Monkeypox-Associated Central Nervous System Disease: A Case Series and Review.

OBJECTIVE: Monkeypox virus (MPXV) disease has been declared a public health emergency by the World Health Organization, creating an urgent need for neurologists to be able to recognize, diagnosis, and treat MPXV-associated neurologic disease. METHODS: Three cases of MPXV-associated central nervous system (CNS) disease occurring during the 2022 outbreak, and their associated imaging findings are presented, with 2 cases previously published in a limited capacity in a public health bulletin. RESULTS: Three previously healthy immunocompetent gay men in their 30s developed a febrile illness followed by progressive neurologic symptoms with presence of a vesiculopustular rash. MPXV nucleic acid was detected by polymerase chain reaction (PCR) from skin lesions of 2 patients, with the third patient having indeterminate testing but an epidemiologic link to a confirmed MPXV disease case. Cerebrospinal fluid demonstrated a lymphocytic pleocytosis, elevated protein, and negative MPXV-specific PCR. In 2 patients, magnetic resonance imaging of the brain and spine demonstrated partially enhancing, longitudinally extensive central spinal cord lesions with multifocal subcortical, basal ganglia, thalamic, cerebellar, and/or brainstem lesions. The third patient had thalamic and basal ganglia lesions. All patients received 14 days of tecovirimat, and 2 patients also received multiple forms of immunotherapy, including intravenous immunoglobulin, pulsed high-dose steroids, plasmapheresis, and/or rituximab. Good neurologic recovery was observed in all cases. INTERPRETATION: MPXV can be associated with CNS disease. It is unclear whether this is from a parainfectious immune-mediated injury or direct CNS viral invasion. ANN NEUROL 2023;93:893-905.

Cranial Nerve Palsies in the Setting of Arachnoid Cysts: A Case Series and Literature Review.

BACKGROUND: Arachnoid cysts (ACs) are benign lesions typically believed to not cause neurologic defects in the adult population and are most often found incidentally on imaging. We describe 2 patients with ACs potentially leading to isolated cranial nerve (CN) dysfunction. METHODS: We describe 2 patients, 1 with a fourth nerve palsy and the other with a sixth nerve palsy found to have ACs on MRI brain imaging in locations that potentially caused a compressive CN palsy. We review previous literature of ACs presenting with CN III, IV, or VI palsy. RESULTS: Patient 1 was a 62-year-old man who presented with a 22-year history of diplopia with strabismus examination consistent with a congenital CN IV palsy. Despite multiple surgeries, his CN IV palsy insidiously worsened. An AC in the posterior fossa with mass effect on the quadrigeminal plate and asymmetric atrophy of the right superior oblique was identified on imaging. Patient 2 was an 80-year-old man who presented with an 18-year history of diplopia and was found to have a left esotropia and abduction deficit consistent with complete CN VI palsy. An AC in the left cavernous sinus was identified on imaging. He underwent strabismus surgery with satisfactory resolution of diplopia. We identified a total of 18 previously published cases: 8 reports of CN III palsy, 4 reports of CN IV palsy, and 6 reports of CN VI palsy. Patient ages ranged from 1 to 67 with a median of 34.5. In 16/18 (89%) cases, the diagnosis of ACs was made within 1 year of symptom onset. Surgical removal of the AC was successful in resolving diplopia in 7/12 (58%) cases. In no case was strabismus surgery performed as primary treatment. CONCLUSIONS: Although ACs are typically congenital asymptomatic lesions, we present a case series of 2 patients with ACs in anatomic locations that potentially caused chronic, progressive, isolated CN palsies leading to strabismus. Our literature review revealed that most published cases detailing this clinical scenario resulted in neurosurgical fenestration of ACs with mixed results. Our cases represent 2 patients with AC-associated CN palsy treated with strabismus surgery.

Predictors of salvage therapy for parasagittal meningiomas treated with primary surgery, radiosurgery, or surgery plus adjuvant radiotherapy.

