamic therapy (PDT) represents an innovative treatment modality with promising results for intracranial malignant gliomas. However, its use for spinal malignant gliomas has not yet been explored, despite their pathological similarity to intracranial malignant gliomas.

Observations: The present case of a 28-year-old female patient with recurrent spinal DMG represents the first documented report concerning the effects of PDT on spinal malignant gliomas, with the pathological postmortem findings. Despite the successful application of PDT to the resection cavity of the spinal DMG, cervical spinal cord edema developed soon after surgery. The blood flow to the spinal cord parenchyma was thought to be altered but reversible. The patient's overall survival was 21 months after the initial surgery.

Lessons: This case highlights the potential of PDT as a feasible and promising adjunctive treatment for spinal malignant gliomas. The observed clinical course and pathological findings may provide valuable insights for future research and therapeutic development. https://thejns.org/doi/10.3171/CASE25751.

Background: Lymphomas originating from the dura mater of the skull, known as primary dural lymphomas (PDLs), are rare. Among these, cases of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) are exceptionally uncommon, and no standardized treatment protocols have been established. The authors report a rare case of CLL/SLL manifesting as PDL mimicking a skull base meningioma and review the relevant literature.

Observations: A 71-year-old male presented with an extra-axial tumor extending from the petroclival to middle cranial fossa, which demonstrated progressive enlargement over the course of 1 year. The preoperative diagnosis was meningioma, and the patient subsequently underwent preoperative embolization followed by partial tumor resection. Histological examination revealed CLL/SLL. Postoperatively, the residual lesion was treated with radiation therapy, and no recurrence has been observed for > 3 years.

Lessons: CLL/SLL arising as PDL is difficult to distinguish from meningioma based on clinical and radiological findings. However, it generally shows favorable outcomes when treated with a combination of surgery and low-dose radiation therapy. https://thejns.org/doi/10.3171/CASE25831.

Background: Collision tumors, defined as the coexistence of two distinct neoplasms with independent origins in the same anatomical location, are rare entities that pose diagnostic and therapeutic challenges. While pituitary adenomas and meningiomas are individually common intracranial tumors, their coexistence as collision tumors is exceptionally uncommon, with only a handful of cases reported in the literature. Even fewer involve growth hormone-secreting pituitary adenomas, and none have involved sphenoid wing meningiomas to date.

Observations: The authors present the case of a 54-year-old female who presented with progressive expressive aphasia, right-sided weakness, gait imbalance, and memory decline. Initial MRI demonstrated a large dural-based mass involving the left sphenoid wing and sellar region; the tumor was resected in staged procedures and diagnosed as a meningothelial meningioma. Fourteen months later, follow-up imaging revealed a distinct sellar lesion. Endoscopic endonasal resection demonstrated a pituitary adenoma extensively involved by the meningioma, establishing the diagnosis of a collision tumor.

Lessons: The authors highlight the importance of recognizing the potential for dual pathology in complex intracranial tumors. This case describes the limitations of preoperative imaging in diagnosing collision tumors. Careful long-term surveillance and multidisciplinary management are essential for accurate diagnosis and optimal treatment of collision tumors. https://thejns.org/doi/10.3171/CASE25709.

Background: Spinal hypertrophic pachymeningitis is a rare manifestation of immunoglobulin G4 (IgG4)-related disease. This condition presents significant diagnostic and therapeutic challenges, and the literature on its surgical management remains limited.

Observations: A 55-year-old woman with fibromyalgia and a 20-pack-year smoking history presented with 3 months of worsening back pain, lower extremity weakness, and numbness, corresponding to a modified Japanese Orthopaedic Association (mJOA) score of 10 at presentation. MRI revealed a mass-like epidural process spanning T1-5 with cord compression. Decompression was achieved with T1-5 laminectomies, during which dura mater thickening was observed and subsequently debulked. Pathology revealed findings consistent with possible IgG4 pachymeningitis. While the patient initially regained full strength in her lower extremities with surgery and medical management, she required a second surgery due to recurrent symptoms and renewed compression and dural thickening. She underwent a second dural debulking and decompression with duraplasty, which alleviated her recurrent symptoms. She remained free of recurrent symptoms at her 1-year postoperative follow-up, with an mJOA score of 17.

Lessons: This case demonstrates the effective use of a multidisciplinary team and recurrent surgical management, incorporating adjuncts such as intraoperative ultrasound and dural patch closure in the management of pachymeningitis causing significant cord compression, ultimately leading to lasting positive clinical improvement. https://thejns.org/doi/10.3171/CASE25313.

Background: Proximal ulnar and median nerve injuries with Sunderland grade IV or V disruptions often lead to incomplete recovery of intrinsic hand muscles despite partial sensory and extrinsic motor recovery. However, traditional nerve transfers provide inconsistent intrinsic reinnervation. Persistent intrinsic deficits impair fine motor skills, such as pinch and grasp strength, and may necessitate secondary interventions.

Observations: Four patients with complete, isolated proximal ulnar or median nerve injuries underwent intrapalm donor nerve transfers. Procedures included transfer of the opponens pollicis motor branch to the terminal division of the deep motor branch of the ulnar nerve and the abductor digiti quinti motor branch to the thenar branch of the median nerve. All patients showed functional improvements in pinch and grip strength postoperatively. None required secondary tendon transfers, and no donor site morbidity was observed. In 3 ulnar nerve cases, Froment's sign converted from positive to negative. In the median nerve case, visible thumb abduction was restored.

Lessons: Intrapalm donor nerve transfers show effective options for restoring intrinsic hand function and improving fine motor skills in patients with isolated proximal ulnar or median nerve injuries. https://thejns.org/doi/10.3171/CASE25744.

Background: Intradural spinal lipomas are a form of closed dysraphism and a frequent cause of tethered cord. Although usually adipocyte predominant, rare lesions may contain additional histological elements that can yield unexpected intraoperative behavior.

Observations: A 13-year-old boy presented with progressive right foot deformity, distal weakness, gait limitation, and a low conus at S1-2 with a large terminal lipoma on spine MRI. He underwent detethering with debulking and L5 osteoplastic laminoplasty under intraoperative neuromonitoring (IONM). During microsurgical dissection, a pink band along the right aspect of the lipoma appeared unlike a nerve root. Targeted stimulation produced contraction within the thecal sac. The segment was isolated and divided under IONM guidance. Histopathological analysis confirmed skeletal muscle fibers intertwined with lobulated adipose tissue and fibrovascular elements of the filum terminale, consistent with a myolipoma. The patient had no postoperative sensory changes and progressed satisfactorily.

Lessons: Electrically evoked lipoma contraction is a physiological signature suggestive of myolipoma and can refine the extent of safe untethering. Although IONM adds value beyond evoked potential mapping by eliciting myogenic contraction and helping discriminate resectable elements from neural structures, the resulting EMG tracing becomes difficult to interpret in the presence of muscle and detethering proceeds based on visual assessment. https://thejns.org/doi/10.3171/CASE25811.