Context: X-linked adrenoleukodystrophy (X-ALD) is a rare genetic demyelinating disease caused by mutations in ABC1 gen associated with an impairment of beta- oxidation of very long chain fatty acids (VLCFA) in peroxisomes. It causes accumulation of VCLFA in tissues affecting majoritary the central nervous system, testicles and the adrenal córtex resulting in symptoms which provides restricted neurological prognosis and sequels. Methods: Specific data related of a clinical case through prontuary and complementary exams in a patient attended at Santa Casa de Misericórdia de São Paulo hospital. Case report: Male patient, 39 years old, complaning about vomiting, hyperpigmented skin associated with abolish, psicoses, urinary incontinence, temporal and spacial confusion as well as were found: hyperkalaemia, hyponatremia, hypoglycemia, elevated ACTH levels, basal cortisol decresead, antibody anti-21-hidroxilase non reagente, screening for infectious agents were carried out and infection subsequently ruled out. Were observed in MRI Brain: hypersignal in cerebral white matter on T2-FLAIR sequence bilaterally in which the occipitoparietal region, frontal lobe and basal ganglia were more affected. After metabolic and hydroelectric disorders estabilization using Prednisone, Fludrocortisone per day for 5 days, he evolved with worsening of cognitive and behavioral status until nowdays. Actually, he is totally dependent on his basic activities. Conclusions: It is a rare disease, but it must be recognized by every neurologist, since it is can affect other systems and can leave serious sequelae.

Background: The Three Territory Sign (TTS) is a radiologic marker of ischemic stroke associated with malignant neoplastic diseases (Trousseau Syndrome) and corresponds to a rare stroke etiology. Case Report: Female, 62-year-old patient, with comorbidities of smoking, hypertension and diabetes, presented with a sudden faciobrachial-predominant left hemiparesis settled in the day before the admission. Diagnosed with a metastatic rectal adenocarcinoma seven months before, she underwent a rectosigmoidectomy three months ago and developed deep vein thrombosis, starting anticoagulant therapy with rivaroxaban 20mg daily. A Magnetic Resonance Imaging (MRI) revealed several lesions with restricted diffusion in multiple vascular territories, bilaterally, corresponding to ischemic stroke. Etiologic investigation did not detect signs of cardioembolism, nor significant vessel stenosis or unstable atherosclerotic plaques. In admission, she had a D-dimer level of 11,43μg (0- 0,5μg/mL). Conclusion: The evidence of TTS is about six times more frequent in stroke related to malignancies compared to cardioembolic etiology. The D-dimer is a marker of malignancies in cryptogenic stroke, elevated in 75% of cases. The most common associated neoplasms are pulmonary (40%) and gastrointestinal (33,3%). In the MRI, the lesions can be isolated or gathered, generally small and peripheral. There is no evidence regarding the ideal preventive therapy. It is necessary to reinforce the importance of investigating malignancies in patients presenting with cryptogenic stroke and TTS, a syndrome that is still poorly recognized.