Intrinsic epidermoid cyst of the brainstem in children-review and case report.

PURPOSE: The study aimed to summarize all published cases of intrinsic brainstem epidermoid cysts in a timeline to highlight the specific characteristics and individualize the disease, in addition to discussing the best treatment used. METHODS: The scientific literature on pediatric cases of intrinsic epidermoid cysts of the brainstem was analyzed. We present the case of a 1.5-year-old male with incidental presentation, who was treated with gross total resection. We summarize all previously published cases to individualize the disease. RESULTS: We identified 21 patients, including 10 boys and 11 girls, with a mean age of 4.85 (1-15) years at the time of surgery. The most frequent symptoms were cranial nerve palsy (71.4%), pyramidal tract deficit (57.14%), and headache (52.38%). Among the affected cranial nerves, VII was the most frequently reported in 10 patients. CONCLUSION: Brainstem epidermoid cysts are extremely rare pathologies with relevant age involvement in young children. The treatment objective should be the maximum resection of the lesion through a careful approach and with the appropriate tools for the functional preservation of the patient.

Adult-onset Alexander disease with brainstem and cervical cord enhancing lesions.

Leukodystrophies are a group of genetic diseases with diverse clinical features and prominent involvement of the central nervous system white matter. We describe a 27-year-old man who presented with a progressive neurological disease, and striking involvement of the brainstem and symmetrical white matter lesions on MR scanning. Having excluded several other causes of leukodystrophy, we confirmed Alexander disease when a genetic panel showed a probable pathogenic variant in GFAP: p.Leu359Pro. Clinicians should suspect Alexander disease in people with a progressive neurological motor decline who has pyramidal and bulbar signs and compatible neuroimaging.

Stereotactic biopsy for lesions in brainstem and deep brain: a single-center experience of 72 cases.

Stereotactic biopsies for lesions in the brainstem and deep brain are rare. This study aimed to summarize our 6-year experience in the accurate diagnosis of lesions in the brain stem and deep brain and to discuss the technical note and strategies. From December 2011 to January 2018, 72 cases of intracranial lesions in the brainstem or deep in the lobes undergoing stereotactic biopsy were retrospectively reviewed. An individualized puncture path was designed based on the lesion's location and the image characteristics. The most common biopsy targets were deep in the lobes (43 cases, 59.7%), including frontal lobe (33 cases, 45.8%), temporal lobe (4 cases, 5.6%), parietal lobe (3 cases, 4.2%), and occipital lobe (3 cases, 4.2 %). There were 12 cases (16.7%) of the brainstem, including 8 cases (11.1%) of midbrain, and 4 cases (5.6%) of pons or brachium pontis. Other targets included internal capsule (2 cases, 2.8%), thalamus (3 cases, 4.2%), and basal ganglion (12 cases, 16.7%). As for complications, one patient developed acute intracerebral hemorrhage in the biopsy area at 2 h post-operation, and one patient had delayed intracerebral hemorrhage at 7 days post-operation. The remaining patients recovered well after surgery. There was no surgery-related death. The CT-MRI-guided stereotactic biopsy of lesions in the brainstem or deep in the brain has the advantages of high safety, accurate diagnosis, and low incidence of complications. It plays a crucial role in the diagnosis of atypical, microscopic, diffuse, multiple, and refractory lesions.

Brainstem hyperintensity in patients with vestibular schwannoma is associated with labyrinth signal on magnetic resonance imaging but not vestibulocochlear tests.

OBJECTIVES: Focal hyperintensity in the dorsal brainstem (HDB) has been described in large cerebellopontine angle tumours and is thought to represent vestibular nuclei degeneration, but its functional significance has not been thoroughly investigated. Our aim was to analyse its relationship to imaging characteristics of the tumour and inner-ear structures and to vestibulocochlear functional tests. METHODS: We retrospectively reviewed 54 patients with a histological diagnosis of vestibular schwannoma (VS). Magnetic resonance imaging tumour characteristics (size, cystic composition and distance from the cochlear aperture), signal intensity ratio of the cochlea and vestibule in fluid-attenuated inversion recovery (FLAIR) and fast imaging employing steady-state acquisition (FIESTA)/fast spin-echo imaging with variable flip angles (CUBE) and vestibulocochlear function tests (audiometry, auditory brainstem response (ABR) and video head impulse testing (vHIT)) were obtained. Statistical analyses were performed to evaluate their relation to focal HDB. RESULTS: Focal HDB was found in 22% of VS. It was significantly associated with large (p < 0.001) and cystic (p = 0.004) tumours and also with tumours located further from the cochlear aperture (p = 0.039). The signal intensity ratio of the cochlea on FLAIR was higher in patients with HDB (p < 0.014), but this difference was not observed in FIESTA/CUBE (p = 0.981). Audiometry, ABR and vHIT results did not significantly differ in patients with HDB, but ABR results were worse in patients with higher cochlear signal intensity on FLAIR sequences (p = 0.026). CONCLUSIONS: Focal HDB in patients with VS was associated with increased signal intensity ratio of the cochlea on FLAIR in patients with VS but not directly to the results of vestibulocochlear function tests.

