RODRIGO DE HOLANDA MENDONCA
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/45 - Laboratório de Fisiopatologia Neurocirúrgica, Hospital das Clínicas, Faculdade de Medicina
LIM/45 - Laboratório de Fisiopatologia Neurocirúrgica, Hospital das Clínicas, Faculdade de Medicina
8 resultados
Resultados de Busca
Agora exibindo 1 - 8 de 8
- Desmin-associated myofibrillar myopathy with cap-like structures in the muscle biopsy(2016) SILVA, A.; ESTEPHAN, E.; MORENO, C.; MENDONCA, R.; NISHIMURA, P.; GALINDO, L.; CARVALHO, M.; ABATH-NETO, O.; ZANOTELI, E.
- Brachio-cervical inflammatory myopathy with lymphoid follicle-like structures in a patient with scleroderma(2018) SILVA, A.; VIANNA, M.; MENDONCA, R.; ZANOTELI, E.
- Lumbar catheter placement for nusinersen administration in a SMA 2 patient with spinal deformities and previous spinal surgery(2018) MENDONCA, R.; SILVA, A.; VELASCO, O.; CARDEAL, D.; CONTI-REED, U.; ZANOTELI, E.
- Clinical, histological and radiological responses to methylprednisolone in HIV-associated rod myopathy(2017) SILVA, Andre M. S.; MENDONCA, Rodrigo H.; MORENO, Cristiane A. M.; ESTEPHAN, Eduardo P.; HELITO, Paulo V. P.; CARVALHO, Mary S.; ZANOTELI, EdmarSkeletal muscle involvement as a neurologic manifestation in individuals with HIV is rare, especially as rod myopathy. We describe a 41-year-old male with HIV infection who presented progressive proximal muscle weakness and limb-girdle atrophy. A muscle magnetic resonance image showed bilateral fatty infiltration and post-contrast enhancement in the arm and thigh muscles. The muscle biopsy revealed intracytoplasmic aggregates with appearance of nemaline rod bodies with Gomori trichrome staining and electron microscopy in most fibers. The patient underwent six cycles of intravenous methylprednisolone pulses, presenting clinical improvement. Post-treatment muscle biopsy showed fewer nemaline bodies and muscle magnetic resonance image depicted a pronounced reduction of muscular edema. These findings corroborate that deposition of nemaline bodies in these patients might be related to an immune response triggered by the virus.
- A curable myopathy manifesting as exercise intolerance and respiratory failure(2018) SILVA, A.; MENDONCA, R.; SOARES, D.; CALLEGARO, D.; CALDAS, V.; CARVALHO, M.; ZANOTELI, E.
- Motor unit number index (MUNIX) in children and adults with 5q-spinal muscular atrophy: Variability and clinical correlations(2021) MENDONCA, Rodrigo Holanda; MACHADO, Ligia Maria Sotero; HEISE, Carlos Otto; POLIDO, Graziela Jorge; MATSUI, Ciro; SILVA, Andre Macedo Serafim; REED, Umbertina Conti; ZANOTELI, EdmarSpinal muscular atrophy (SMA) is a motor neuron disease associated with progressive muscle weakness and motor disability. The motor unit number index (MUNIX) is a biomarker used to assess loss of motor units in later-onset SMA patients. Twenty SMA patients (SMA types 3 and 4), aged between 7 and 41 years, were clinically evaluated through the Hammersmith Motor Functional Scale Expanded and the Spinal Muscular Atrophy-Functional Rating Scale. The patients underwent compound motor action potential (CMAP) and MUNIX studies of the right abductor pollicis brevis, abductor digiti minimi and tibialis anterior (TA) muscles. Age-matched healthy controls (n = 20) were enrolled to obtain normative CMAP and MUNIX values from the same muscles. Compared to healthy controls, SMA patients showed significant reductions in MUNIX values among all muscles studied, whereas CMAP showed reductions only in the weaker muscles (abductor digiti minimi and TA). MUNIX variability was significantly higher in the SMA group than in the control group. MUNIX variability in TA correlated with CMAP variability. Motor functional scores correlated with TA MUNIX. The MUNIX study is feasible in later-onset SMA patients, and TA MUNIX values correlate with disease severity in patients with mild motor impairment.
- A common CHRNE mutation (c.130dupG) in Brazilian patients with congenital myasthenic syndrome(2017) ESTEPHAN, E.; SILVA, A.; MENDONCA, R.; CALDAS, V.; ZAMBON, A.; MARCHIORI, P.; HEISE, C.; REED, U.; ZANOTELI, E.
- Nemaline myopathy related to HIV infection with a good response to immunosuppression(2016) SILVA, A.; MENDONCA, R.; ESTEPHAN, E.; MORENO, C.; CARVALHO, M.; ZANOTELI, E.