Apoplexy Inside a Giant Medial Sphenoid Wing Meningothelial (Grade I) Meningioma: An Extremely Rare but a Potentially Dangerous Complication.

Hemorrhage associated with meningiomas is extremely rare and most commonly occurs in convexity meningiomas of higher grade or the angioblastic variety. Moreover, bleeding associated with a meningioma usually occurs in the form of a subdural hematoma or subarachnoid hemorrhage. We report a case of giant left medial sphenoid wing meningioma with histopathological diagnosis of a meningothelial type with apoplexy. A 54-year-old female presented with clinical features suggestive of apoplexy. Her neuroimaging demonstrated a large left medial sphenoid wing meningioma with features suggestive of an intratumoral bleed with mass effect. Gross total excision of the tumor was done with the good postoperative outcome. The biopsy came out to be Grade I meningothelial meningioma. Apoplexy in meningiomas is extremely rare with a reported incidence of 0.5%-2.4%, especially in a nonconvexity meningioma with histopathological diagnosis of meningothelial variety. Early diagnosis and prompt surgical intervention are critical as meningiomas associated with apoplexy are associated with high morbidity.

Isolated Langerhans Cell Histiocytosis Masquerading as Intradural Extramedullary Meningioma: Review on Histiocytic Disorders of Spine.

The histiocytic disorders are pathological diagnosis and rarely affects spine. The spinal involvement is characterized by lytic lesions and painful symptoms. Isolated intradural extramedullary involvement is rare presentation. A 15-year-old female patient presented with nontraumatic cervical compressive myelopathy and was operated electively with preoperative diagnosis of meningioma. The histopathology was surprisingly Langerhans cell histiocytosis. In lineage of histiocytic development, the Langerhans cells develop into matured dendritic cells and lose its Birbeck granules and CD1a antigenicity. With the understanding of histiocyte lineage system, the disorders concerned with central nervous system are classified into dendritic cell disorders and macrophages-related disorders. In this article, we have discussed on histiocytic disorders of central nervous system and management guidelines in case one comes across such histopathology.

Health-related quality of life after surgery in supratentorial gliomas.

INTRODUCTION: With improvements in overall and progression-free survival in gliomas, current focus in neurosurgical oncology has largely shifted to the issue of quality of life (QOL) after treatment. There are not too many prospective studies evaluating QOL in these patients. We prospectively analyzed the health related quality of life (HRQOL) using the short form (SF) 36 questionnaire among patients harbouring a supratentorial glioma who underwent surgery at a tertiary care center in India. METHODS: HRQOL was evaluated prospectively in 103 patients, between May 2016 and November 2017, at Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow. Score improvements were evaluated in terms of the smallest difference that a patient perceived to be clinically beneficial. The responsiveness of scores was assessed as minimum clinically important difference (MCID), effect size (ES) and relative efficiency. Cronbach's alpha co-efficient was used to assess the reliability of the result obtained. RESULTS: The mean pre-operative score was the highest in the mental health domain (50.21±21.45) and significantly lower in patients with raised intracranial pressure (ICP), pre-operative deficits, a poor Karnofsky performance scale (KPS) score, in large tumors (>4cm) located in an eloquent location and in deep-seated tumors. A significant improvement was noted across all domains except the physical role (P-value 0.20). Overall, the general health domain was most responsive to change with the largest t-value (6.56) and 55.7% patients achieved their target MCID in general health (GH) domain. Among all the factors studied, only a low baseline SF-36 score was significantly associated with a change in QOL after surgery. CONCLUSION: Most parameters of HRQOL improved following surgery. Only small-to-moderate improvements occured in the early follow-up period. Large improving trends were noted on a long-term basis, irrespective of the histo-pathological grade. Similar improvements were also noted in patients who subsequently underwent re-surgery despite the development of post-operative complications or new deficits. Also, a low baseline QOL score heralded a poor survival.

Pathologic Laughter as an Early and Unusual Presenting Symptom of Petroclival Meningioma: a Case Report and Review of the Literature.

BACKGROUND: Pathologic laughter is inappropriate, involuntary, and unmotivated laughter episodes that may or may not be associated with mirth or amusement. Although associated with many diffuse brain pathologies, its association with intracranial focal mass lesions causing ventrolateral brainstem compression, like petroclival meningioma, is very rare. The exact pathophysiology of this interesting and unusual clinical symptom is unknown, but probably involves disinhibition and release of the so-called coordination center located in the upper brainstem due to compression by the tumor. CASE DESCRIPTION: A 26-year-old woman presented with recurrent episodes of inappropriate and involuntary laughter, which significantly affected her quality of life, for 2 years. These episodes did not resolve, and a magnetic resonance imaging of the brain showed a giant petroclival meningioma causing upper brainstem compression. Near-total excision of the tumor was done using an extended middle fossa approach. To our surprise, the pathologic laughter subsided immediately after surgery. CONCLUSIONS: Pathologic laughter may be the only symptom of a focal mass lesion causing ventrolateral upper brainstem compression, like petroclival meningioma, well before other neurological sign/symptoms appear. Tumors causing ventral brainstem compression must be ruled out before the patient is sent for a psychiatric evaluation.

