Gustavo Novelino Simão

Extradural arachnoid cysts in children

BackgroundExtradural arachnoid cysts in the spine are uncommon causes of spinal cord compression in the pediatric population that are thought to arise from congenital defects in the dura mater. Most reports describe such cysts as communicating with the intrathecal subarachnoid space through a small defect in the dura. Excision of the cyst with obliteration of the communicating dural defect is the mainstay of treatment in symptomatic patients. Solitary extradural arachnoid cysts have been reported in several studies, but multiple extradural arachnoid cysts are very rarely reported in children.Materials and methodsThe authors report a case of multiple extradural spinal arachnoid cysts in a 14-year-old boy who presented progressive lower extremity weakness, myelopathy, and severe gait ataxia. Magnetic resonance (MR) of the spine demonstrated four extradural arachnoid cysts extending from T-1 to T-9. The patient underwent a thoracic laminoplasty for en bloc resection of the spinal extradural arachnoid cysts. Postoperatively, the patient’s motor strength and ambulation improved immediately.ConclusionsMultiple spinal extradural arachnoid cysts are rarely reported in the literature. Excision of the cysts at the spinal cord level leads to a favorable outcome.

Obstructive Sleep Apnea Is Frequent in Patients with Hypertensive Intracerebral Hemorrhage and Is Related to Perihematoma Edema

Background: Obstructive sleep apnea (OSA) is related to increased systemic inflammation and arterial hypertension. We hypothesize that OSA is frequent in patients with acute hypertensive intracerebral hemorrhage (ICH) and is related to the perihematoma edema. Methods: Thirty-two non-comatose patients with a hypertensive ICH underwent polysomnography in the acute phase. Perihematoma edema volume was measured on CT scans at admission, after 24 h (early control) and after 4–5 days (late control). The Spearman coefficient (rs) was used for correlations. Results: OSA occurred in 19 (59.4%) patients. The apnea-hypopnea index was correlated with relative edema at admission CT (rs = 0.40; p = 0.031), early CT (rs = 0.46; p = 0.011) and at late CT (rs = 0.59; p = 0.006). Conclusions: OSA is highly frequent during the acute phase of hypertensive ICH and is related to perihematoma edema.

Effect of Multiple Cranial Burr Hole Surgery on Prevention of Recurrent Ischemic Attacks in Children with Moyamoya Disease

Moyamoya disease (MMD) is an uncommon cerebrovascular disorder characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. Direct and indirect bypass techniques have been devised with the aim of promoting neoangiogenesis. The current study aimed to investigate the role of multiple cranial burr hole (MCBH) operations in the prevention of cerebral ischemic attacks in children with MMD. Seven children suffering from progressive MMD were submitted to the MCBH and arachnoid opening technique. Ten to 20 burr holes were drilled in the fronto-temporo-parieto-occipital area of each hemisphere in each patient, depending on the site and extent of the disease. All patients were evaluated pre- and postoperatively by means of Barthel index (BI), CT, MR, angio-MR, and angiography. Patients had no recurrence of ischemic attacks postoperatively. Neoangiogenesis was observed in both hemispheres. One patient developed a persistent subdural collection after surgery, thus requiring placement of a subdural-peritoneal shunt. Postoperative BI was statistically significantly improved (P=0.02). This report suggests that MCBH for revascularization in MMD is a simple procedure with a relatively low risk of complications and effective for preventing cerebral ischemic attacks in children. In addition, MCBH may be placed as an adjunct to other treatments for MMD.

X-linked adrenoleukodystrophy in heterozygous female patients: women are not just carriers

UNLABELLED X-linked adrenoleukodystrophy (X-ALD) is a recessive X-linked disorder associated with marked phenotypic variability. Female carriers are commonly thought to be normal or only mildly affected, but their disease still needs to be better described and systematized. OBJECTIVES To review and systematize the clinical features of heterozygous women followed in a Neurogenetics Clinic. METHODS We reviewed the clinical, biochemical, and neuroradiological data of all women known to have X-ADL. RESULTS The nine women identified were classified into three groups: with severe and aggressive diseases; with slowly progressive, spastic paraplegia; and with mildly decreased vibratory sensation, brisk reflexes, and no complaints. Many of these women did not have a known family history of X-ALD. CONCLUSIONS Heterozygous women with X-ADL have a wide spectrum of clinical manifestations, ranging from mild to severe phenotypes.

