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Open access

Christine Marlow, Joshua A. Cuoco, Kristine Ravina, Cole A. Sloboda, and John J. Entwistle

BACKGROUND

Pure arterial malformations are characterized as unique cerebrovascular lesions with a dilated, coil-like appearance and tortuous arteries without early venous drainage. Historically, these lesions have been described as incidental findings with a benign natural history. However, pure arterial malformations can rarely demonstrate radiographic progression and develop associated focal aneurysms with an unclear risk of rupture. Whether radiographic progression of these lesions or the presence of an associated aneurysm warrants treatment remains controversial.

OBSERVATIONS

A 58-year-old male presented with sudden-onset left hemiparesis. Computed tomography revealed a large, acute, right frontotemporoparietal intraparenchymal hemorrhage with underlying irregular curvilinear calcifications. Diagnostic cerebral angiography revealed a dysplastic right middle cerebral artery dissecting aneurysm along the M2 segment associated with a pure arterial malformation, which was treated with endovascular flow diversion in a delayed fashion.

LESSONS

Pure arterial malformations with associated focal aneurysms may not exhibit a benign natural history as once thought. Intervention should be considered for ruptured pure arterial malformations to mitigate the risk of rerupture. Asymptomatic patients with a pure arterial malformation with an associated aneurysm should at least be followed closely with interval radiographic imaging to evaluate for malformation progression or changes in aneurysmal morphology.

Open access

Lisa B. E. Shields, Vasudeva G. Iyer, Yi Ping Zhang, and Christopher B. Shields

BACKGROUND

Differentiating foot drop due to upper motor neuron (UMN) lesions from that due to lower motor neuron lesions is crucial to avoid unnecessary surgery or surgery at the wrong location. Electrodiagnostic (EDX) studies are useful in evaluating patients with spastic foot drop (SFD).

OBSERVATIONS

Among 16 patients with SFD, the cause was cervical myelopathy in 5 patients (31%), cerebrovascular accident in 3 (18%), hereditary spastic paraplegia in 2 (12%), multiple sclerosis in 2 (12%), chronic cerebral small vessel disease in 2 (12%), intracranial meningioma in 1 (6%), and diffuse brain injury in 1 (6%). Twelve patients (75%) had weakness of a single leg, whereas 2 others (12%) had bilateral weakness. Eleven patients (69%) had difficulty walking. The deep tendon reflexes of the legs were hyperactive in 15 patients (94%), with an extensor plantar response in 9 patients (56%). Twelve patients (75%) had normal motor and sensory conduction, 11 of whom had no denervation changes of the legs.

LESSONS

This study is intended to raise awareness among surgeons about the clinical features of SFD. EDX studies are valuable in ruling out peripheral causes of foot drop, which encourages diagnostic investigation into a UMN source for the foot drop.

Open access

Shivani Baisiwala, Myungjun Ko, Humza Zubair, Kevin Li, Andrew C. Vivas, Richard Everson, Linda Liau, Ausaf Bari, and Kunal S. Patel

BACKGROUND

Gliosarcoma is a rare and highly malignant cancer of the central nervous system with the ability to metastasize. Secondary gliosarcoma, or the evolution of a spindle cell–predominant tumor after the diagnosis of a World Health Organization grade IV glioblastoma, has also been shown to metastasize. There is little information on metastatic secondary gliosarcoma.

OBSERVATIONS

The authors present a series of 7 patients with previously diagnosed glioblastoma presenting with recurrent tumor and associated metastases with repeat tissue diagnosis consistent with gliosarcoma. The authors describe the clinical, imaging, and pathological characteristics in addition to carrying out a systematic review on metastases in secondary gliosarcoma.

LESSONS

The present institutional series and the systematic review of the literature show that metastatic secondary gliosarcoma is a highly aggressive disease with a poor prognosis.

Open access

Juan Silvestre G. Pascual, Madeleine de Lotbiniere-Bassett, Katrina Hannah D. Ignacio, David Ben-Israel, Jessica M. Clark, and Yves P. Starreveld

BACKGROUND

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare headache disorder that has been associated with pituitary adenomas. Resection has been posited to be curative.

