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Aneurysmal dissection of the internal carotid artery in the cavernous segment is an uncommon lesion, as well as the association with subarachnoid hemorrhage. Its description is related to direct or indirect trauma of the neck region. We report on a 26 year-old male patient who presented in the imediate postoperative after an orthognathic surgery with paralysis the right ocular cranial nerves (IIIº °, IVº ° and VIº°) with ophthalmoplegia, without chemosis or proptosis and with subarachnoid hemorrhage in the cranial tomography. Angiography disclosed a dissecting aneurysm of the right internal carotid artery in the cavernous segment, presenting with rebleeding during his evolution. The therapeutic option was arterial embolization with platinum microcoils. After extensive literature review, endovascular treatment was suggested.

Objectives: Symptomatic intracranial atheromatous disease has an unfavorable prognosis. The endovascular treatment with percutaneous transluminal angioplasty (PTA) stent assisted must be held in accordance with the efficacy, safety, complications and the risk of restenosis in the long term. Materials and methods: A study conducted between 1996 and 2008, includes 28 patients with 30 cases of stenosis located in the internal carotid artery (11), basilar artery (14) and vertebral artery (5). All cases were symptomatic even under anticoagulation treatment and the stenosis grade > 60% (average 83.5%). Results: There was a significant reduction in the degree of stenosis (less than 50%), with an average residual stenosis of 36.8%. There were two complications, with reperfusion bruising (6.6%). In the long term monitoring, we found a single case of restenosis and no patient had a transient or permanent ischemic stroke. Conclusion: The treatment of intracranial stenosis with stentassisted angioplasty is effective, with a low complication rate and restenosis in this series.

Intracranial circumferential fusiform aneurysms of the posterior circulation involving arterial branches or perforating vessels are difficult to treat. This article shows an endovascular reconstruction technique not yet described, using a telescoping self-expandable stent (LEO+) and flow-diverter device (SILK) at different surgical times. Two patients with circumferential fusiform aneurysm, one being an aneurysm of the segments P2 and P3 of the posterior cerebral artery, diagnosed after a headache, and the other a partially thrombosed aneurysm of the lower basilar artery, diagnosed following ischemia of the brain stem. Endovascular treatment was performed by means of a vascular reconstruction technique that used at different surgical times: overlapping; a telescoped self-expandable stent, LEO+; and a flow-diverter device, SILK. Angiographic control was carried out at 6 and 12 months, to evaluate arterial patency, flow maintenance in the arterial branches and perforating vessels, and thrombosis of the aneurysm. The combined use at different surgical times of the self-expandable stent and flow-diverter device was technically successful in both patients. There were no complications during the procedure, nor in the long-term follow-up with full arterial vascular reconstruction, maintenance of cerebral perfusion and complete aneurysm occlusion at the 6- and 12-month angiographic follow-up. There was no aneurysm recanalization nor intra-stent stenosis. Circumferential fusiform aneurysm of the posterior circulation involving arterial branches or perforating vessels to the brain stem may be treated with this arterial reconstruction technique at different surgical times, using the self-expandable stent called LEO+ and the flow-diverter device SILK, minimizing the risk of complications and failure of the endovascular technique, with the potential for arterial reconstruction with thrombosis of the aneurysmatic sac, as well as flow maintenance in the eloquent arteries, in this type of cerebral aneurysm.

The authors have constructed a brief timeline of major clinical research related to hereditary spastic paraplegia (HSP). This timeline summarizes the evolution of HSP research, from the first clinical descriptions by Adolf von Strümpell in 1880 to the present day, with the transformation of these diseases into a rapidly-growing and heterogeneous group of neurogenetic diseases. RESUMO Os autores constroem uma breve linha do tempo com as principais pesquisas clinicas relacionadas as paraplegias espásticas hereditárias. Desde a descrição clínica inicial em 1880, feita por Adolf von Strümpell, até os dias atuais com a transformação dessas doenças em um grupo de doenças neurogenéticas com grande variabilidade na apresentação fenotípica e genotípica.