Surgical outcome in cortical ependymoma: A single centre experience of 18 cases.

Cortical ependymomas (CE) are rare subset of supratentorial ependymoma which are located in the peripheral cortical rim without any connection to the ventricular lining. With limited cases previously reported, current knowledge on diagnosis and management of tumors is lacking. We present the largest single center experience on CE reported so far and highlight their clinico-radiological aspects, histopathological features as well the results of their surgical excision. We studied 18 CE patients undergoing surgical excision at our center between September 2009 to November 2017. Clinical, radiological, histopathological and operative data was obtained from hospital records. Functional assessment of our patients were done using the Karnofsky's performance score (KPS). Survival analysis was done using Kaplan-Meier method and log rank test. Mean age of patients in our study group was 19 ±â€¯11.1 years. Frontal lobe was the most frequently involved region. Features of raised intracranial pressure like holocranial headache (n = 15, 83.33%) and vomiting (n = 9, 50%) were most common presenting complaints in our study. Gross total resection of tumor was achieved in eleven patients (61.11%). Histopathology showed equal number (n = 9) of WHO grade 2 and 3 ependymoma. During 111 months follow-up, four patients (22.22%) developed recurrence and three patients (16.66%) died. Five years overall survival (OS), progression-free survival (PFS) rate were 74.3% and 70.7%. In view of higher risk of progression to higher histo-pathological grade and local recurrence years after surgical excision, a long clinical and radiological follow-up is advised.

Cervicothoracic Spinal Dysraphism: Unravelling the Pandora's Box.

INTRODUCTION: Cervicothoracic spinal dysraphism (CTSD) is relatively rare with reported incidence of 1%-6.5%. The entity has a separate spectrum of associations with other anomaly such as split cord malformation, Chiari malformation, and corpus callosum agenesis as compared with its lumbosacral counterpart. In this study, we have highlighted the associated anomalies (AAs). To the best of our knowledge, this study is one of the largest series reported before. MATERIALS AND METHODS: In this study, we included the patients with spinal dysraphism operated between December 2007 and December 2017 at the Department of Neurosurgery. All patients underwent neurological and radiological examinations followed by surgical excision of the sac and exploration of the intradural sac. Neurological, orthopedic, and urological abnormalities were analyzed in our retrospective description. The last available follow-up in hospital records was taken for outcome assessment. RESULTS: Of 34 cases, 18 were women and 16 were men. Seven of nine patients in cervical group had AAs, whereas 22 of 25 patients in dorsal group had AA. In this study, we taken 6 patients with Chiari malformation, 4 with corpus callosum agenesis, 7 with hydrocephalus, and 16 with split cord malformation. The follow-up ranges from 1 year to 11 years with a mean of 59.45 months. CONCLUSION: The management strategy and association with other congenital anomalies separate CTSD as different clinical entity as compared with their lumbosacral counterparts. These patients show relatively favorable outcome with regard to neurological, orthopedic, and urological symptoms. Early surgical intervention after proper radiological and clinical evaluation is recommended.

Completely Thrombosed Giant Intracranial Aneurysm with Spontaneous Thrombosis of the Parent Artery: Is It Nature's Divine Intervention and a Self-Cure?

BACKGROUND: Although partial thrombosis is common with intracranial aneurysms, complete aneurysmal thrombosis is extremely rare. An even rarer and seldom reported phenomenon is the spontaneous complete occlusion of the parent artery in the presence of a thrombosed aneurysm. Given the unclear natural history of this phenomenon, therapeutic decision making may be rather difficult. Here we report a case of right cavernous carotid artery aneurysm with spontaneous right internal carotid artery (ICA) thrombosis in a 45-year-old man. CASE DESCRIPTION: Our patient presented almost 1 month after an episode of sudden-onset severe holocranial headache associated with vomiting that had resolved completely within 2 days. He was neurologically intact at the time of presentation. A computed tomography (CT) scan done at the time of ictus showed a rounded hyperdensity with the epicenter at the right parasellar region along with nonvisualization of the ipsilateral ICA on CT angiography. Magnetic resonance imaging (MRI) suggested a diagnosis of a completely thrombosed right cavernous segment ICA aneurysm. Intra-arterial angiography confirmed a complete ipsilateral ICA thrombosis and a good arterial cross-flow from the left side to the bilateral anterior and middle cerebral artery territories. We started the patient on oral aspirin and a close clinicoradiologic follow-up after having decided against any active intervention. CONCLUSIONS: Spontaneous thrombosis of an intracranial aneurysm and the parent artery is rare, with only a few cases reported in the literature. In the absence of symptoms and adequate collateral circulation, these patients are perhaps best managed conservatively.

