A 17-year-old man with normal blood pressure presented with acute bilateral blindness, and retro-orbital pain two days after treatment with tocilizumab (TCZ) for juvenile idiopathic arthritis. The diagnosis was posterior reversible encephalopathy syndrome (PRES), made after clinical examination and MRI (Figure). Tocilizumab was discontinued and the patient partially improved. To the best of our knowledge, there are no reports of this association (PRES and TCZ) in PubMed. This manuscript describes a new association between TCZ and PRES based on imaging findings, in which the patient presented with more severe imaging findings and did not have complete recovery of the symptoms 1,2,3 .
Introduction: Guillain-Barré syndrome is an acute/subacute set of clinical features of immune mediated polyradiculoneuropathy, typically after respiratory or gastrointestinal viral infection. In this scenario, we described a case of the syndrome mentioned after infection by sars-cov-2 virus. Objectives and Methods: Description of a clinical case after analysis of medical history and complementary exams, in addition to literature review. Design and Setting: Case report, type of descriptive study, developed in the Institute of Medical Assistance to the State Public Servant of São Paulo. Results: A 68-year-old female patient with clinical and laboratory diagnosis of coronavirus infection, hospitalized in need of oxygen supplementation, which evolved after twelve days of symptoms with hypoesthesia on legs and feet and progressively ascending and symmetrical flaccid paraparesis that led to tetraparesis. Neurological examination showed tetraparesis (muscle strength: grade III in MMSS and grade II in MMII), hyporeflexia in MMSS and reflexes abolished in the lower limbs, plantar skin reflex in flexion in both feet, preserved facial mimicry. About complementary tests, it presented cerebrospinal fluid with albuminocytologic dissociation (cell: 1, protein: 89, glucose: 86), RT-PCR for sars-cov-2 research in cerebrospinal fluid, inconclusive, in addition to four-limb electroneuromyography performed after 19 days of onset of neurological condition, indicated polyradiculoneuropathy with involvement of sensory and motor fibers, primarily demyering. Treatment with human immunoglobulin 400 mg/kg/day for 05 days was started. The reported patient was dismissed from the hospital with significant improvement, presenting muscle strength: grade V in MMSS and grade IV in MMII and already with the ability to walk. Conclusions: the case describes a classic neurological complication associated with a virus that was once non-circulating, but currently with a big clinical relevance.
Context: Syphilis is a pleomorphic, insidious disease and an important differential diagnosis of ocular and CNS involvement. Its recognition and treatment are extremely important, given the high morbidity of its natural history. Case report: A 79-years-old woman started bilateral, intermittent and progressive visual turbidity, evolving in 5 months with pain on eye movement and intense throbbing bilateral headache, worse on the right, plus photophobia, and poor painkillers response. After 3 months, she presented fleeting amaurosis and was admitted to our service. On examination: severe low visual acuity, relative afferent pupillary defect, red desaturation and papilla edema. In CSF: hyperproteinorrachia and negative VDRL. Prednisone 60mg/day was started due to papillitis. Blood analysis showed 1/8 reagent VDRL, with other serologies, tumor and rheumatology markers negatives. She received crystalline Penicillin for 14 days, obtaining remission of headache, papilla edema and improved visual acuity. After 12 days, the visual acuity worsened, so Penicillin was extended to 21 days with 7g of methylprednisolone. After 3 days, the patient recovers the visual acuity she had before. Conclusions: The present study describes neuro-ophthalmological manifestation of syphilis in an immunocompetent individual. Although there is still controversy in the literature, in this case, high dose short-term corticoids was chosen, due to the severity of the loss of visual acuity, obtaining a favorable therapeutic response.
Context: Vogt-Koyanagi-Harada syndrome (VKH) is a rare, multisystemic, autoimmune disease mediated by a Th1 response against melanocytes in the eye, inner ear, central nervous system, skin and hair. In this article, we report a case of VKH with severe visual impairment and discuss the therapeutic response to corticotherapy followed by the use of Adalimumab, a tumor necrosis factor (TNFα) inhibitor. Case report: A 61-year-old black woman started bilateral frontal headache of severe intensity, associated with bilateral eye pain, hyperemia and watery eyes, progressing with visual turbidity with gradual worsening, seeing only figures after eight days. After ten days bilateral hypoacusis started, also progressive. She denied eye movement pain, diplopy, dizziness, fever, joint pain or skin injuries. On examination, visual acuity (VA) in RE: hand movement for 30 cm, LE: light perception, fundus of the eye with serous bilateral retinal detachment. CSF with 155 lymphomonocyte predominance cells, proteins: 73, negative bacterioscopy and cultures. Pulsotherapy was performed for 7 days followed by 1g of cyclophosphamide and maintenance therapy with fortnightly Adalimumab. Two months after discharge, she presented VA in RE: 20/200 and LE: counting fingers at 1 meter. Conclusions: Aggressive and early treatment with immunosuppression is key to the effective treatment of VKH. Immunotherapy can be used in patients who are unresponsive to corticosteroid doses. Biological agents that target TNFα have effective results in non-infectious uveitis. Adalimumab is a safe and effective option, which also reduces the need for chronic corticosteroid therapy. The prognosis depends on the early diagnosis and treatment.
Context: PML is a dymyeliniating disease of the brain, caused by JC virus infection reactivation in immunocompromised patients, especially by AIDS, hematological disease and immunosuppressive therapies. Case report: A 67-year-old woman was diagnosed with multiple myeloma (MM) in 2018 and use of bortezomib/cyclophosphamide/dexamethasone and thalidomide was ineffective. She underwent treatment with monthly daratumumab starting in January 2020. After one year, she experienced progressive amnesia, apathy and confusion. At admission, examination revealed apathy, monosyllabic communication and frontal release, progressing to mutism and abulia. T2 FLAIR-weighted MRI of the brain performed in March 2021 showed a hyperintense non-enhancing lesion affecting thalamus, internal capsule, lentiform and deep white matter of left lobes. MRI performed one month before symptoms onset showed a small lesion in subinsular region – indicating incipient involvement. Cerebrospinal fluid PCR was positive for JC viruses, and PML was diagnosed. Conclusions: This report proves that concomitant hematologic and drug- immunocompromised patients presenting with neurological symptoms should be investigated for PML. There are few reports in the literature of PML occurring in MM, especially after use of daratumumab, an anti-CD38 monoclonal antibody. Recently, one small case series demonstrated some improvement in pembrolizumab (a checkpoint inhibitor)-treated PML, but no routinely therapy is recommended. Understanding severity of both disease, patient was discharged receiving conservative treatment.
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