OBJECTIVE: Parasagittal meningiomas (PM) are treated with primary microsurgery, radiosurgery (SRS), or surgery with adjuvant radiation. We investigated predictors of tumor progression requiring salvage surgery or radiation treatment. We sought to determine whether primary treatment modality, or radiologic, histologic, and clinical variables were associated with tumor progression requiring salvage treatment. METHODS: Retrospective study of 109 consecutive patients with PMs treated with primary surgery, radiation (RT), or surgery plus adjuvant RT (2000-2017) and minimum 5 years follow-up. Patient, radiologic, histologic, and treatment data were analyzed using standard statistical methods. RESULTS: Median follow up was 8.5 years. Primary treatment for PM was surgery in 76 patients, radiation in 16 patients, and surgery plus adjuvant radiation in 17 patients. Forty percent of parasagittal meningiomas in our cohort required some form of salvage treatment. On univariate analysis, brain invasion (OR: 6.93, p < 0.01), WHO grade 2/3 (OR: 4.54, p < 0.01), peritumoral edema (OR: 2.81, p = 0.01), sagittal sinus invasion (OR: 6.36, p < 0.01), sagittal sinus occlusion (OR: 4.86, p < 0.01), and non-spherical shape (OR: 3.89, p < 0.01) were significantly associated with receiving salvage treatment. On multivariate analysis, superior sagittal sinus invasion (OR: 8.22, p = 0.01) and WHO grade 2&3 (OR: 7.58, p < 0.01) were independently associated with receiving salvage treatment. There was no difference in time to salvage therapy (p = 0.11) or time to progression (p = 0.43) between patients receiving primary surgery alone, RT alone, or surgery plus adjuvant RT. Patients who had initial surgery were more likely to have peritumoral edema on preoperative imaging (p = 0.01). Median tumor volume was 19.0 cm3 in patients receiving primary surgery, 5.3 cm3 for RT, and 24.4 cm3 for surgery plus adjuvant RT (p < 0.01). CONCLUSION: Superior sagittal sinus invasion and WHO grade 2/3 are independently associated with PM progression requiring salvage therapy regardless of extent of resection or primary treatment modality. Parasagittal meningiomas have a high rate of recurrence with 80.0% of patients with WHO grade 2/3 tumors with sinus invasion requiring salvage treatment whereas only 13.6% of the WHO grade 1 tumors without sinus invasion required salvage treatment. This information is useful when counseling patients about disease management and setting expectations.

Blood pressure reduction, decreased diffusion on MRI, and outcomes after intracerebral hemorrhage.

BACKGROUND AND PURPOSE: Decreased diffusion (DD) consistent with acute ischemia may be detected on MRI after acute intracerebral hemorrhage (ICH), but its risk factors and impact on functional outcomes are not well-defined. We tested the hypotheses that DD after ICH is related to acute blood pressure (BP) reduction and lower hemoglobin and presages worse functional outcomes. METHODS: Patients who underwent MRI were prospectively evaluated for DD by certified neuroradiologists blinded to outcomes. Hemoglobin and BP data were obtained via electronic queries. Outcomes were obtained at 14 days and 3 months with the modified Rankin Scale, a functional scale scored from 0 (no symptoms) to 6 (dead). We used logistic regression for dependence or death (modified Rankin Scale score 4-6). RESULTS: DD distinct from the hematoma was found on MRI in 39 of 95 patients (41%). DD was associated with greater BP reductions from baseline and a higher risk of dependence or death at 3 months (odds ratio, 4.8; 95% confidence interval, 1.7-13.9; P=0.004) after correction for ICH score (1.8 per point; 95% confidence interval, 1.2-3.1; P=0.01). Lower hemoglobin was associated with worse ICH score, larger hematoma volume, and worse outcomes, but not DD. CONCLUSIONS: DD is common after ICH, associated with greater acute BP reductions, and associated with disability and death at 3 months in multivariate analysis. The potential benefits of acute BP reduction to reduce hematoma growth may be limited by DD. The prevention and treatment of cerebral ischemia manifested as DD are potential methods to improve outcomes.