Intracranial Castleman’s disease presenting as hypopituitarism

Castleman’s disease is an atypical lymphoproliferative disorder that may present as a localized or multicentric form. The involvement of the central nervous system is rare. We describe here a case of Castleman’s disease with involvement of the hypothalamus and meninges, presenting as hypopituitarism. Radiological and clinical pathological features are emphasized and a review of the literature is presented.

Chordoid glioma of the third ventricle.

Background Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle with both glial and chordoid features. It was first described by Brat in 1998. Since there is no detailed information available on the outcome after surgery and adjuvant treatment, we reviewed the literature.

Hemispheric dysplasia and hemimegalencephaly: imaging definitions

Background and PurposeHemispheric dysplasia (HD) and hemimegalencephaly (HME) are both brain malformations with early clinical manifestation including developmental delay and intractable epilepsy. Sometimes the differentiation of these conditions is not simple. HME is an anomaly of cortical development caused by a combination of neural proliferation and cell migration dysfunction, showing lobar or hemispheric enlargement. On the other hand, HD shows no brain hypertrophy, and even brain atrophy, eventually.Patients and MethodsTo compare both conditions, we reviewed clinical, MRI, and histopathology of 23 patients with developmental delay and refractory epilepsy treated with hemispheric surgery.ResultsHistologically, both groups presented polymicrogyria, focal cortical dysplasia, gray matter (GM) heterotopia, pachygyria, and agyria. The white matter (WM) showed different degrees of gliosis and myelin impairment. Even though with no specificity in histopathology, the degree of lesion was more impressive on HME. The combination of WM dysmyelination and hypertrophy leads to the so called hamartomatous appearing. Although not all HME showed brain enlargement and some HD might show no size changes or atrophy, the size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions.ConclusionBrain MRI was the best diagnostic tolls because it allowed together high contrast resolution, whole brain coverage and spatial distribution analysis. HD and HMD showed brain asymmetry tendency, but in opposite directions. The size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions.

Conjugate upward gaze paralysis with unilateral ptosis caused by a unilateral midbrain infarction

A 73-year-old woman with atrial fibrillation presented with a sudden right hemiparesis, with diplopia and left ptosis, and was admitted at an Emergency Unit. The neurological examination found fluctuations on consciousness level, predominant crural right hemiparesis and right central facial paralysis without sensitive abnormalities. The first ophthalmological evaluation showed normal pupillary reflexes, total left ptosis and paresis of adduction of the left eye, with conjugated horizontal palsy for right gaze and conjugated vertical palsy for upward and downward gaze on saccadic and smooth pursuit eye movements. The convergence showed paresis of left eye, with reactive pupils, and oculocephalic test was normal. A head CT had no acute ischaemic signs, and after 4 days, she was discharged. The brain magnetic resonance (MR) performed 15 days after the ictus showed a clearly defined left paramedian tegmental mesencephalic infarct (figure 1). Two months after the stroke, the patient had a remarkable improvement of ocular motility, presenting paresis of levator palpebrae, medial and inferior …