Type 1 Split Cord Malformation: The Significance of the 3D Orientation of the Bony Spur, Its Clinical Relevance, and Surgical Nuances.

BACKGROUND: Split cord malformation (SCM) remains an important cause of tethered cord syndrome. SCM type 1, wherein a bony spur separates the two dural sacs, represents a challenging condition to treat. The management can be further complicated by the other associated dysraphic states in the same patient. The bony spur at the level of split cord has been variably classified in the literature. There is, however, a paucity of literature dealing with the three-dimensional disposition of the bony spur and its clinical and operative implications. MATERIALS AND METHODS: Our study included 50 patients (male/female = 24:26) of SCM type 1. The orientation of bony spur was categorized into ventral complete, ventral incomplete, and dorsal types along with simultaneous assessment of their direction in sagittal and axial planes in multiplanar computed tomography (CT) scan. Surgical plan was individualized according to each patient's morbid anatomy. RESULTS: The most common site of bony spur was lumbar region (n = 24). Twenty-four (48%) patients had ventral complete type spur, 19 (38%) had ventral incomplete type, and 7 (14%) had dorsal bony spur. Dorsal bony spurs presented with a greater incidence of motor symptoms (71.4%) and bladder disturbances (28.5%), whereas the ventral incomplete type of bony spur was predominantly associated with sensory symptoms (42.1%). Patients with dorsal bony spurs showed better postoperative outcomes when compared to their ventral counterparts. CONCLUSION: Multiplanar assessment of the bony spur using CT scan helps in surgical drilling and improves the outcome. The dorsal SCM variety showed better clinical outcomes when compared to their ventral bony spurs.

Cystic Cervical Dysraphism: Experience of 12 Cases.

INTRODUCTION: Cystic spinal dysraphism of the cervical region is a relatively rare entity, which is more frequently associated with congenital anomalies such as split cord malformation, Chiari malformation, and corpus callosum agenesis, when compared to their lumbosacral counterpart. In our study, we have highlighted the clinical spectrum, associated anomalies (both neural and extra-neural), and surgical nuances of these. MATERIALS AND METHODS: This study is a retrospective analysis of 225 patients from June 2010 to April 2017. Twelve patients who were between the age of 1 month and 16 years were included in our study. Average age was 32.6 months, and there were five female patients and seven male patients. All patients underwent neurological and radiological examinations followed by surgical excision of the sac and exploration of the intradural sac using the standard microsurgical technique. Neurological, Orthopedic and urological outcomes were studied in our description. RESULTS: Of the 12 cases, 9 patients (75%) had some associated anomaly. Four of the 12 patients (25%) had split cord malformation, 3 had corpus callosum agenesis, and 5 had Chiari malformation. Patients with cervical spina bifida cystica (SBC) present with less neurologic deficits and greater association with CCA. CONCLUSION: The management strategy and association with other congenital anomalies separates cervical dysraphism as a different clinical entity rather than just group. These patients rather show favorable outcome with regard to neurologic, orthopedic, and urologic problems as compared to their caudal counterpart. Early surgical intervention even before the onset of symptoms is recommended. A proper radiological and urological evaluation is warranted.

Aspergilloma Mimicking Olfactory Groove Meningioma.

Central nervous system aspergillosis is rare with reported mortality rate of 13-50% involving foci in paranasal sinuses and even higher mortality rates (80-100%) in patients of immune-compromised status. Modern day imaging offers an opportunity for early diagnosis, but findings are minimal. A typical finding is that of a space occupying mass lesion with iso-hypointense signal on T1-weighted sequences and extreme hypointense appearance on T2-weighted. This results from the concentration of ferromagnetic substances (iron/zinc/magnesium/manganese) within the lesion. Gadolinium enhancement pattern varies from homogeneous to peripheral ring enhancement. The immune-competent hosts present with homogeneous enhancement while those with immune compromise bear more variable radiological appearance. Due to such radiological appearance, meningioma or tuberculoma is considered in differential earlier than fungal granuloma, if not for the hypointense appearance on T2-weighted sequences which strongly points to fungal granuloma. Through this case report, we attempt to highlight the unusual radiological presentation of the entity.

