Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with prominent motor symptoms. ALS patients may also manifest frontal behavior and cognitive decline, including impairment in facial emotion recognition. We aimed to investigate whether deficits in emotion recognition are associated with frontal behavior symptoms in ALS. Methods: We enrolled two groups of participants: 1) patients with probable or definite sporadic ALS (n= 21, 11 male/10 female; median age 62 years-old; median disease duration = 3 years) and 2) age and education matched controls (n = 25, 18 male/11 female; median age 61 years-old). The Facial Emotion Recognition Test (FERT) was applied to all participants. Patients underwent the Cambridge Behavior Inventory-Revised (CBI-R) and were classified according to the presence of frontal behavioral symptoms into two groups: ALS with no behavioral symptom (ALSns, n = 9) and ALS with at least one behavioral symptom (ALSbs, n = 12). Results: Apathy and mood symptoms were the most frequent neuropsychiatric symptoms in ALS. ALS patients performed worse than controls in the recognition of sadness (p<0.004). There was no difference between controls and ALSns for all FERT scores, but ALSbs had lower performance than controls in sadness (p<0.003). Conclusion: Emotion recognition deficit may be a marker of frontal behavior in ALS.

O coronavirus diasease 2019 (COVID-19) may be associated with severe neurological complications, among them, encephalitis. Neurological manifestations are assigned to several mechanisms, such as direct viral infection and immunemediated processes. This case series aims to describe patients who presented rhombacephalitis after or during severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Case report 1: Female, 26-year-old, had mild COVID-19 infection, followed by nausea and vomiting, which evolved to headache and vertigo, later associated with dysarthria, dysphagia, diplopia, blurred visual, hypoacusis, postural instability and right hemiparesis. Brain resonance image showed hypersignal in the midbrain, pons and medula oblonga. Normal cerebrospinal fluid. Therapy with methylprednisolone was performed, as the same time with ampicilin therapeutic test, followed by plasmapheresis,all without response. Progressed with difficulty in trunk support, use of nasogastric tube and Cognitive impairment. After neurological decline and septic shock, he evolved to death. Case report 2: Female, with suspected psychiatric diagnosis, 27-yearold, interned due to left peripheral facial paralysis associated with right hemiparesis, diplopia, ataxia, dysarthria, strabismus and fever. Positive for COVID-19. Brain resonance with lesions in the cerebellum, midbrain, bridge and white matter of the cerebral hemispheres, some with contrast uptake. Normal cerebrospinal fluid. Performed empirical treatment with ampicillin and acyclovir, in addition to therapy with methylprednisolone, partial improvement. In eleven months, it evolved with worsening of symptoms, associated with seizures, convulsive status and coma. Repeated corticosteroids, followed by immunoglobulin and plasmapheresis. She was discharged with severe neurological sequelae and is still in rehabilitation. This is an extremely relevant and current topic, considering the world scenario of COVID-19 and the lack of evidence for the treatment of possible neurological conditions related to SARS-CoV-2 infection.

Introduction: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare monoclonal lambda plasma cell disorder, with multisystem presentation leading to a challenging diagnosis. The aim is to report a case of a rare hematologic disease, with neurologic manifestation widely seen in daily practice. Case report: Male, 35-year-old, with a history of distal weakness of the lower limbs, symmetrical, subacute onset and progressive worsening, associated with ascending numbness, lower back pain and fatigue. Neurological examination showed motor deficit in extremities (plantar flexion and extension), hypoactive reflexes of lower limbs, in addition to distal sensory deficit (tactile and painful). Electroneuromyography confirmed axonal sensory-motor neuropathy in both lower limbs. Biochemical testing revealed a monoclonal gammaglobulin peak with a predominance of lambda chains and an elevated serum vascular endothelial growth factor (VEGF) concentration. Magnetic resonance imaging detected sclerotic lesions in vertebral bodies of thoracic and lumbar spine. Furthermore, patient also had mild pigmentary changes in the skin of the extremities of lower limbs and polycythemia. Accordingly with the evaluation was possible to make POEMS diagnosis and patient is currently in preparation for an autologous bone marrow transplant. Conclusion: Polineuropathy is a neurological hallmark of the disease and should lead to evaluation for POEMS, when associated with a monoclonal protein detected. Elevated serum VEGF can be a biomarker that supports the diagnosis. In summary, early recognition is essential, because enables adequate treatment, which contributes to stop progressive worsening of symptoms, especially debilitating neurologic ones.