Stereotactic biopsy for lesions in brainstem and deep brain: a single-center experience of 72 cases

Stereotactic biopsies for lesions in the brainstem and deep brain are rare. This study aimed to summarize our 6-year experience in the accurate diagnosis of lesions in the brain stem and deep brain and to discuss the technical note and strategies. From December 2011 to January 2018, 72 cases of intracranial lesions in the brainstem or deep in the lobes undergoing stereotactic biopsy were retrospectively reviewed. An individualized puncture path was designed based on the lesion's location and the image characteristics. The most common biopsy targets were deep in the lobes (43 cases, 59.7%), including frontal lobe (33 cases, 45.8%), temporal lobe (4 cases, 5.6%), parietal lobe (3 cases, 4.2%), and occipital lobe (3 cases, 4.2 %). There were 12 cases (16.7%) of the brainstem, including 8 cases (11.1%) of midbrain, and 4 cases (5.6%) of pons or brachium pontis. Other targets included internal capsule (2 cases, 2.8%), thalamus (3 cases, 4.2%), and basal ganglion (12 cases, 16.7%). As for complications, one patient developed acute intracerebral hemorrhage in the biopsy area at 2 h post-operation, and one patient had delayed intracerebral hemorrhage at 7 days post-operation. The remaining patients recovered well after surgery. There was no surgery-related death. The CT-MRI-guided stereotactic biopsy of lesions in the brainstem or deep in the brain has the advantages of high safety, accurate diagnosis, and low incidence of complications. It plays a crucial role in the diagnosis of atypical, microscopic, diffuse, multiple, and refractory lesions.

Continuous epileptiform discharges during sleep as an evolutionary pattern in patients with congenital Zika virus syndrome.

Congenital Zika virus syndrome (CZVS) is associated with severe neurological deficits. Clinical characteristics of epilepsy and the electroencephalographic (EEG) pattern in CZVS were documented in infancy. In this study, we aimed to describe the EEG findings observed during the follow-up of children with CZVS. Seventy-six EEGs of 55 children (60% female; mean age = 50 months) with confirmed CZVS were analyzed, considering the background, interictal, and ictal epileptiform discharges. Continuous (or almost continuous) epileptiform discharges during non-rapid eye movement sleep were identified in 22 (40%) patients. In 20 (90.1%) patients, the pattern was symmetrical, with an anterior predominance of the epileptiform activity. All patients with this pattern had epilepsy, which was severe in 15 (68.2%) and demanded polytherapy in 19 (86.4%). Subcortical calcifications (77.3%) and multifocal EEGs (72.8%) in earlier ages occurred more often in patients with this pattern. Other unspecific interictal EEG patterns were focal epileptiform discharges in 23 (41.8%) and multifocal activity in six (10.9%). In CZVS, continuous (or almost continuous) epileptiform discharges during sleep emerge as a pattern after the second year of life. This was associated with severe and drug-resistant epilepsy, but not necessarily with an apparent regression. Subcortical calcifications and multifocal epileptiform discharges in infancy are associated with this pattern.

Microsurgical Resection of a Left Cerebral Peduncle Cavernous Malformation: 3-Dimensional Operative Video.

Brainstem cavernous malformations are challenging lesions considering the numerous eloquent structures frequently related. Surgical resection is the preferred treatment if the patient is symptomatic and the lesion can be safely resected. We present the case of a right-handed 38-yr-old female, presenting with progressive impairment of her handwriting. Physical examination showed a right-sided grade 4/5 hemiparesis. Preoperative imaging was suggestive of a left cerebral peduncle cavernous malformation with a recent area of hemorrhage. The most superficial portion of the lesion was on the surface of the brainstem in the supratrigeminal safe entry zone of the pons. A frontotemporal craniotomy was performed, followed by a pretemporal transtentorial approach. Prior to performing brainstem incision, the area was stimulated, and no motor evoked potential was recorded. The hematoma was then evacuated, and the cavernous malformation was dissected and removed. The capsule was also dissected and removed, using neurophysiological monitoring to guide this procedure. The lesion was completely resected, and the patient was discharged on postoperative day 7 with a right-sided hemiparesis grade 3/5, which improved to grade 5/5 after 4 mo. The patient presented an improvement of her symptoms, with no new neurological deficits. Brainstem cavernomas can be safely removed in selected cases, using the adequate safe entry zone and the appropriate surgical approach. The pretemporal route can be used to reach the anterolateral aspect of the upper part of the brainstem, as it combines the advantages of both transsylvian and subtemporal approaches. An informed consent was obtained from the patient for publication of this operative video.

