Cerebral Cavernous Malformations: Patient-Reported Outcome Validates Conservative Management.

BACKGROUND: Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels lined by a single layer of endothelium. In contradistinction to arteriovenous malformations, these lesions do not have smooth muscle or elastin in their lining and they are angiographically occult, and the MRI is the most sensitive test for CCM detection. CCM are one of the most prevalent vascular malformations of the central nervous system, affecting about 0.4-0.6% of the general population. The main complication of this malformation is the risk of bleeding, which may cause neurological deficits that affect the quality of life (QoL) in patients. When symtomatic, they may be surgically treated for relieving the mass effect and seizures refractory to drug uses, hemorrhage and drug-refractory epilepsy. Patient-reported outcome (PRO) may be a strategy that can be used to evaluate QoL of CCM population and was used in a sample of non-operated patients. METHODS: An observational, cross-sectional analysis to evaluate the PRO using the SF-36 and EuroQol 5 dimensions (EQ-5D) questionnaires of QoL added to functional metrics using the Karnofsky Performance Status (KPS) in 49 patients not submitted to intervention and with long-term follow-up. RESULTS: During the 364 person-years of follow-up, there was an average of individual follow-up of 7.42 years. The mean age was 46.8 years (18-84) - 57% of them were female, 71% had superficial lesions, and 65% had the familial form. Comparisons of SF-36 dimensions with KPS graded <100 had a worse score only in terms of the pain (p = 0.04), vitality (p = 0.001), and general state of health (p = 0.03) domains. The domain mental health was worse in patients without surgical indication (p = 0.032). The functional capacity domain had the highest overall grading in the group. The EQ-5D dimensions of mobility (p = 0.03) and pain/discomfort (p = 0.001) were the ones with lower score compared to KPS <100. CONCLUSION: The study is the first to evaluate, with validated tools, the PRO of non-operated CCM patients and has demonstrated in a selected group of patients that it was possible to achieve long-term clinical stability, thereby maintaining QoL and functional neurological outcome.

Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management.

Neurocysticercosis (NCC) is an endemic disease and important public health problem in some areas of the World and epilepsy is the most common neurological manifestation. Multiple intracranial lesions, commonly calcified, are seen on cranial computed tomography (CT) in the chronic phase of the disease and considered one of the diagnostic criteria of the diagnosis. Magnetic resonance imaging (MRI) is the test that better depicts the different stages of the intracranial cysts but does not show clearly calcified lesions. Cerebral cavernous malformations (CCM), also known as cerebral cavernomas, are frequent vascular malformations of the brain, better demonstrated by MRI and have also epilepsy as the main form of clinical presentation. When occurring in the familial form, cerebral cavernomas typically present with multiple lesions throughout the brain and, very often, with foci of calcifications in the lesions when submitted to the CT imaging. In the countries, and geographic areas, where NCC is established as an endemic health problem and neuroimaging screening is done by CT scan, it will be important to consider the differential diagnosis between the two diseases due to the differences in adequate management.

Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management / Neurocisticose, cavernoma cerebral familiar e calcificações intracranianas: diagnóstico diferencial e acompanhamento adequado

ABSTRACT Neurocysticercosis (NCC) is an endemic disease and important public health problem in some areas of the World and epilepsy is the most common neurological manifestation. Multiple intracranial lesions, commonly calcified, are seen on cranial computed tomography (CT) in the chronic phase of the disease and considered one of the diagnostic criteria of the diagnosis. Magnetic resonance imaging (MRI) is the test that better depicts the different stages of the intracranial cysts but does not show clearly calcified lesions. Cerebral cavernous malformations (CCM), also known as cerebral cavernomas, are frequent vascular malformations of the brain, better demonstrated by MRI and have also epilepsy as the main form of clinical presentation. When occurring in the familial form, cerebral cavernomas typically present with multiple lesions throughout the brain and, very often, with foci of calcifications in the lesions when submitted to the CT imaging. In the countries, and geographic areas, where NCC is established as an endemic health problem and neuroimaging screening is done by CT scan, it will be important to consider the differential diagnosis between the two diseases due to the differences in adequate management.

RESUMO A neurocisticercose (NCC) é um importante problema endêmico de saúde pública em algumas áreas do mundo, sendo epilepsia sua manifestação clínica mais comum. Múltiplas lesões intracranianas, geralmente com calcificações visualizadas em tomografia computorizada de crânio, são interpretadas como um dos critérios diagnósticos na fase crônica da doença. A ressonância magnética é o melhor teste de imagem para identificar a doença em diferentes estágios de sua forma cística mas apresenta limitações para demonstrar lesões calcificadas. Malformações cavernosas cerebrais, ou cavernomas, são malformações vasculares comuns ao sistema nervoso e epilepsia é também a sua forma mais frequente de apresentação. Na sua forma familiar cavernomas apresentam-se tipicamente com múltiplas lesões encefálicas e, frequentemente, com focos de calcificações na TC. Em alguns países, e determinadas regiões geográficas, onde neurocisticercose é endêmica, a neuroimagem mais usada para diagnóstico é a TC de crânio. Nesse contexto torna-se importante estabelecer bases para o diagnóstico diferencial entre as duas doenças, devido às diferentes formas de acompanhamento e tratamento adequado.

Cavernous Malformation in the Trigeminal Distribution: A Case Report of Aggressive Presentation and Management.

BACKGROUND: Cavernous malformation (CM) is a vascular malformation found in the encephalic parenchyma, spinal cord, nerve roots, and extraneural tissue. CM in the trigeminal distribution is exquisitely uncommon and its biological behavior not completely understood. The clinical picture might be diverse, depending on the affected sector of the trigeminal architecture, and literature debating its pathobiology is scarce. CASE DESCRIPTION: We describe a case of 56-year-old woman who presented with left trigeminal neuralgia and a rapidly growing cavernous malformation of the entire distribution of the fifth nerve. The clinical picture evolved to a progressive gait ataxia and follow-up neuroimaging showed a large intracranial mass leading to a brainstem compression. After microsurgical resection, the mass proved to be a typical CM of the trigeminal root. CONCLUSION: We present an uncommonly aggressive progression of a CM of the trigeminal root, Gasserian ganglion, and cavernous sinus evolving to severe brainstem compression. The documentation of this unique case as well as its management is presented is discussed.