Phaeohyphomycosis is caused by pigmented fungi that are not normally pathogenic. Fonsecaea is a rare cause of cerebral infections, most of which are caused by F. monophora. Brain infections caused by F. pedrosoi are very rare, and there are only a few case reports describing this. Most infections take the form of abscesses (epidural or intracerebral). The authors report a rare case of a contained fungal granuloma caused by F. pedrosoi. The patient presented with epilepsy, which was treated as a case of extratemporal lesion–related epilepsy. The diagnosis was made after resection. The authors describe the clinical course of this patient.
Venkatesh S. Madhugiri, Indira Devi Bhagavatula, Anita Mahadevan and Nagarathna Siddaiah
Venkatesh S. Madhugiri, Mario K. C. Teo, Joli Vavao, Teresa Bell-Stephens and Gary K. Steinberg
Brainstem arteriovenous malformations (AVMs) are rare lesions that are difficult to diagnose and treat. They are often more aggressive in their behavior when compared with their supratentorial counterparts. The consequence of a brainstem hemorrhage is often devastating, and many patients are in poor neurological status at presentation. The authors examine the factors associated with angiographically confirmed cure and those affecting management outcomes for these complex lesions.
This was a retrospective analysis of data gathered from the prospectively maintained Stanford AVM database. Lesions were grouped based on their location in the brainstem (medulla, pons, or midbrain) and the quadrant they occupied. Angiographic cure was dichotomized as completely obliterated or not, and functional outcome was dichotomized as either independent or not independent at last follow-up.
Over a 23-year period, 39 lesions were treated. Of these, 3 were located in the medulla, 14 in the pons, and 22 in the midbrain. At presentation, 92% of the patients had hemorrhage, and only 43.6% were functionally independent. Surgery resulted in the best radiographic cure rates, with a morbidity rate of 12.5%. In all, 53% of patients either improved or remained stable after surgery. Absence of residual nidus and female sex correlated with better outcomes.
Brainstem AVMs usually present with hemorrhage. Surgery offers the best chance of cure, either in isolation or in combination with other modalities as appropriate.
Venkatesh S. Madhugiri, Sudheer Ambekar, Shane F. Strom and Anil Nanda
The volume of scientific literature doubles approximately every 7 years. The coverage of this literature provided by online compendia is variable and incomplete. It would hence be useful to identify “core” journals in any field and validate whether the h index and impact factor truly identify the core journals in every subject. The core journals in every medical specialty would be those that provide a current and comprehensive coverage of the science in that specialty. Identifying these journals would make it possible for individual physicians to keep abreast of research and clinical progress.
The top 10 neurosurgical journals (on the basis of impact factor and h index) were selected. A database of all articles cited in the reference lists of papers published in issues of these journals published in the first quarter of 2012 was generated. The journals were ranked based on the number of papers cited from each. This citation rank list was compared with the h index and impact factor rank lists. The rank list was also examined to see if the concept of core journals could be validated for neurosurgical literature using Bradford's law.
A total of 22,850 papers spread across 2522 journals were cited in neurosurgical literature over 3 months. Although the top 10 journals were the same, irrespective of ranking criterion (h index, impact factor, citation ranking), the 3 rank lists were not congruent. The top 25% of cited articles obeyed the Bradford distribution; beyond this, there was a zone of increased scatter. Six core journals were identified for neurosurgery.
The core journals for neurosurgery were identified to be Journal of Neurosurgery, Neurosurgery, Spine, Acta Neurochirurgica, Stroke, and Journal of Neurotrauma. A list of core journals could similarly be generated for every subject. This would facilitate a focused reading to keep abreast of current knowledge. Collated across specialties, these journals could depict the current status of medical science.
Mario K. Teo, Venkatesh S. Madhugiri and Gary K. Steinberg
A systematic review
Venkatesh S. Madhugiri, Sudheer Ambekar, V. R. Roopesh Kumar, Gopalakrishnan M. Sasidharan and Anil Nanda
Spinal aneurysms (SAs) are rare lesions. The clinicoradiological features and the exact degree of their association with comorbid conditions such as arteriovenous malformations (AVMs) and coarctation of the aorta have not been definitively described. The ideal management paradigm has not been established. The authors reviewed literature to determine the clinical patterns of presentation, management, and outcome of spinal aneurysms.
A systematic review of literature was performed using 23 separate strings. A total of 10,190 papers were screened to identify 87 papers that met the inclusion criteria. A total of 123 SAs could be included for analysis.
