- Ocular involvement in neurolymphomatosis. [Journal Article]
- AJAm J Ophthalmol Case Rep 2018; 10:148-151
- CONCLUSIONS: Our patient is only the second histological demonstration of ciliary nerve involvement by NL, and the first, to our knowledge, of primary NL spreading secondarily from the ciliary nerves into the choroid. Our patient demonstrates that NL, though rare, should be included in the differential diagnosis of ocular cranial nerve palsies and ophthalmoplegia.
- Moebius Syndrome with Hypoglossal Palsy, Syndactyly, Brachydactyly, and Anisometropic Amblyopia. [Journal Article]
- CCureus 2018 Mar 16; 10(3):e2334
- Moebius syndrome is a rare cause of congenital facial and abducens palsy. It is sometimes associated with musculoskeletal abnormalities and other cranial nerve palsies. Genetics and ischemic insults ...
Moebius syndrome is a rare cause of congenital facial and abducens palsy. It is sometimes associated with musculoskeletal abnormalities and other cranial nerve palsies. Genetics and ischemic insults to the fetus are considered to be the cause of this syndrome. We report here a 12-year-old female patient who was presented to us with poor cosmesis of her face, with associated decreased vision and lagophthalmos in her left eye. She didn't have any signs of exposure keratopathy in the affected eye. Her best-corrected vision was 20/20 and 20/60 in right and left eyes respectively. The cause of decreased vision in her left eye was found to be anisometropic amblyopia, due to asymmetric hyperopic astigmatism in her eyes. She did not report diplopia in any gaze position. Examination of her cranial nerve revealed left facial, abducens, and hypoglossal nerve palsy, leading us to the diagnosis of Moebius syndrome. Apart from that, she had syndactyly in one of her hands, and brachydactyly in both. Since the eyes were straight in their primary position, no surgical intervention was carried out for her lagophthalmos, which was measured to be only 2 mm. This was to prevent any post-operative iatrogenic ptosis. The condition requires a multidisciplinary approach involving the opinions of a neuro-ophthalmologist, strabismologist, and oculoplastics for the management of the complications associated with the disease.
- Brain-stem hemangioblastomas: The seemingly innocuous lesion in a perilous location. [Journal Article]
- NINeurol India 2018 May-Jun; 66(3):779-796
- CONCLUSIONS: Significant improvement is achievable in neurological status in patients following successful extirpation of a brain-stem hemangioblastoma, despite a turbulent perioperative period. Leaving tumour capsule adherent to the brain-stem often helps in preserving brain-stem function. Postoperatively, the patients should be monitored for their respiratory and lower cranial nerve status to prevent aspiration pneumonitis.
- An Unusual Presentation of Kikuchi-Fujimoto Disease with Recurrent Subdural Effusion. [Journal Article]
- CCureus 2018 Mar 10; 10(3):e2302
- A 24-year-old man complained of a right temporal headache for four weeks. The patient denied any trauma or previous anticoagulation use. He also reported tender right facial swelling. His physical ex...
A 24-year-old man complained of a right temporal headache for four weeks. The patient denied any trauma or previous anticoagulation use. He also reported tender right facial swelling. His physical exam was unrevealing except for right cranial nerve (CN) VI palsy, right parotid enlargement, and cervical adenopathy. Laboratory findings were significant for mild leukopenia at 3300 cells/uL. The computed tomography (CT) scan obtained showed a chronic left subdural effusion with a 4 mm midline shift and confirmed right parotid enlargement and cervical lymphadenopathy. Surgical burr hole evacuation was done and the fluid was sent for wound culture analysis. The infectious diseases service recommended initiating antibiotics, which were later stopped due to cerebrospinal fluid (CSF) cultures with no growth of any organisms. His CN VI palsy resolved during admission. The patient was discharged with follow-up for biopsy. The patient was lost to follow-up. The patient presented to the emergency department (ED) three months later, with a left-sided frontal headache. A repeat CT scan showed a new, right-sided fluid collection outside the brain parenchyma. Burr hole evacuation was done again and purulent fluid was drained. Antibiotics were held this time, but anti-tuberculous therapy was initiated empirically. The otolaryngology service was consulted and a lymph node biopsy was performed. The pathology showed histiocytic necrotizing lymphadenitis. A dural biopsy was done as well and was consistent with histiocytic necrotizing lymphadenitis involving the dura. Cultures from the subdural fluid did not grow any organism. The patient remained neurologically intact. He improved after surgery was done to drain the fluid and was managed by analgesics. The cultures from the extra-parenchymal fluid collection remained negative for pathogens and tuberculous mycobacteria. The patient was discharged with rheumatology clinic follow-up. He saw the rheumatologist six weeks after the discharge. During his clinic visit, the patient reported no recurrence of headaches, fevers, rash, or joint pain. Our patient had a rare presentation of Kikuchi-Fujimoto disease, in which he had a subdural fluid collection resulting in neurological complications that required surgical intervention.