Case Report: Anterior Spinal Cord Ischemia Following Embolization of Cerebellar Arteriovenous Malformation: An Illustrative Case and Review of Spinal Cord Vascular Anatomy.

Spinal cord ischemia (SCI) is a rare entity with high mortality and morbidity which can arise from causes such as atherosclerosis, aortic dissection or aneurysm, thromboembolic events or systemic hypotension, and is a potential complication of spinal surgery. Published literature contains very few reports of SCI as a complication of intracranial interventions, highlighting the uncommon nature of SCI in these circumstances. We report the occurrence of anterior SCI in a 69-year-old patient following successful embolization of a cerebellar arteriovenous malformation (AVM), marked by upper extremity weakness, lower extremity paraplegia, loss of bladder and bowel control, and hypercapnic respiratory failure requiring mechanical ventilation. Magnetic resonance imaging (MRI) demonstrated upper cervical diffusion restriction and T2/STIR hyperintensity. Unusually, SCI occurred in this case without intraprocedural catheter wedging or obvious flow limitation, prolonged procedure time, hypercoagulable state, or general hypotension. We review previous cases in the literature as well as spinal cord vascular anatomy, and discuss the possible etiologies of this complication. Spinal cord ischemia could be a very rare complication of neuroendovascular procedures even in the absence of warning signs and should be carefully evaluated in patients with suspected neurologic symptoms after such procedures.

Autologous non-myeloablative haemopoietic stem cell transplantation in relapsing-remitting multiple sclerosis: a phase I/II study.

BACKGROUND: Autologous non-myeloablative haemopoietic stem cell transplantation is a method to deliver intense immune suppression. We evaluated the safety and clinical outcome of autologous non-myeloablative haemopoietic stem cell transplantation in patients with relapsing-remitting multiple sclerosis (MS) who had not responded to treatment with interferon beta. METHODS: Eligible patients had relapsing-remitting MS, attended Northwestern Memorial Hospital, and despite treatment with interferon beta had had two corticosteroid-treated relapses within the previous 12 months, or one relapse and gadolinium-enhancing lesions seen on MRI and separate from the relapse. Peripheral blood haemopoietic stem cells were mobilised with 2 g per m2 cyclophosphamide and 10 microg per kg per day filgrastim. The conditioning regimen for the haemopoietic stem cells was 200 mg per kg cyclophosphamide and either 20 mg alemtuzumab or 6 mg per kg rabbit antithymocyte globulin. Primary outcomes were progression-free survival and reversal of neurological disability at 3 years post-transplantation. We also sought to investigate the safety and tolerability of autologous non-myeloablative haemopoietic stem cell transplantation. FINDINGS: Between January, 2003, and February, 2005, 21 patients were treated. Engraftment of white blood cells and platelets was on median day 9 (range day 8-11) and patients were discharged from hospital on mean day 11 (range day 8-13). One patient had diarrhoea due to Clostridium difficile and two patients had dermatomal zoster. Two of the 17 patients receiving alemtuzumab developed late immune thrombocytopenic purpura that remitted with standard therapy. 17 of 21 patients (81%) improved by at least 1 point on the Kurtzke expanded disability status scale (EDSS), and five patients (24%) relapsed but achieved remission after further immunosuppression. After a mean of 37 months (range 24-48 months), all patients were free from progression (no deterioration in EDSS score), and 16 were free of relapses. Significant improvements were noted in neurological disability, as determined by EDSS score (p<0.0001), neurological rating scale score (p=0.0001), paced auditory serial addition test (p=0.014), 25-foot walk (p<0.0001), and quality of life, as measured with the short form-36 (SF-36) questionnaire (p<0.0001). INTERPRETATION: Non-myeloablative autologous haemopoietic stem cell transplantation in patients with relapsing-remitting MS reverses neurological deficits, but these results need to be confirmed in a randomised trial.

Linkage to chromosome 2q36.1 in autosomal dominant Dandy-Walker malformation with occipital cephalocele and evidence for genetic heterogeneity.

