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Volumen 4, Asunto 5 (2016)

Reporte de un caso

Successfully Treated Waldenstrom Macroglobulinemia with Nine-year Follow-up: Case Report and Review of Neurologic Manifestations

Raymond L Rosales, Melanie Leigh D Supnet, James Daniel H Villanueva and Laurence Adlai Morillo

Waldenström’s Macroglobulinemia (WM) is a rare disease, accounting for approximately 6% of all B cell lymphoproliferative disorders, with clinical features that are due to either infiltration of neoplastic cells or properties of the circulating IgM. This case report documents a Filipino-Chinese woman who had constellation of anemia, thrombocytopenia, cranial and spinal involvement, and sensory-motor polyneuropathy. Further evaluation showed clear pattern of WM demonstrated by IgM monoclonal gammopathy, presence of plasmacytoid cells on bone marrow aspirate and biopsy, and presence of B-cell markers on flow cytometry. Regimen of dexamethasone, rituximab and cyclophosphamide was given every 21 days for six courses. Follow-up studies showed negative results in serum protein and IgG electrophoresis. Repeat electrodiagnostic studies showed improved demyelinating neuropathies of the affected nerves and repeat neuroimaging showed reduction in size of the previously noted intracranial nodules and clearing of the spinal cord lesions. Appropriate treatment and follow up is very important in treating patients with WM. This report documents a serially monitored patient who remained in remission for nine years after prompt initiation of treatment. A review focusing on the neurologic sequel of WM was then performed.

Comentario

The Current Role of Non-invasive Treatments in Traumatic Brain Injury

Joao Gustavo Rocha Peixoto dos Santos, Wellingson Silva Paiva and Manoel Jacobsen Teixeira

Due to the morbidity associated with traumatic brain injury (TBI), it is essential to have a rehabilitation plan, preferably multi-professional, to obtain the maximum degree of recovery of neurological and neuropsychological functions. Three types of non-invasive treatment techniques have been studied in patients with TBI and shown promising: Repetitive Transcranial Magnetic Stimulation (rTMS), the transcranial direct current stimulation (tDCS), and transcranial Led Therapy. The displayed data of this article implies that these non-invasive techniques can be used as an effective therapeutic approach to increase brain function in neurocognitive disorders and are a promising treatment for traumatic brain injury patients.

Reporte de un caso

Subarachnoid Haemorrhage after Stereotactic Catheterization of Intracerebral Hematoma

Seong Hoon Jeong and Hyo Joon Kim

The authors report the complication that occurred after stereotactic catheterization in a 71-year-old patient with a spontaneous intracerebral hematoma (ICH). After stereotactic catheterization, a subarachnoid haemorrhage (SAH) was found in the post-operative CT scan. An SAH is a rare complication related with stereotactic catheterization. We investigated the cause and reported about a newly occurring SAH after stereotactic catheterization.

Artículo de investigación

H.P. Acthar® Gel in Dermatomyositis and Polymyositis Treatment Registry: An Interim Analysis

Todd Levine, Justin Malone, Petros Efthimiou, Rup Tandan, Ara Dikranian, Aidan Levine and David Saperstein

Introduction: Therapies for dermatomyositis and polymyositis (DM/PM) include corticosteroids, immunosuppressants, and intravenous immunoglobulin (IVIg). A high proportion of patients with DM/PM are refractory to therapy. H.P. Acthar® Gel, repository corticotropin injection (RCI) is a potential anti-inflammatory treatment.

Methods: Patients enrolled in the Acthar in Dermatomyositis and Polymyositis Treatment registry were monitored for changes in the clinical parameters of DM/PM after initiation of RCI twice weekly in doses up to 80 IU per subcutaneous injection.

Results: RCI treatment effectively altered the clinical course of DM/PM in 14 of 24 patients. Positive responses to treatment were associated with disease activity at baseline and duration of treatment. Mild to moderate adverse events were reported.

Conclusions: This is the largest observational study of RCI in treatment of DM/PM to date and results indicate that RCI may be an effective, tolerable treatment for refractory patients. Controlled studies are necessary to identify any additional associations between disease state and response to RCI treatment.

Reporte de un caso

Gastrointestinal Leiomyosarcoma with Disease Progression to the Central Nervous System: Case Report

Joao Gustavo Rocha Peixoto dos Santos, Iuri Santana Neville,Luiz Guilherme Cernaglia Aureliano Lima, Guilherme Alves Lepski and Manoel Jacobsen Teixeira

Introduction: Leiomyosarcomas (LMS) are rare malignant neoplasms with smooth muscle differentiation, which occur more commonly in the uterus, digestive tract or retroperitoneum. Before 2000, the mesenchymal gastric tumors were divided in leiomyomas, leiomyoblastomas and leiomyosarcomas. After discovery of the role of KIT oncogene, a distinct entity in the mesenchymal gastrointestinal tumors line has surfaced: GIST (Gastrointestinal Stomal Tumors). The true gastric leiomyosarcoma has become a rare entity. The presentation with brain metastasis had only been reported in the pre-GIST era. Case report: A female, 57 years old, admitted in October 2013, for investigation of abdominal pain, finding a lesion in the left illiac fossa continuing with the terminal ileum. She was submitted to the resection of the lesion with histopathological result showing leiomyosarcoma (6 mitotic figures/10 high power field; IHQ: vimentin positive, S-100 negative, desmin positive). Started chemotherapy. However, presented progression of the disease for subcutaneous tissue, liver (submitted to radiotherapy) and nervous system (only one lesion in the posterior fossa). The patient was submitted in March 7th 2016 to suboccipital craniotomy and en-block resection of the lesion. The microscopic examination revealed a fasicular spindle cell malignant neoplasm, with necrotic areas, with intense and diffuse desmin and smooth muscle actin-positivity in immunohistochemical study, which confirmed the leiomyosarcoma metastatic lesion hypothesis. The patient evolved well during the postoperative period, it was peformed the postoperative MRI, that showed a satisfactory resection. She remained hospitalized until the fourth postoperative day, with no complications, and was then discharged for outpatient follow-up. Conclusion: We have described a case of gastric leiomyosarcoma, emphasizing that even in front of the rarity of leiomyosarcoma itself as a primary lesion (especially after the discovery of GIST as an independent mesenchymal line tumor), this lesion may progress and present central nervous system metastasis, a fact not before reported in literature.

Reporte de un caso

Failure to Thrive or Failure to Think? The Importance of a Systematic Approach in the Evaluation of the Failing Elderly Patient

Anton Camaj and Deborah A. Levine

A 67-year-old male with a history of alcohol dependence and opioid addiction was admitted to the inpatient medicine unit from the emergency department with a diagnosis of failure to thrive. He was hemodynamically stable with an oxygen saturation of 98% on 2 litres of oxygen by nasal cannula. The patient appeared thin, dishevelled and intoxicated, but was alert and conversant. He had numerous scrapes and bruises in various stages of healing across his body. His neurological exam was described as “non-focal, moving all 4 extremities.”

Artículo de investigación

Acupuncture in Primary Headache Disorders; Review of the Evidence

Syed Amir Zaidi and Fayyaz Ahme

Primary headache disorders affect large number of people worldwide. Migraine and tension type headaches constitute 95% of all headaches. Both tension type headaches and migraine impose huge socioeconomic impact on society. The role of non-pharmacological interventions in management of primary headaches is not entirely clear. Acupuncture is one of the most historical non pharmacological ways of treating migraine and tension type headaches. The evidence in support of its effectiveness however is conflicting. This review evaluates the available evidence regarding effectiveness of acupuncture for migraine and tension type headaches.

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