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: Neuroschistosomiasis is a rare but severe complication of schistosomiasis that is often underdiagnosed and can affect both the brain and the spinal cord. CNS involvement occurs during hepato-intestinal or intestinal phase of the disease, when the Schistosoma eggs or adult worms reach the vertebral venous plexuses via Batson plexuses. Objective: To report a case of a patient with conus medullaris syndrome caused by spinal cord schistosomiasis whose symptoms had great improvement after undergoing treatment with praziquantel and prednisone. Case report: A 45-year-old woman, from Bahia, Brazil, with no significant medical history, presented with intense pain in her legs from knees below, associated with progressive loss of strength, tingling and dysesthesias in both lower limbs, causing gait impairment followed by urinary retention. At physical examination, grade III paraparesis in proximal limbs and grade IV in distal limbs and unsteady dysbasic gait were observed, no meningeal signs were found. MRI study revealed hyperintense signal in medulla at the level of T12-L1 to conus medullaris in T2 sequences, mainly on central portions and medulla, also slight impregnation with gadolinium showing dotted pattern was observed, suggestive findings of inflammatory myelitis. KatoKatz test was performed evidencing Schistosoma eggs. After diagnosis, patient was treated with prednisone and praziquantel for 5 weeks. At the end the of treatment, she has showed progressive improvement of her symptoms. In our last evaluation, the patient showed a great recovery of movement and strength, now grade IV in proximal limbs and grade V in distal limbs as well as a steadier gait. Urinary retention is still present, and she still needs intermittent catheterization for the time being Conclusion: Neuroschistosomiasis, when symptomatic, is a severe disorder in which can cause significant incapacity and morbidity. It is an underdiagnosed disorder but has been increasingly reported in populations in endemic areas and in tourists. Prognosis depends largely on early diagnosis and treatment.

Background: Phoenicopterus chilensis is a South American wild bird classified a species near threatened in the National List of Endangered Species of the International Union for Conservation of Nature. With the increase of the contact between human population and wild animals, this species’ habitat is becoming increasingly vulnerable, with a declining population. Due to the importance of its conservation, the knowledge of abnormalities that affect this species becomes essential. This report aims to describe the first diagnosed case of unilateral renal agenesis in chilean-flamingo.Case: A captive male chilean-flamingo (Phoenicopterus chilensis) was found dead in its enclosure and then was referred at necropsy. The cause of death was the presence of tracheal fungal granuloma, caused by Aspergillus sp. Macroscopically, during the examination of the cellomatic cavity, the absence of the right kidney was noted, also evidencing the absence of the caudal renal vein. Emerging from the caudal division of the kidney was noted a blind-end renal vessel (Figure 1). For the histopathological examination, the remaining kidney was fixed in 10% formalin, processed by paraffin embedding technique and stained with Hematoxylin and Eosin (HE). When analyzing the left kidney parenchyma, preserved histological architecture was noted, without any changes in the tissue structures of the organ. Thus, no hypertrophy by compensatory mechanisms of the remaining kidney was observed (Figure 2).Discussion: Unilateral renal agenesis is a very rare congenital defect in dogs, cats and also in birds. It occurs in the complete absence of one of the kidneys, a situation with which the animal can live satisfactorily if there is a normal kidney to assume the functions. In association, ipsilateral ureteral agenesis and compensatory hypertrophy of the remaining kidney may occur. At the time of necropsy, due to the remaining kidney has normal size and within topographic limits, dorsally in contact with the pelvis and sinsacrum, the absence of compensatory hypertrophy was suspected, which was confirmed by histopathological examination. Clinical signs of unilateral renal agenesis, when present, are related to renal failure. The signs develop when the remaining kidney fails to fully absorb the other's functions and fails to maintain the organism's homeostasis. In the case of this report, the specimen showed no clinical signs related to renal agenesis, probably because was no functional compensation for the single kidney. This condition was only noticed after flamingo necropsy, who death because tracheal fungal granuloma, caused by Aspergillus sp. Other reported ways of diagnosing this malformation in animals while alive were imaging and abdominal cavity surgery. The etiopathogenesis of unilateral renal agenesis in animals is uncertain, however, the hereditary cause is commonly described in rats and small animals. It cannot be concluded that the same happened with the animal of this study due to the fact of lack of data regarding the parents and history of animal. It is concluded that in unilateral renal agenesis, the compensatory hypertrophy of the remaining kidney and any clinical symptoms may not be present. Necroscopic and complementary exams are essential to obtain the diagnosis of unilateral renal agenesis. Knowing that the chilean-flamingo is considered as a species near threatened of extinction, it is important to know the abnormalities that affect this species, because this information may be essential for conservation programs.

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