Purpose Cerebral venous sinus thrombosis (CVST) is an unusual and potentially life-threatening condition with variable and nonspecific clinical symptoms and high morbimortality rates. Standard therapy consists of systemic anticoagulation; although there is no clear evidence about the best choice for treatment, intravenous heparin is used as the first-line treatment modality. Intravenous sinus thrombolysis can be an effective and relatively safe treatment for acutely deteriorating patients who have not responded to conventional therapy. This case report presents the possibility of endovascular treatment in multiple steps with mechanical thrombolysis with balloon, local pharmacological thrombolysis and stenting, in a patient with a severe form of CVST. Case summary A 67-year-old woman presented severe headache, agitation and confusion with diagnosis of venous sinus dural thrombosis in both lateral sinus and torcula. After 24 h there was neurological worsening evolving with seizures and numbness even after starting heparin, without sinus recanalization; CT scan showed left temporal intracerebral hemorrhage. We decided to take an endovascular approach in multiple steps. The first step was mechanical static thrombolysis with balloon; the second step was dynamic mechanical thrombolysis with a balloon partially deflated and “pulled”; the third step was local thrombolysis with Actilyse™; finally, the fourth step was angioplasty and reconstruction of the sinuses using multiple carotid stents and complete angiographic recanalization of both sinuses and torcula. After 24 h of endovascular treatment there was full clinical recovery and no tomographic complications. Conclusion This result shows that mechanical clot disruption, intrasinus thrombolysis and reconstruction of wall sinuses with stenting can be an endovascular option in the severe form of CVST with intracerebral hemorrhage and rapid worsening of neurological symptoms. Although this type of treatment can re-channel the occluded sinuses, further comparative and randomized studies are needed to clarify its efficacy versus other therapeutic modalities.

The endovascular treatment of spontaneous dural cavernous sinus fistula (DAVF) can be accomplished by arterial approach, just with symptoms relief, or by numerous venous approaches through the inferior petrosal sinus, ophthalmic vein, anterior or posterior intercavernous sinus and facial vein. Our case suggests the approach to the cavernous sinus via the foramen ovale and emissary veins puncture as an alternative when there is no possibility of venous approach conventionally described. A 76-year-old woman presented with right conjunctival hyperemia, exophthalmos, intraocular pressure increasing and visual deficits in a period of six months. Angiographic diagnosis of spontaneous DAVF isolated from the cavernous sinus, Barrow Type C, with exclusive venous drainage through the superior ophthalmic vein. Endovascular treatment was performed under general anesthesia. Attempts to approach the cavernous sinus through the inferior petrosal sinus ipsilateral and contralateral intercavernous, facial vein and pterygoid plexus, as well as by dissection and direct puncture of the superior ophthalmic vein were not possible. An approach to the cavernous sinus was performed by puncturing the foramen ovale, catheterization of the emissary vein of the foramen ovale with occlusion of the fistula with microcoils. There was a symptomatic regression with gradual normalization of intraocular pressure, exophthalmos and conjunctival hyperemia in three months. The approach to the cavernous sinus through the foramen ovale and catheterization of the emissary cranial skull base vein is an exception and should be considered in cases of spontaneous and isolated DAVF not accessible by a conventional approach.

Ten percent of all strokes are due to spontaneous cerebral hemorrhages. They are associated to drugs (licit and illicit) in 9.5% of all cases in young adults. This is a case report of a 44-year-old man, without previous morbidities, who presented a sudden onset headache and arterial hypertension 24 hours after use of naphazoline as nasal decongestant. Cranial tomography showed right thalamus hemorrhage. Cerebral angiography showed no aneurisms, vascular malformations or vasculitis. No other risk factors were found during investigation in this patient and the stroke was attributed to naphazoline exposition. Dez por cento de todos os eventos vasculares encefálicos são devido às hemorragias intracerebrais espontâneas, associados a drogas (lícitas e ilícitas) em 9,5% de todos os casos em adultos jovens. Relatamos o caso de um homem de 44 anos de idade, sem doenças prévias, que apresentou cefaléia súbita e hipertensão arterial 24 horas após o uso de congestionante nasal contendo nafazolina. A tomografia de crânio evidenciou hemorragia talâmica. Durante a investigação não foram encontrados outros fatores de risco e a hemorragia foi atribuída à exposição à nafazolina.

