ObjectiveWe report a case of biopsy-proven giant cell arteritis after an initial presentation of area postrema syndrome.MethodsA 65-year-old man was evaluated using MRI, temporal artery biopsy, and ultrasound.ResultsThe patient presented with refractory nausea, vomiting, and hiccups that caused weight loss without any other neurologic or clinical symptoms. His MRI scan 15 days later revealed a hyperintense sign on the area postrema with no abnormal diffusion or contrast enhancement, compatible with isolated area postrema syndrome. An extensive workup for inflammation and other etiologies including neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody disorder, and multiple sclerosis (MS) showed negative results. The patient responded to treatment with methylprednisolone. Two months after the initial clinical manifestation, the patient developed fatigue, headache, and scalp tenderness. He was diagnosed with giant cell arteritis after ultrasonography and biopsy were performed. He responded well to oral glucocorticoids and had only 1 relapse during tapering. He has not had arteritic ischemic optic neuropathy or any new episodes of area postrema syndrome.DiscussionThis case demonstrates the importance of expanding the differential diagnosis in patients with area postrema syndrome and no other signs of NMOSD.
Introduction: The investigation of hemorrhagic demyelinating cerebral lesions is challenging with diverse etiologies. We report a case of varicella zoster virus (VZV) infection with prominent hemorrhagic demyelinating component and systemic thrombosis. Objectives: Describe a case of hemorrhagic demyelinating lesions and systemic thrombosis secondary to varicella zoster central nervous system infection. Case report: A 45-year-old man presented with a one-week history of left leg strength loss associated with pyramidal liberation on the left side. Brain MRI showed two expansive hemorrhagic lesions, in the frontoparietal lobe and in the cingulate gyrus region. There were T2/FLAIR hypersignal in the subcortical area of both cerebral hemispheres and in the cervical cord, suggestive of demyelination. His arterial magnetic resonance imaging with vessel wall imaging showed focal tapering on the pial branches of the middle cerebral artery, but his digital angiography showed no abnormalities. The lumbar puncture had 8 cells, (lymphocyte predominance), 52 proteins/ dL, and 53 mg/dL of glucose. In two days, he developed a left side hemiplegia associated with seizures and pulmonary thromboembolism, demanding lung mechanical thrombectomy and anticoagulation. He had a positive lupus anticoagulant and a positive VZV IgG in the cerebrospinal fluid associated with positive oligoclonal bands. He received intravenous acyclovir with complete resolution of the symptoms. Conclusion: The clinical manifestations of VZV infection are wide, and we should suspect of this infection in patients with central nervous system demyelinating lesions, even without other skin lesions.
Introduction: We present two case reports of rare and aggressive presentation of multiple myeloma, one of the most common hematological malignancies. To our knowledge, there are very few case reports of meningeal invasion by multiple myeloma, denoting the importance of describing the clinical features and the complementary investigation necessary to develop a better approach to these patients. Cases presentation: In case 1, we present a 64 years old patient with vague symptoms of headache associated with exacerbated tendinous reflexes and previous diagnosis of multiple myeloma. In case 2, a 58 years old presents with auricular plenitude, headache and encephalopathy, and a medical antecedent of multiple myeloma. Both were diagnosed with different sites of multiple myeloma central nervous system invasion, case 1 with predominant meningeal infiltration and case 2 with intraparenchymal infiltration. Conclusion: Analyzing these cases, we conclude that besides the rarity of the meningeal accometiment in myeloma patients compared to other hematological disorders, the identification of clinical signals and symptoms suggestive of this invasion is necessary to provide better health care to these patients.
Introduction: Bickerstaff encephalitis is a rare disease and considered a spectrum of the Guillain Barré syndrome. We present a case report of subacute onset of gait impairment, impaired consciousness, ophthalmoplegia, respiratory dysfunction and severe dysautonomia with atypical and diffuse brainstem and diencephalic white matter abnormalities, a positive seric GQ1B and neurological recovery after two cycles of immunoglobulin and plasmapheresis. The aim is to describe an atypical case of Bickerstaff encephalitis. Case report: A 30-year-old man was admitted with a ten days history of vertigo, dysphagia, bilateral ptosis and tetraparesis with brachial predominance. His examination showed appendicular ataxia, tetraparesis, ophthalmoplegia and ptosis. Due to the clinical presentation, we started treatment with immunoglobulin but he evolved with need of mechanical ventilation and had two cardiac arrests due to severe dysautonomia. His lumbar puncture showed no albuminocytologic dissociation. His magnetic resonance imaging showed nonspecific foci signal hyperintensities on FLAIR (Fluid-Attenuated Inversion Recovery) in cerebellum, hippocampus and globus pallidus. His serum GQ1B testing was positive, confirming the initial clinical hypothesis of Bickerstaff encephalitis. He was submitted to plasmapheresis and a second cycle of immunoglobulin, with complete clinical recovery six months after. Conclusion: We described a case of atypical Bickerstaff encephalitis, highlighting the importance of recognizing atypical clinical patterns for an appropriate treatment.