The mean age of patients at presentation was 38 years; 10% of patients were aged less than 10 years and nearly 50% were greater than 38 years. Spinal aneurysms can be divided into 2 groups: those associated with AVMs (SA-AVMs, or Type 1 SAs) and those with isolated aneurysms (iSAs, or Type 2 SAs). Patients with Type 2 SAs were older and more likely to present with bleeding than those with Type 1 SAs. The acute syndromes can be divided into 3 groups of patients: those with spinal syndrome, those with cranial/craniospinal syndrome, and those with nonspecific presentation. Overall, 32.6% presented with angiography-negative cranial subarachnoid hemorrhage (SAH). Presentation with evidence of cord dysfunction (myelopathy/weakness/sensory loss/bladder involvement) correlated with poor outcome, as did presentation with hemorrhage and association with other comorbid conditions. Surgery and endovascular therapy both led to comparable rates of complete aneurysm obliteration for Type 2 SAs, whereas for the AVM-associated Type 1 SAs, surgery led to better rates of lesion obliteration. The authors propose a classification scheme for spinal aneurysms based on whether the lesion is solitary or is associated with a coexistent spinal AVM; this would also imply that the ideal therapy for the aneurysm would differ based on this association.
The clinical and radiological patterns that influence outcome are distinct for Type 1 and Type 2 SAs. The ideal treatment for Type 1 SAs appears to be excision, whereas surgery and endovascular therapy were equally effective for Type 2 SAs.
Madhugiri S. Venkatesh, Paritosh Pandey, B. Indira Devi, Kiran Khanapure, Sathyanarayana Satish, Somanna Sampath, Bangalore A. Chandramouli and Kolluri V. R. Sastry
Pediatric cases of infratentorial subdural empyema (SDE) are both rare and associated with high rates of morbidity and mortality. The goal of this study was to report patient characteristics, treatment, and outcome in an exclusively pediatric series of SDE cases.
A series of 14 pediatric cases of infratentorial SDE wasretrospectively analyzed. All patients were treated between 1994 and 2004. Sixty-four percent of the patients were boys; the majority of cases occurred during the summer months. Clinical features included headache, fever, vomiting, meningism, and otorrhea. Cerebellar signs were found only in 21% of patients. In 85.7% of the cases, the patients presented with a depressed level of consciousness (Glasgow Coma Scale Scores 11–15). In 79.6%, pus collection was seen over the cerebellar convexity; interhemispheric and tentorial collections were also observed in some cases. Hydrocephalus was present in 92.9% of patients. Five patients required external ventricular drainage during surgery or postoperatively. Shunt placement was required in 21% of cases.
All patients were treated with antibiotic therapy and surgery (bur holes in 21% of the cases, craniectomy in 79%). Pus cultures demonstrated microbial infection in 71.4%, and polymicrobial infection in 21%. Four patients required repeated surgery for reaccumulation of pus. Minor postoperative complications developed in three patients. All 14 patients survived. At follow up, the Glasgow Outcome Scale scores were 4 or 5 in all cases.
Early diagnosis and prompt surgical treatment are crucial in cases of SDE. With appropriate surgery, antibiotic therapy, and management of hydrocephalus, good outcome can be expected.
Venkatesh Shankar Madhugiri, Sudheer Kumar Gundamaneni, Vani Santosh, Barath Jagadisan, Gopalakrishnan Madhavan Sasidharan, Rathakrishnan V. Roopesh-Kumar, Awdhesh Kumar Yadav, Manish Singh, Ramesh Ananthakrishnan, Nisha Pariarath and Niranjan Biswal
In this report the authors describe a rare case of a fulminant, pyogenic, necrotizing infection of the spinal cord and brain. Necrotizing lesions of the brain and spinal cord are usually infectious in origin and are associated with high rates of morbidity and death. Although the pathogens responsible have been identified in a few instances, the causal factors remain unknown in many cases. An 11-year-old girl developed acute, rapidly progressive paraplegia with bladder involvement and sensory loss below T-10. She had been treated recently for a Staphylococcus aureus infection of the knee joint precipitated by a penetrating injury with organic matter in the aftermath of a cyclone. Although appropriate antibiotic therapy was instituted, the spinal cord infection progressed to involve the entire spinal cord, brainstem, and brain. This fulminant course was marked by a rapid deterioration in the patient's clinical condition, ultimately leading to her death. Magnetic resonance imaging demonstrated a previously undescribed pattern of longitudinal enhancement along the spinal cord, as well as the white matter tracts in the brainstem and brain. The possible route of spread of infection along the neuraxis is postulated to be the potential space along the white matter tracts. Treatment is not standardized due to the rarity of the condition.