We previously reported a Vietnamese-American family with isolated autosomal dominant occipital cephalocele. Upon further neuroimaging studies, we have recharacterized this condition as autosomal dominant Dandy-Walker with occipital cephalocele (ADDWOC). A similar ADDWOC family from Brazil was also recently described. To determine the genetic etiology of ADDWOC, we performed genome-wide linkage analysis on members of the Vietnamese-American and Brazilian pedigrees. Linkage analysis of the Vietnamese-American family identified the ADDWOC causative locus on chromosome 2q36.1 with a multipoint parametric LOD score of 3.3, while haplotype analysis refined the locus to 1.1 Mb. Sequencing of the five known genes in this locus did not identify any protein-altering mutations. However, a terminal deletion of chromosome 2 in a patient with an isolated case of Dandy-Walker malformation also encompassed the 2q36.1 chromosomal region. The Brazilian pedigree did not show linkage to this 2q36.1 region. Taken together, these results demonstrate a locus for ADDWOC on 2q36.1 and also suggest locus heterogeneity for ADDWOC.

Coil embolization of posttraumatic pseudoaneurysm of the ophthalmic artery causing subarachnoid hemorrhage. Case report.

The authors report a unique case of subarachnoid hemorrhage caused by a traumatic pseudoaneurysm of the ophthalmic artery, which was successfully treated with coil embolization. Clinical and imaging features, as well as the relevant literature, are described.

Primary treatment of an indirect carotid cavernous fistula by injection of N-butyl cyanoacrylate in the dural wall of the cavernous sinus.

BACKGROUND: Carotid cavernous fistulae are abnormal communications between the carotid artery system and the cavernous sinus. Endovascular treatment is the treatment of choice, but because of their heterogeneous etiology and anatomy, an appropriate treatment plan must be tailored for each patient. CASE DESCRIPTION: A 77-year-old diabetic woman presented with rapid onset of right eye pain, conjunctivitis, and chemosis. Angiography revealed an indirect CCF located in the posterior wall of the right cavernous sinus. N-Butyl cyanoacrylate was injected directly into the fistula site in the dural wall via a microcatheter, resulting in a cure. CONCLUSION: We describe an unconventional treatment of an indirect CCF from an IPS approach. One type D(2) CCF was treated successfully using only N-butyl cyanoacrylate injected directly at the fistula site. This was achieved by microcatheterization of the fistula in the posterior wall of the right cavernous sinus.

Extradural spinal meningioma as a source of plasmacytoid cells. A case report.

BACKGROUND: Meningiomas, tumors that often affect middle-aged and elderly people, occasionally arise in the spine, typically at the thoracic level. The cytologic findings in meningiomas include whorls and syncytial clusters of bland-looking cells with scattered, psammomatous calcifications and intranudclear cytoplasmic inclusions. However, in many cases, not all these findings are seen, and in rare cases, unusual cytomorphologic features are observed. CASE: A case of spinal meningioma was located in the extradural compartment and composed predominantly of singly scattered cells with a plasmacytoid appearance, demonstrated on fine needle aspiration biopsy smear preparations. The cell block showed more typical features of meningioma, and the diagnosis was supported by the results of immunohistochemical staining. CONCLUSION: The diagnosis of spinal meningioma is readily made by employing magnetic resonance imaging. The diagnosis can be difficult to confirm pathologically when atypical histologic findings are present, as in this case, with prominent plasmacytoid features. Sections from the cell block and immunohistochemical stains as well as clinical and radiologic findings were extremely helpful in arriving at the final diagnosis.

Radiology Education of Physician Extenders: What Role Should Radiologists Play?