OBSERVATIONS

A 60-year-old female presented with a 10-year history of SUNCT, which had been medically refractory. Sellar magnetic resonance imaging (MRI) showed a 2 × 2 mm nodule in the right anterolateral aspect of the pituitary. Endoscopic endonasal transsphenoidal resection of the pituitary microadenoma with neuronavigation was performed. The patient felt immediate relief from the headaches. Postoperative MRI showed persistence of the pituitary microadenoma and the resection tract to be inferomedial to the lesion. The right middle and partial superior turbinectomy site was close to the sphenopalatine foramen (SPF). The patient was discharged on postoperative day 1 and remained headache-free without any medications at the 4-month follow-up.

LESSONS

Resection of pituitary lesions associated with SUNCT may not necessarily be the cause of SUNCT resolution. Manipulation of the middle and superior turbinate close to the SPF may lead to a pterygopalatine ganglion block. This may be the mechanism of cure for SUNCT in patients with related pituitary lesions who undergo endonasal resection.

Open access

Alexander J. Schupper, Alex Devarajan, Dong-Seok Lee, Enrique Perez, and Raj K. Shrivastava

BACKGROUND

The complexity of posterior fossa surgery can often lead to rare complications due to the anatomy involved. Vestibular schwannoma resection is a common pathology in the posterior fossa, often requiring surgical intervention. Given the proximity of this space to the brainstem, cranial nerve VII/VIII complex, and posterior inferior cerebellar artery (PICA), neurovascular complications are not infrequent. A rare vascular complication from this surgical approach is a lateral medullary infarction from injury to the lateral medullary segment of the proximal PICA, leading to central hypoventilation syndrome (CHS).

OBSERVATIONS

This report presents a unique case of a 51-year-old man who underwent a retrosigmoid craniectomy for resection of a vestibular schwannoma. Following surgery, the patient was unable to be weaned off the ventilator and was noted to become apneic while he slept, a clinical picture consistent with Ondine’s curse.

LESSONS

This report discusses the anatomical considerations of this surgical corridor leading to this complication and the management of a patient with acquired Ondine’s curse and reviews the scarce literature on this uncommon cause of acquired CHS.

Open access

Tomohiro Okuyama, Kota Kurisu, Masaaki Hokari, Kei Miyata, Kazuki Uchida, Katsuyuki Asaoka, Koji Itamoto, and Miki Fujimura

BACKGROUND

An intratumoral aneurysm encased within the associated intracranial tumor is rare, and hemorrhage caused by its rupture is even more rare. While urgent and adequate surgical treatment is important, the treatment can be difficult given the limited understanding of this rare condition.

OBSERVATIONS

A 69-year-old man who had undergone meningioma surgery 30 years prior presented with a disturbance in consciousness. Magnetic resonance imaging revealed massive intracerebral and subarachnoid hemorrhage. A round, partially calcified mass, which was diagnosed as recurrent meningioma, was also observed. Subsequent cerebral angiography revealed that the source of the hemorrhage was an intratumoral aneurysm in the dorsal internal carotid artery (ICA) encased within the recurrent meningioma. Urgent surgical ICA trapping and high-flow graft bypass were conducted. The postoperative course was uneventful, and he was referred to another hospital for rehabilitation.

LESSONS

This is the first case report of a ruptured intratumoral aneurysm being treated with urgent combined revascularization and parent artery trapping surgery. This surgical approach may be a feasible treatment option for such a challenging condition. Additionally, this case highlights the importance of diligent long-term follow-up after skull-base surgery, as minor intraoperative vascular wall injury may trigger the development and rupture of an intracerebral aneurysm.

Open access

Mao Vásquez, Luis J. Saavedra, Hector H. García, Evelyn Vela, Jorge E. Medina, Miguel Lozano, Carlos Hoyos, and William W. Lines-Aguilar

BACKGROUND

Trigeminal neuralgia (TN) is a frequent neurosurgical problem negatively influencing the quality of life of patients. The standard surgical treatment is microvascular decompression for primary cases and decompression of the mass effect, mainly tumors, for secondary cases. Neurocysticercosis (NCC) in the cerebellopontine angle is a rare etiology of TN. The authors report a case in which NCC cysts around the trigeminal nerve coexisted with a vascular loop, which compressed the exit of the trigeminal nerve from the pons.

OBSERVATIONS

A 78-year-old woman presented with a 3-year history of persistent severe pain in the left side of her face, refractory to medical treatment. On gadolinium-enhanced magnetic resonance imaging, cystic lesions were observed around the left trigeminal nerve and a vascular loop was also present and in contact with the nerve. A retrosigmoid approach for cyst excision plus microvascular decompression of the trigeminal nerve was successfully performed. There were no complications. The patient was discharged without facial pain.