Sellar and parasellar abnormalities

1. Attending Physician in the Neuroradiology Department, University of São Paulo Medical School (FMRP-USP), Radiologist at Cedirp, Ribeirão Preto, SP, Brazil. E-mail: gustavonsimao@gmail.com. The sellar and parasellar regions constitute an anatomically complex area comprising various important neurovascular structures within a small space. The sellar region includes the sella turcica and the pituitary gland, together with the ventral adenohypophysis and dorsal neurohypophysis. The parasellar region encompasses the cavernous sinuses, suprasellar cistern, hypothalamus, and ventral inferior third ventricle. Anatomic localization is essential in the creation of a differential diagnosis between sellar and parasellar lesions. The sellar and parasellar regions can be involved in neoplastic, inflammatory/ granulomatous, infectious, and vascular diseases, any of which can arise from the pituitary gland, infundibular stalk, hypothalamus, cranial nerves, vascular structures, leptomeninges, or skull base(1). There are more than 30 processes that can involve the sellar or parasellar region, the entities most commonly seen in general practice including macroadenoma, microadenoma, empty sella, craniopharyngioma, hypothalamicchiasmatic glioma, and meningioma(1). Radiologic imaging of the pituitary gland and the parasellar region is challenging because of the small size of the pituitary gland and its close proximity to many important structures. With its high contrast, spatial resolution, and multiplanar capabilities, magnetic resonance imaging (MRI) is the modality of choice to study various diseases of the central nervous system(2–5) and can be diagnostic if a process originates from the sellar or parasellar region, as well as characterizing its regional spread. Various MRI sequences have proven to be robust tools for tissue characterization and can determine whether a mass is solid, cystic, hemorrhagic, or fatty, which narrows the differential diagnosis, depending on the location. A standard protocol for MRI of the pituitary gland and parasellar region consists of thin-section (2–3 mm) sagittal and coronal T1-weighted images with and without contrast enhancement. Thin-section T2-weighted imaging can be supplemented to look for cystic lesions. In addition, one T2-weighted scan covering the entire brain should be performed(6). For some indications, such as the detection of microadenoma, dynamic contrast-enhanced imaging of the pituitary gland should be obtained(1). Computed tomography (CT) continues to play a role in the evaluation of bone structures, because it can delineate osseous erosion with great detail and characterize calcified tumor matrices(7). Interpretative imaging strategies for sellar and parasellar lesions are required to make an accurate differential diagnosis. Hess and Dillon(8) include some key considerations on that front: determining the normal imaging appearance of the gland and infundibulum, in terms of size and enhancement pattern; localizing the abnormalities as entirely intrasellar, sellar and suprasellar, or entirely suprasellar; characterizing the lesions as entirely solid, entirely cystic, or mixed solid and cystic; categorizing the lesion margins as circumscribed or invasive; distinguishing imaging features that are unique or highly suggestive of cysts, low T2 signal intensity, calcification, or fluid-fluid levels; and identification of mass effect on the optic apparatus, invasion of the cavernous sinuses, and abnormalities located elsewhere in the brain. The article authored by Eduardo et al.(9) and published in this issue of Radiologia Brasileira makes a significant contribution to the understanding of sellar and parasellar abnormalities. The authors provide an overview of the most relevant MRI and CT characteristics of pituitary tumors, as well as congenital, vascular, inflammatory, and infectious lesions, found in the sellar/parasellar region, in order to increase the accuracy of the differential diagnosis.

Monitoring optic chiasmatic-hypothalamic glioma volumetric changes by MRI in children under clinical surveillance or chemotherapy

PurposeOptic pathway gliomas represent 5% of pediatric brain tumors and are typically low-grade lesions. Because of their unpredictable clinical course, adequate treatment approaches have been controversial, involving surveillance, surgery, chemotherapy, and radiotherapy. In this study, we use volumetric imaging to compare evolution of optic chiasmatic-hypothalamic gliomas (OCHG) treated with and without chemotherapy, analyzing tumor volume variation during the overall period.MethodsA total of 45 brain MRI were retrospectively analyzed for 14 patients with OCHG. Volumetric assessment of the lesions was performed by a neuroradiologist, using software DISPLAY. OCHG patients were allocated into two groups: group 1 (n = 8) who underwent chemotherapy and group 2 (n = 6) who did not receive chemotherapy. Outcome analysis was performed comparing tumor volume evolution of these two groups.ResultsThe results showed a reduction of 4.4% of the volume of the lesions for group 1 after the end of chemotherapy, with an increase of 5.3% in volume in the late follow-up examination. For group 2, we found a slight reduction (5%) of the overall volume of the lesions, both with no statistical significance (p > 0.05).ConclusionsFrom the limited series analyzed in this study, no significant differences were observed in relation to the volume change of lesions treated or not treated with chemotherapy. Larger prospective clinical trials are needed to better evaluate the effect of chemotherapy and radiological response of OCHG.