- Clinical, Radiologic, Pathologic, and Molecular Characteristics of Long-Term Survivors of Diffuse Intrinsic Pontine Glioma (DIPG): A Collaborative Report From the International and European Society for Pediatric Oncology DIPG Registries. [Journal Article]
- JCJ Clin Oncol 2018 May 10; :JCO2017759308
- Purpose Diffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborate...
Purpose Diffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborated to compare clinical, radiologic, and histomolecular characteristics between short-term survivors (STSs) and long-term survivors (LTSs). Materials and Methods Data abstracted from registry databases included patients from North America, Australia, Germany, Austria, Switzerland, the Netherlands, Italy, France, the United Kingdom, and Croatia. Results Among 1,130 pediatric and young adults with radiographically confirmed DIPG, 122 (11%) were excluded. Of the 1,008 remaining patients, 101 (10%) were LTSs (survival ≥ 2 years). Median survival time was 11 months (interquartile range, 7.5 to 16 months), and 1-, 2-, 3-, 4-, and 5-year survival rates were 42.3% (95% CI, 38.1% to 44.1%), 9.6% (95% CI, 7.8% to 11.3%), 4.3% (95% CI, 3.2% to 5.8%), 3.2% (95% CI, 2.4% to 4.6%), and 2.2% (95% CI, 1.4% to 3.4%), respectively. LTSs, compared with STSs, more commonly presented at age < 3 or > 10 years (11% v 3% and 33% v 23%, respectively; P < .001) and with longer symptom duration ( P < .001). STSs, compared with LTSs, more commonly presented with cranial nerve palsy (83% v 73%, respectively; P = .008), ring enhancement (38% v 23%, respectively; P = .007), necrosis (42% v 26%, respectively; P = .009), and extrapontine extension (92% v 86%, respectively; P = .04). LTSs more commonly received systemic therapy at diagnosis (88% v 75% for STSs; P = .005). Biopsies and autopsies were performed in 299 patients (30%) and 77 patients (10%), respectively; 181 tumors (48%) were molecularly characterized. LTSs were more likely to harbor a HIST1H3B mutation (odds ratio, 1.28; 95% CI, 1.1 to 1.5; P = .002). Conclusion We report clinical, radiologic, and molecular factors that correlate with survival in children and young adults with DIPG, which are important for risk stratification in future clinical trials.
- Microsurgical Resection of Glomus Jugulare Tumors With Facial Nerve Reconstruction: 3-Dimensional Operative Video. [Journal Article]
- ONOper Neurosurg (Hagerstown) 2018 May 08
- Paragangliomas are tumors originating from the paraganglionic system (autonomic nervous system), mostly found at the region around the jugular bulb, for which reason they are also termed glomus jugul...
Paragangliomas are tumors originating from the paraganglionic system (autonomic nervous system), mostly found at the region around the jugular bulb, for which reason they are also termed glomus jugulare tumors (GJT). Although these lesions appear to be histologically benign, clinically they present with great morbidity, especially due to invasion of nearby structures such as the lower cranial nerves. These are challenging tumors, as they need complex approaches and great knowledge of the skull base. We present the case of a 31-year-old woman, operated by the senior author, with a 1-year history of tinnitus, vertigo, and progressive hearing loss, that evolved with facial nerve palsy (House-Brackmann IV) 2 months before surgery. Magnetic resonance imaging and computed tomography scans demonstrated a typical lesion with intense flow voids at the jugular foramen region with invasion of the petrous and tympanic bone, carotid canal, and middle ear, and extending to the infratemporal fossa (type C2 of Fisch's classification for GJT). During the procedure the mastoid part of the facial nerve was identified involved by tumor and needed to be resected. We also describe the technique for nerve reconstruction, using an interposition graft from the great auricular nerve, harvested at the beginning of the surgery. We achieved total tumor resection with a remarkable postoperative course. The patient also presented with facial function after 6 months. The patient consented with publication of her images.
- Angiocentric lymph proliferative disorder (lymphomatoid granulomatosis) in a person with newly-diagnosed HIV infection: a case report. [Journal Article]
- BIBMC Infect Dis 2018 May 08; 18(1):210
- CONCLUSIONS: Albeit rare, ALPD should be considered in the differential diagnosis of central nervous system lesions in persons with HIV once common etiologies have been eliminated. Furthermore, ALPD involving the central nervous system may occur in in the absence of documented EBV infection in the central nervous system.
- Dural arteriovenous fistula of the lateral foramen magnum region: A review. [Journal Article]
- INInterv Neuroradiol 2018 Jan 01; :1591019918770768
- The lateral foramen magnum region is defined as the bilateral occipital area that runs laterally up to the jugular foramen. The critical vasculatures of this region are not completely understood. Dur...