Case report: A 62-year-old patient was transferred from another hospital with complaints of subacute torpor and fever two months after an allogeneic bone marrow transplant. He had the diagnosis of chronic myelomonocytic leukemia type 2 four years before the beginning of the neurological symptoms. The disease remained stable for two years after the diagnosis, but progressed and he was submitted to a bone marrow transplant. The complications during the procedure were a febrile neutropenia, that resolved with a cycle of antibiotics, and a reactivation of cytomegalovirus (CMV) infection (he had a high risk to reactivation due to the status of antibodies of the donator, that was negative, and he had positive antibodies). He remained stable and was discharged from the hospital 30 days after the transplant. Two months after the transplant, the fever and torpor began, and he went to the emergency department in his hometown. There, he was diagnosed with a positive coronavirus disease 2019 (COVID-19) infection and reactivation of cytomegalovirus, with prescription of endovenous ganciclovir, without clinical improvement. His clinical symptoms evolved to loss of balance and progressive torpor, and was transferred to our hospital for clinical evaluation. In the initial examination, he was febrile, torporous, he was not collaborative to the neurological examination due to the level of consciousness. He was hypotonic and had withdrawal from a painful stimulus on four limbs. His tendinous reflexes were hyperactive and symmetrical. Due to his level of consciousness, the cerebellar maneuvers were not testable. He was then submitted to orotracheal intubation due to neurological deterioration. In his complementary investigation, the serum polymerase chain reaction (PCR) for CMV was already negative and his PCR for COVID-19 was also negative. The electroencephalogram showed slow generalized periodic discharges. His computerized tomography showed no abnormalities, so he was submitted to a lumbar puncture that showed 8 cells, with 97% lymphocytes, 74 mg/dL of protein, 26 mg/dL of glucose with no neoplasic cells and a positive PCR for Toxoplasma gondii. The PCR for mycobacterium tuberculosis, cryptococcosis, and viruses were negative. After the results, we started sulfadiazine and pyrimethamine and he was submitted to a magnetic resonance imaging that showed multiple nodular foci of hypersignal in T2/FLAIR (T2-weighted-Fluid-Attenuated Inversion Recovery) and diffusion abnormalities on the cortical surface of the cerebellar hemispheres, thalamus, basal ganglia and subcortical white matter of the cerebral hemispheres, being more evident in the cerebellum and basal ganglia. Besides the atypical radiological findings for neurotoxoplasmosis, his serologic testing on the serum showed an positive immunoglobulin M and immunoglobulin G for Toxoplasma gondii. He had no clinical improvement with the antibiotics, and so, ten days after he was submitted to a new magnetic resonance imaging showing increase in the size and number of multiple small focal lesions with hypersignal on T2/FLAIR in the brain parenchyma, highlighting confluent lesions in the basal ganglia and focal lesions in the cerebellar hemispheres, which show contrast uptake, as well as in the thalamus and midbrain. His neurological symptoms evolved and he showed a minimally conscious state, with spasticity in the four limbs. He was submitted to a new lumbar puncture that showed 1 cell, 78 mg/dL of protein and 50 mg/dl of glucose, an improvement, despite clinical and radiological worsening. Because of the clinical worsening, he was submitted to a brain biopsy. His cerebral biopsy was negative for neoplasic infiltration of the cerebral nervous system, viral infections, cryptococcus and toxoplasmosis. It showed an interstitial CD3 lymphocytic inflammation with vasculitis and frequent macrophages, very rare CD20 lymphocytes and p24 and SV-40 negative. His neuropathological criteria were compatible with CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids). The patient was submitted to five days of metilprednisolone and with oral prednisone after, with slow clinical improvement, but probably due to the lack of systemic clinical status of the patient and delayed diagnosis because of the atypical presentation, the patient died of pneumonia.
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