Several drugs and toxic substances can cause muscular abnormalities and are frequent causes of acquired myopathies. We present a series of 32 patients, predominance of young adult patients, diagnosed with toxic myopathy. The most common substances inducing myopathy were corticosteroids (56.2%) followed by the propoxyphene, neuroleptics, zidovudine and drug-induced hypokalemia. The investigation showed normal serum creatine kinase levels in 65.4%, myopathic pattern of the needle electromyography in 40% and the more frequent histological diagnosis of the muscle biopsy was type 2 fiber atrophy (59.3%). Clinical features, etiology, course of the disease, serum levels of muscular enzymes, electromyographic features and, especially, muscle biopsy features are discussed.

Cerebrotendinous xanthomatosis is a treatable rare autossomal recessive disease characterized by lipid storage secondary to a sterol 27-hydroxylase deficiency in the formation of cholic and chenodeoxycholic acids. We describe two Brazilian brothers with cognitive impairement and chronic diarrhea. One of them also presents bilateral cataracts. Neurological findings were progressive walking deficit, limb ataxia and pyramidal signs. Both patients had bilateral Achilles tendon xanthomata. Magnetic resonance image showed signal alterations in cerebellar hemispheres. We describe these cases with molecular genetic analysis confirming diagnosis and comparing with previous literature. The CYP27A1 gene study showed a C1187T mutation on exon 6. Xantomatose cerebrotendínea é doença autossômica recessiva tratável causada pelo acúmulo de lipídeos por deficiência da enzima 27-esterol hidroxilase na produção de ácido cólico e deoxicólico. Descrevemos dois irmãos brasileiros com dificuldade cognitiva e diarréia crônica. Um deles apresentava catarata bilateral. Os achados neurológicos foram dificuldade progressiva para deambular, ataxia de membros e sinais piramidais. Ambos tinham xantomas de tendão aquileu bilateralmente. O exame de ressonância magnética revelou áreas de sinal hiperintenso em ambos os hemisférios cerebelares. Descrevemos os casos com diagnóstico genético comparando-os com a literatura. O estudo do gene CYP27A1 demonstrou a mutação C1183T no exon 6.

Lipoid proteinosis (LP) is an autosomal recessive disease that typically presents with papular, verrucous, poxlike, or acneiform scars and lesions and hoarseness. LP was recently mapped to the 1q21 locus and shown to result from mutations in the extracellular matrix protein 1 gene (ECM1). Epilepsy, mental retardation, and hippocampal calcifications can occur. The authors describe a patient with generalized dystonia caused by striatal calcifications.

A actinomicose do sistema nervoso central (SNC) é afecção rara, caracterizada pela formação de abscesso único e de evolução crônica. Os autores descrevem o caso de um paciente jovem, imunocompetente, com actinomicose cerebral oriunda de foco primário cervicofacial, submetido a ressecção cirúrgica e antibioticoterapia. Ressalta-se a importância do conhecimento da entidade e de suas principais formas de acometimento, como a base do diagnóstico precoce dessa enfermidade potencialmente curável e de bom prognóstico. Central nervous system actinomycosis is a rare but treatable chronic suppurative bacterial infection. The case of a young immunocompetent male with actinomycosis of the CNS is presented. The abscess originated from a primary cervico-facial infection and was located in the left parasellar region. After excision of the mass, that showed Actinomyces colonies, the patient was treated with intravenous Penicillin for 42 days followed by oral administration of the drug for 30 days. After surgery the patient was left with mild sequelae that had improved by the last follow-up, 7 months later. A new CT scan at that time revealed no residual disease or recurrence. The early diagnosis of cerebral actinomycosis relies essentially on a clinical suspicion. Hence it is imperative to be aware of the natural history of this infection and its various modes of presentation.