Dorsally exophytic glioblastoma of the pons.

Brainstem gliomas are rare tumours in adults, accounting for only 1%-2% of all intracranial gliomas. They are recognised as a heterogeneous group, in which most are malignant tumours. Brainstem gliomas are classified into four major groups according to the growth pattern on imaging, namely diffuse, focal, exophytic and cervicomedullary. Such a classification system is also useful for surgical decision making. The exophytic variant is extremely rare having anecdoctal reports in the literature. We report the case of an adult patient affected by an exophytic glioblastoma of the pons, which was submitted to subtotal resection followed by radiation therapy and chemotherapy with a longer overall survival. To the best of our knowledge, this is the seventh adult patient reported of an exophytic brainstem glioblastoma.

Frequency of brain MRI abnormalities in neuromyelitis optica spectrum disorder at presentation: A cohort of Latin American patients.

BACKGROUND: Brain magnetic resonance imaging (BMRI) lesions were classically not reported in neuromyelitis optica (NMO). However, BMRI lesions are not uncommon in NMO spectrum disorder (NMOSD) patients. OBJECTIVE: To report BMRI characteristic abnormalities (location and configuration) in NMOSD patients at presentation. METHODS: Medical records and BMRI characteristics of 79 patients with NMOSD (during the first documented attack) in Argentina, Brazil and Venezuela were reviewed retrospectively. RESULTS: BMRI abnormalities were observed in 81.02% of NMOSD patients at presentation. Forty-two patients (53.1%) showed typical-NMOSD abnormalities. We found BMRI abnormalities at presentation in the brainstem/cerebellum (n = 26; 32.9%), optic chiasm (n = 16; 20.2%), area postrema (n = 13; 16.4%), thalamus/hypothalamus (n = 11; 13.9%), corpus callosum (n = 11; 13.9%), periependymal-third ventricle (n = 9; 11.3%), corticospinal tract (n = 7; 8.8%), hemispheric white matter (n = 1; 1.2%) and nonspecific areas (n = 49; 62.03%). Asymptomatic BMRI lesions were more common. The frequency of brain MRI abnormalities did not differ between patients who were positive and negative for aquaporin 4 antibodies at presentation. CONCLUSION: Typical brain MRI abnormalities are frequent in NMOSD at disease onset.

Stereotactic Drainage of Brainstem Abscess With the BrainLab Varioguide™ System and the Airo™ Intraoperative CT Scanner: Technical Case Report.

BACKGROUND AND IMPORTANCE: Stereotactic biopsies or needle aspirations of posterior fossa lesions are technically challenging. Here we report a novel technique for performing these procedures employing the Airo™ intraoperative computed tomographic (CT) scanner and the VarioGuide™ articulated arm (BrainLab, Munich, Germany). CLINICAL PRESENTATION: A 62-yr-old woman presented with an irregularly shaped, enhancing lesion of the left pons/middle cerebellar peduncle. Slowed diffusion on magnetic resonance imaging suggested an abscess, but no definitive infectious agent/source could be identified. When the patient deteriorated despite broad-spectrum antibiotic therapy, she was taken to the operating room for stereotactic drainage of the abscess employing the described technique. A specific infectious agent (Eikenella corrodens) was identified from the aspirate, allowing for tailored antibiotic therapy. The procedure was well tolerated and the patient made a full recovery with minimal neurological sequelae. CONCLUSION: The combination of the Airo™ intraoperative CT and the Varioguide™ articulated arm allows for safe, accurate, and efficient targeting of posterior fossa lesions.

[Reversible posterior leukoencephalopathy syndrome with isolated involvement of the brainstem and spinal cord]. / Leucoencefalopatia posterior reversible de afectacion troncoencefalica y medular aislada.

TITLE: Leucoencefalopatia posterior reversible de afectacion troncoencefalica y medular aislada.