Eric S. Sussman, Venkatesh Madhugiri, Mario Teo, Troels H. Nielsen, Sunil V. Furtado, Arjun V. Pendharkar, Allen L. Ho, Rogelio Esparza, Tej D. Azad, Michael Zhang and Gary K. Steinberg
Revascularization surgery is a safe and effective surgical treatment for symptomatic moyamoya disease (MMD) and has been shown to reduce the frequency of future ischemic events and improve quality of life in affected patients. The authors sought to investigate the occurrence of acute perioperative occlusion of the contralateral internal carotid artery (ICA) with contralateral stroke following revascularization surgery, a rare complication that has not been previously reported.
This study is a retrospective review of a prospective database of a single surgeon’s series of revascularization operations in patients with MMD. From 1991 to 2016, 1446 bypasses were performed in 905 patients, 89.6% of which involved direct anastomosis of the superficial temporal artery (STA) to a distal branch of the middle cerebral artery (MCA). Demographic, surgical, and radiographic data were collected prospectively in all treated patients.
Symptomatic contralateral hemispheric infarcts occurred during the postoperative period in 34 cases (2.4%). Digital subtraction angiography (DSA) was performed in each of these patients. In 8 cases (0.6%), DSA during the immediate postoperative period revealed associated new occlusion of the contralateral ICA. In each of these cases, revascularization surgery involved direct anastomosis of the STA to an M4 branch of the MCA. Preoperative DSA revealed moderate (n = 1) or severe (n = 3) stenosis or occlusion (n = 4) of the ipsilateral ICA and mild (n = 2), moderate (n = 4), or severe (n = 2) stenosis of the contralateral ICA. The baseline Suzuki stage was 4 (n = 7) or 5 (n = 1). The collateral supply originated exclusively from the intracranial circulation in 4/8 patients (50%), and from both the intracranial and extracranial circulation in the remaining 50% of patients. Seven (88%) of 8 patients improved symptomatically during the acute postoperative period with induced hypertension. The modified Rankin Scale (mRS) score at discharge was worse than baseline in 7/8 patients (88%), whereas 1 patient had only minor deficits that did not affect the mRS score. At the 3-year follow-up, 3/8 patients (38%) were at their baseline mRS score or better, 1 patient had significant disability compared with preoperatively, 2 patients had died, and 1 patient was lost to follow-up. Three-year follow-up is not yet available in 1 patient.
Acute occlusion of the ICA on the contralateral side from an STA-MCA bypass is a rare, but potentially serious, complication of revascularization surgery for MMD. It highlights the importance of the hemodynamic interrelationships that exist between the two hemispheres, a concept that has been previously underappreciated. Induced hypertension during the acute period may provide adequate cerebral blood flow via developing collateral vessels, and good outcomes may be achieved with aggressive supportive management and expedited contralateral revascularization.
Venkatesh S. Madhugiri, Mario K. C. Teo, Erick M. Westbroek, Steven D. Chang, Michael P. Marks, Huy M. Do, Richard P. Levy and Gary K. Steinberg
Arteriovenous malformations (AVMs) of the basal ganglia and thalamus are particularly difficult lesions to treat, accounting for 3%–13% of all AVMs in surgical series and 23%–44% of malformations in radiosurgery series. The goal of this study was to report the results of multimodal management of basal ganglia and thalamic AVMs and investigate the factors that influence radiographic cure and good clinical outcomes.
This study was a retrospective analysis of a prospectively maintained database of all patients treated at the authors’ institution. Clinical, radiological, follow-up, and outcome data were analyzed. Univariate and multivariate analyses were conducted to explore the influence of various factors on outcome.
The results and data analysis pertaining to 123 patients treated over 32 years are presented. In this cohort, radiographic cure was achieved in 50.9% of the patients. Seventy-five percent of patients had good clinical outcomes (stable or improved performance scores), whereas 25% worsened after treatment. Inclusion of surgery and radiosurgery independently predicted obliteration, whereas nidus diameter and volume predicted clinical outcomes. Nidus volume/diameter and inclusion of surgery predicted the optimal outcome, i.e., good clinical outcomes with lesion obliteration.
Good outcomes are possible with multimodal treatment in these complex patients. Increasing size and, by extension, higher Spetzler-Martin grade are associated with worse outcomes. Inclusion of multiple modalities of treatment as indicated could improve the chances of radiographic cure and good outcomes.