LESSONS

Albeit rare, TN secondary to NCC cysts should be considered in the differential diagnosis in NCC-endemic regions. In this case, the cause of the neuralgia was probably both problems, because when both were treated, the patient improved.

Open access

Kevin K. Kumar, Angus Toland, Nancy Fischbein, Martha Morrell, Jeremy J. Heit, Donald E. Born, and Gary K. Steinberg

BACKGROUND

Schizencephaly is an uncommon central nervous system malformation. Intracranial lipomas are also rare, accounting for approximately 0.1% of brain “tumors.” They are believed to be derived from a persistent meninx primitiva, a neural crest–derived mesenchyme that develops into the dura and leptomeninges.

OBSERVATIONS

The authors present a case of heterotopic adipose tissue and a nonshunting arterial vascular malformation arising within a schizencephalic cleft in a 22-year-old male. Imaging showed right frontal gray matter abnormality and an associated suspected arteriovenous malformation with evidence of hemorrhage. Brain magnetic resonance imaging revealed right frontal polymicrogyria lining an open-lip schizencephaly, periventricular heterotopic gray matter, fat within the schizencephalic cleft, and gradient echo hypointensity concerning for prior hemorrhage. Histological assessment demonstrated mature adipose tissue with large-bore, thick-walled, irregular arteries. Mural calcifications and subendothelial cushions suggesting nonlaminar blood flow were observed. There were no arterialized veins or direct transitions from the arteries to veins. Hemosiderin deposition was scant, and hemorrhage was not present. The final diagnosis was consistent with ectopic mature adipose tissue and arteries with meningocerebral cicatrix.

LESSONS

This example of a complex maldevelopment of derivatives of the meninx primitiva in association with cortical maldevelopment highlights the unique challenges from both a radiological and histological perspective during diagnostic workup.

Open access

Tian-Min Lai, Kun-Xin Lin, Ying Fu, Ling Fang, and Wen-Long Zhao

BACKGROUND

Mechanical thrombectomy (MT) has been proved to be a highly effective therapy to treat acute ischemic stroke due to large vessel occlusion. Often, the ischemic core extent on baseline imaging is an important determinant for endovascular treatment eligibility. However, computed tomography (CT) perfusion (CTP) or diffusion-weighted imaging may overestimate the infarct core on admission and, consequently, smaller infarct lesions called “ghost infarct cores.”

OBSERVATIONS

A 4-year-old, previously healthy boy presented with acute-onset, right-sided weakness and aphasia. Fourteen hours after the onset of symptoms, the patient presented with a National Institutes of Health Stroke Scale (NIHSS) score of 22, and magnetic resonance angiography demonstrated a left middle cerebral artery occlusion. MT was not considered because of a large infarct core (infarct core volume: 52 mL; mismatch ratio 1.6 on CTP). However, multiphase CT angiography indicated good collateral circulation, which encouraged MT. Complete recanalization was achieved via MT at 16 hours after the onset of symptoms. The child’s hemiparesis improved. Follow-up magnetic resonance imaging was nearly normal and showed that the baseline infarct lesion was reversible, in agreement with neurological improvement (NIHSS score 1).

LESSONS

The selection of pediatric stroke with a delayed time window guided by good collateral circulation at baseline seems safe and efficacious, which suggests a promising value of vascular window.

Open access

Davaine Joel Ndongo Sonfack, Bilal Tarabay, Jesse Shen, Zhi Wang, Ghassan Boubez, Daniel Shédid, and Sung-Joo Yuh

BACKGROUND

Pneumorrhachis and pneumocephalus are rare conditions in which air is found within the spinal canal and brain, respectively. It is mostly asymptomatic and can be located in the intradural or extradural space. Intradural pneumorrhachis should prompt clinicians to search and treat any underlying injury of the skull, chest, or spinal column.

OBSERVATIONS

A 68-year-old man presented with a history of cardiopulmonary arrest together with pneumorrhachis and pneumocephalus following a recurrent pneumothorax. The patient reported acute headaches with no other neurological symptoms. He was managed conservatively with bed rest for 48 hours following thoracoscopic talcage of his pneumothorax. Follow-up imaging showed regression of the pneumorrhachis, and the patient reported no other neurological symptoms.

LESSONS

Pneumorrhachis is an incidental radiological finding that self-resolves with conservative management. However, it can be a complication resulting from a serious injury. Therefore, close monitoring of neurological symptoms and complete investigations should be performed in patients with pneumorrhachis.