Sporadic Cisternal Oculomotor Nerve Schwannoma: A Rare Case with Review of Literature.

Cranial nerve schwannomas usually arise from sensory nerve and the occurrence of schwannoma in a motor nerve is rare, especially in sporadic cases. Oculomotor nerve schwannomas (ONS) are rare and they are unique as they arise from motor nerve. ONS palsy may or may not be the presenting feature of oculomotor schwannoma. We present the case of a young male with ONS, presenting with oculomotor nerve palsy along with features of raised intracranial pressure. Oculomotor schwannoma is described in literature only as case reports, and oculomotor nerve is also a rare site for schwannoma as being a motor nerve. In this article, we describe a case of cisternal ONS with review of pertinent literature.

Mirror Aneurysms Among Multiple Aneurysms: Lesser of the Two Evils.

BACKGROUND: To highlight the management nuances in addressing mirror aneurysms (MirAns) in a subset of multiple aneurysms. METHODS: A retrospective study was carried out and all multiple intracranial aneurysms (n = 70) over 9 years were identified. Exclusion criteria were associated arteriovenous malformations, moyamoya disease, and either traumatic or mycotic aneurysms or those managed by endovascular approach alone. Patients were grouped as MirAn and nonmirror aneurysms (nMirAns). Outcome was assessed by Glasgow Outcome Score at 1 month. The Pearson χ(2) test or Fisher exact test were used to establish association. RESULTS: MirAns (n = 17) accounted for 2.1% of all intracranial aneurysms and 24.3% of multiple aneurysms. Female predilection was eminent in the MirAn group (3.25:1) compared with the nMirAn group (2.5:1). The middle cerebral artery (n = 6) followed by the internal carotid artery-posterior communicating artery (n = 5) were the most common sites of the MirAns. There was no significant difference between the 2 groups for hypertension or age (MirAn, 52 years [± 11.231]; hypertension, 52.9%; nMirAn, 48.58 years [± 12.97]; hypertension, 54.7%). There was a definite trend towards less intraoperative rupture (MirAn, 4/17; nMirAn, 27/53; P = 0.048), decreased intraoperative vasospasm (MirAn, 1; nMirAn, 11) in the MirAn group. Development of infarct was significantly less in the MirAn group (P = 0.027) with a better outcome (13 in MirAn and 26 in nMirAn had good outcome; P = 0.048). CONCLUSIONS: MirAns are not significantly associated with risk factors such as age, hypertension, and diabetes. Intraoperative rupture, vasospasm, and infarct are less common, with a tendency towards better neurologic outcome compared with nMirAns.

Factors Predicting Poor Outcome in a Surgically Managed Series of Multiple Intracranial Aneurysms.

OBJECTIVE: Multiple factors are known to influence outcomes in single-aneurysm subarachnoid hemorrhage, such as Hunt and Hess (H&H) grade, hypertension, etc. We sought to assess the influence of individual risk factors on outcome in surgically managed patients with multiple intracranial aneurysm. METHODS: A retrospective review of consecutive 780 patients of subarachnoid hemorrhage revealed multiple intracranial aneurysm in 63 patients with 146 aneurysms. Clinicoradiologic features, hospital course, and outcome obtained via use of the Glasgow Outcome Score were noted from hospital records. H&H grade was divided into 2 groups as good and poor whereas Glasgow Outcome Score at 1 month was divided into 2 groups as favorable and unfavorable for analysis. To test association among variables, a χ(2) test/Fisher exact test was used. Risk was calculated in exposure group by the use of univariate logistic regression and multivariate analysis (binary logistic regression model). RESULTS: A definite female preponderance was observed (male/female ratio: 1:2.71). The most common site was middle cerebral artery bifurcation (overall and male patients, n = 43); in women, it was internal carotid artery bifurcation. Anterior communicating artery aneurysm was the most common to bleed (n = 22). Early presentation resulted in worse outcomes (<4: 4-21: >21days = 44:37:33, P = .844). Vasospasm and infarct incidence was greatest in the group 4-14 days (n = 37). Infarct evolved in 15 patients (8 in territory of aneurysmal dissection/distribution). Factors influencing patient outcomes were age ≥65 years (P = 0.037), H&H grade (P = 0.04), posterior communicating artery distribution of aneurysm (P = 0.03), hypertension (P = 0.03), infarct (P = 0.001), and hydrocephalus (P = 0.01) CONCLUSIONS: Poor H&H grade, hypertension, posterior communicating artery distribution, elderly age, infarct, and hydrocephalus each influenced poor outcomes at 1 month. Hydrocephalus is predictive of poor outcome.