Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in the world. There are variants. Of the cranial forms, the following stand out: the classic form, facial diplegia with distal paresthesias, pharyngo-cervico-brachial form, polyneuritis cranialis, Miller-Fisher syndrome and Bickerstaff encephalitis. This study aimed to report the case of a 73-year-old male patient, diabetic, former smoker and alcoholic, who presented at the Neurology outpatient clinic of a tertiary hospital in Pernambuco, after emergency care and 19 days of symptoms, reported as sudden cervical weakness, dysarthrophonia, dysphagia and weakness in the right hemiface, three weeks after vaccination (influenza and triple viral) and flu syndrome. He had dyspnea since the onset of the condition, with no progression or fluctuating complaints. The neurological examination showed multiple cranial nerve syndrome (right peripheral pattern facial palsy, reduced elevation of the soft palate and cervical extension paresis) associated with global hypo/arreflexia. Complementary exams showed, in addition to leukocytosis and signs of bronchopathy on chest tomography, cerebrospinal fluid with 00 cells and 48 proteins and electroneuromyography with predominantly sensitive axonal polyneuropathy, decrease in bilateral facial motor amplitude, needle with myopathic pattern. Brain magnetic resonance imaging without alterations. With the possibility of cranial polyradiculoneuritis and a history of dyspnea raised, he was admitted to the intensive care unit to monitor his breathing pattern and dysautonomia. He received antibiotic therapy for seven days due to pneumonia and pulse therapy with IVIG (2 g/kg for five days). He maintained progressive improvement of symptoms. He was discharged with a multidisciplinary outpatient follow-up scheduled. It is concluded, therefore, that the recognition of GBS and variant forms is necessary.
Case presentation: A 70-year-old female was admitted due to imbalance associated to high fever, headache, photophobia, myalgia, and emesis for 5 days. She denied other symptoms or predisposing agents. At examination, there was a global asymmetric cerebellar syndrome, without other findings. Laboratory work-up showed lymphocytosis, leucopenia, thrombocytopenia, and increased liver enzymes. Serologic exams were negative for syphilis, HIV, hepatitis B and C viruses, CMV, rubella, toxoplasmosis, and trypanosomiasis in peripheral blood, and for CMV, herpes simplex and varicella-zoster viruses, and toxoplasmosis in cerebrospinal fluid. Brain MRI with angiography was unremarkable. During hospital stay, there was complete recovery of systemic symptoms and gradual improvement of the cerebellar syndrome. She was discharged for outpatient follow-up, during which a positive IgM (MAC-ELISA) for Dengue virus was identified. First evaluation was 54 days after onset; she had a mild subjective imbalance complaint, with normal neurological exam. She has been followed for four years since onset, remaining asymptomatic, without any cerebellar signs. Discussion: Neurotropism in Dengue fever, as direct viral or immunomediated damage, has been well recognized. Its spectrum comprises many presentations; however, association with cerebellar syndrome is extremely rare, with only seven other cases published. Acute cerebellar ataxia is characterized by normal neuroimaging and complete recovery up to three years. In Dengue cases, recovery happened from one week to two months. Currently, there are no other reports with long-term follow-up. Conclusion: Dengue virus is a rare, but probably underdiagnosed, cause of acute cerebellar ataxia. Recovery is fast, and, in our case, persistent, without recurrence or evolution to any degenerative disease.
Case presentation: A 42-year-old female patient, with no previous comorbidities, complained of tremors and gait disturbance that had started two years before. She mentioned that her legs had fluctuating moving difficulties, associated with pain. At her first evaluation at clinical onset, the case was interpreted as episodic myoclonus, and clonazepam was prescribed with partial improvement. In the initial physical examination, it was observed pathologic glabellar reflex, bilateral hyperreflexia in the lower limbs, and bilateral foot and knee clonus, with no findings in tonus or muscle weakness. The patient was reassessed after one month, showing moderate spasticity in the lower limbs. A variable character of hypertonia was noticed in the following days. Ancillary tests, brain MRI and electroneuromyography was unremarkable. Spinal cord magnetic resonance imaging (MRI) showed hyperintensity in the corticospinal tract, with “owl-eyes” sign. Cerebrospinal fluid (CSF) analysis was unremarkable, except for the presence of oligoclonal bands. Other laboratory findings included serum anti-TPO 1200 U/mL and Anti-GAD65 in CSF 3081UI/ mL. Therefore, the diagnosis of Stiff-Person syndrome was established. The patient was treated with human immunoglobulin with good clinical response. Discussion: Stiff-Person syndrome (SPS) is a rare autoimmune disorder, characterized by insidious, fluctuating, progressive hypertonia. Some case reports have shown signal changes in the spinal cord on MRI, especially associated with amphiphysin autoantibodies. The “owl-eyes” sign is a hypersignal observed in the anterior horns of the spinal cord in the axial MRI T2-weighted imaging, being more commonly described in medullary infarcts and compressive myelopathy. So, this sign is uncommon in SPS. Conclusion: Although rare, Stiff-Person syndrome associated with GAD-65 can be included in the differential diagnosis of myelopathy with the “owl-eyes” sign, if the clinical history is suggestive.
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