As physician extenders (PEs) enter the medical community in large numbers, they have an increasing impact on imaging utilization and imaging-based procedures. Physician assistants (PAs) and nurse practitioners (NPs) have an advanced level of education and some practice autonomously. However, PA and NP programs are not required to provide any basic radiology education. For PEs who did receive basic radiology education during their graduate program, the curriculum is nonstandard and there is a wide variation. PEs working in primary care and nonradiology specialties place imaging orders, review report findings, and answer patient questions. Other PEs working within radiology practices operate as liaisons with patients in diagnostic radiology or perform an increasing number of interventional procedures. Basic radiology education in formal PE certificate programs as well as on-the-job education about radiology may benefit patients, radiologists, and the health-care system. What role, if any, should the radiologist assume for educating PE students and practicing PAs and NPs? This review analyzes the benefits and drawbacks of radiologists educating PEs.

MR angiographic evaluation of platinum coil packs at 1.5T and 3T: an in vitro assessment of artifact production: technical note.

Susceptibility artifact from platinum coil packs impairs the visibility of perianeurysmal soft tissues at conventional 3D time-of-flight MR angiography. These artifacts limit the evaluation for residual-recurrent aneurysm and parent vessel stenosis. Reducing the echo time can decrease the artifact and improve perianeurysmal visualization. The purpose of this study was to assess quantitatively the effect of decreasing the echo time on artifact production at different field strengths and coil pack densities.

Basilar artery herniation into the sphenoid sinus resulting in pontine and cerebellar infarction: demonstration by three-dimensional time-of-flight MR angiography.

We report a unique case of basilar artery herniation into the sphenoid sinus caused by a traumatic skull base fracture, with persistent patency of the basilar artery. Clinical and imaging features, as well as the relevant literature, are described.

Rheolytic catheter thrombectomy, balloon angioplasty, and direct recombinant tissue plasminogen activator thrombolysis of dural sinus thrombosis with preexisting hemorrhagic infarctions.

We describe the case of a 28-year-old obtunded woman who presented with bilateral anterior parietal lobe cortical hemorrhages associated with thrombosis of the superior sagittal sinus, both transverse and sigmoid sinuses, and multiple cortical veins draining into the sagittal sinus. Initial heparin therapy was not effective. A combination of AngioJet rheolytic catheter thrombectomy, balloon angioplasty, and continuous direct superior sagittal sinus recombinant tissue plasminogen activator infusion led to venous recanalization with a successful clinical outcome, without worsening of the preexisting intracranial hemorrhages.

Temporary balloon occlusion to test adequacy of collateral flow to the retina and tolerance for endovascular aneurysmal coiling.

Two carotid ophthalmic artery aneurysms with incorporation of the artery into the neck were referred for endovascular assessment. Temporary balloon occlusion at the aneurysm neck was performed in an attempt to assess the adequacy of collateral flow to the retina. During inflation, the patients reported visual deterioration that resolved upon deflation, which indicates that collateral blood flow was insufficient. The patients were referred for surgical clipping to ensure preservation of the ophthalmic artery.

Coiling of cerebral aneurysm remnants after clipping.

OBJECTIVE: During the past decade, options for the management of aneurysm remnants after clipping have expanded. Advances in aneurysm coiling techniques and technology have allowed for more remnants to be treated safely. We present our experience with this approach and discuss its indications, limitations, and results. METHODS: We retrospectively reviewed the Northwestern Memorial Hospital experience with aneurysm coiling between January 1996 and June 2001. We identified five patients who underwent coiling for aneurysm remnants after clipping. We reviewed the clinical history, all follow-up notes, and all relevant imaging studies. We also reviewed MEDLINE for all articles published in the English language between 1990 and September 2001 that included patients treated with this approach. RESULTS: Complete to near-complete aneurysm occlusion was achieved in all five patients in our study. There was no permanent morbidity or mortality associated with the procedure in any of these patients. In the literature, we found seven articles that discuss 21 patients who were treated with coiling for their remnants. There were no permanent complications reported for these 21 patients. Adequate long-term follow-up in these 21 patients, however, is lacking. CONCLUSION: Complete aneurysm occlusion is the goal of aneurysm clipping. When this goal cannot be achieved safely, coiling of the remnant, if size and morphology are amenable, is a safe option that should be considered. Clinical and angiographic long-term follow-up of patients treated in this manner should be studied and reported.