The lateral foramen magnum region is defined as the bilateral occipital area that runs laterally up to the jugular foramen. The critical vasculatures of this region are not completely understood. Dural arteriovenous fistulas that occur in this region are rare and difficult to treat. Therefore, we searched PubMed to identify all relevant previously published English language articles about lateral foramen magnum dural arteriovenous fistulas, and we performed a review of this literature to increase understanding about these fistulas. Four types of dural arteriovenous fistulas occur in the lateral foramen magnum region. These include anterior condylar confluence and anterior condylar vein dural arteriovenous fistulas, posterior condylar canal dural arteriovenous fistulas, marginal sinus dural arteriovenous fistulas, and jugular foramen dural arteriovenous fistulas. These dural arteriovenous fistulas share similar angioarchitectures and clinical characteristics. The clinical presentations of lateral foramen magnum dural arteriovenous fistulas include pulsatile tinnitus, intracranial hemorrhage, myelopathy, orbital symptoms, and cranial nerve palsy. Currently, head computed tomography, computed tomography angiography, magnetic resonance imaging, magnetic resonance angiography and digital subtraction angiography (DSA) are useful for diagnosing dural arteriovenous fistulas, and of these, DSA remains the "gold standard." Most lateral foramen magnum dural arteriovenous fistulas need to be treated due to their aggressive symptoms, and transvenous embolization presents the best options. During treatment, it is critical to accurately place the microcatheter into the fistula point, and intraoperative integrated computed tomography and DSA data are very helpful. Other treatments, such as transarterial embolization, microsurgery or conservative treatment, can also be chosen. After appropriate treatment, most patients with lateral foramen magnum dural arteriovenous fistulas achieve satisfactory outcomes.
- Clinical experience of intracranial chordoma - A systematic review and meta-analysis of the literature. [Review]
- JCJ Clin Neurosci 2018 Apr 30
- Chordomas are rare neoplasms of the axial skeleton, with a predilection for the clival region. The lack of data about intracranial chordomas has led to inconsistent reporting of their clinical behavi...
Chordomas are rare neoplasms of the axial skeleton, with a predilection for the clival region. The lack of data about intracranial chordomas has led to inconsistent reporting of their clinical behavior and management principles. We performed a systematic review and meta-analysis with the aim of reporting on clinical behavior and management in the literature. We performed an electronic search of the MEDLINE, EMBASE, LILACS, Web of Science, Cochrane, and CINAHL databases. We included studies of patients with intracranial chordoma that had undergone surgical management with 5-year data available. The weighted mean estimate of 5-year OS was 77.4% (95% CI, 72.9-81.7%) whilst the 5-year PFS estimate was 49.4% (95% CI, 41.6-57.3%). A wide variety of surgical approaches were utilised, with transcranial approaches being the commonest. Surgeons achieved gross total resection in 29.4% of cases (95% CI, 17.6-43.0%), Sub-total resection in 60.9% of cases (95% CI, 40.2-79.8%) and partial resection in 19.7% (95% CI; 11.9 to 28.9%). The total complication rate was 28.5% (95% CI, 18.4-39.8%), with the commonest being new cranial nerve palsy (weighted mean 11.1%, 95% CI, 7.11-15.7%). Our review demonstrates that a wide range of management options exist in the literature. These should be tailored to each patient based on factors including tumoral location, local involvement and capabilities of the surgical team. The outcomes of chordoma management have largely been stable with no improvement despite improvements in modern surgical techniques.
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- Vertebral Artery Occlusion Causing Facial Colliculus and Opalski Stroke Syndromes Simultaneously. [Journal Article]
- NNeurologist 2018; 23(3):100-103
- Brainstem stroke syndromes refer to a group of vascular disorders that occur secondary to occlusion of small perforating arteries of the posterior circulation. Although such syndromes result in a cha...
Brainstem stroke syndromes refer to a group of vascular disorders that occur secondary to occlusion of small perforating arteries of the posterior circulation. Although such syndromes result in a characteristic clinical picture, they are challenging to localize. In practice, such syndromes are rarely seen in their pure form, and the clinical consequences of a given vascular lesion are probably less predictable than for arteries in the anterior circulation. If 2 or more brainstem strokes happen simultaneously, localization becomes much harder. In this article, we present a case with 2 embolic strokes that occurred simultaneously because of left vertebral artery occlusion. The first in the right facial colliculus causing right sixth and seventh cranial nerves' palsy. The second happened in the left lateral medulla extending below the pyramidal decussation causing a classic left lateral medullary syndrome with ipsilateral hemiparesis, which constitutes a left Opalski stroke syndrome. To our knowledge, this is the first reported case of these 2 brainstem stroke syndromes happening simultaneously.