Rodrigo Mendonça

Adamantinoma of the spine: case report.

OBJECTIVE:We report a patient with a cervicothoracic spinal and a mandibular adamantinoma. Adamantinoma is a rare malignant neoplasm of bone and, to our knowledge, there have been only five cases of spinal adamantinoma reported. The pathogenesis of the adamantinoma, as well as the management of this extremely rare spinal tumor, is reviewed. CLINICAL PRESENTATION:A 55-year-old man was admitted to our service with cervical pain and signs of C8 and T1 radiculopathy. On physical examination, cervical spine deformity, swelling in the left mandible region, and signs of C8 and T1 radiculopathy were observed. Neuroradiology examinations showed an osteolytic mass of the C6, C7, and T1 vertebral bodies, extending into the lateral masses and transverse processes. After surgical procedures, the patient had clinical improvement. INTERVENTION:Corpectomy of C6, C7, and T1 was performed through a cervicothoracic anterior approach. Anterior stabilization of the spine was obtained using an autologous iliac crest graft and osteosynthesis with an anterior plate. On a second procedure, posterior tumor resection and spinal stabilization were performed. After the 1-year follow-up examination, a new anterior procedure was performed because of tumor recidivity and spine instability. CONCLUSION:Adamantinoma, an extremely rare lesion, is a locally aggressive tumor with slow growth and the potential to metastasize. Although it is an extremely rare occurrence in the spine, adamantinoma should be considered on the diagnosis of tumors of the vertebrae. Neuroradiological examinations are not specific in the differentiation of this tumor from other conditions. This fact, coupled with the limited experience that most physicians in general have in dealing with this tumor, makes the diagnosis and treatment of adamantinoma challenging.

Primary Ewings sarcoma of the spine: case report

Primary malignant sarcomas of the spine are extremely rare. Because of biological heterogeneity, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local control through complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewings sarcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.

Herniation of the cerebellar tonsils after suprasellar arachnoid cyst shunt: case report

It is known that the caudal dislocation of the cerebellar tonsils may occur associated with clinical conditions such as an intracranial mass lesion or Chiari I and II malformation. It may also be acquired after repeated lumbar punctures or lumboperitoneostomy. The occurrence of cerebellar herniation after derivation of intracranial arachnoid cyst is extremely rare, and there are only three cases reported in the medical literature. We present the case of a 9-year-old boy with precocious puberty and suprasellar arachnoid cyst who developed a symptomatic herniation of the cerebellar tonsils three years after a cystoperitoneostomy. The patient underwent a suboccipital craniectomy with duraplasty and partial tonsillectomy, showing afterwards, remission of the symptoms. We discussed the pathogenesis suggested in the literature.

Cerebral cholesterol granuloma: case report

Intracranial cholesterol granulomas are rare lesions, and have been registered in petrous apex region. The presence of an intracerebral cholesterol granuloma is an uncommun event. We report the case of a 20-years-old woman who undergone craniotomy for resection of a temporal mass. Pathologic examination show a cholesterol granuloma.

Bilateral isodense epidural hematoma: case report

We present a case of a severe head injured 23 year-old male patient. The initial CT scan disclosed bilateral epidural hematoma, isodense with the brain, thus being a pitfall in diagnosis. Brief case report, image and literature review are presented.

Glioblastoma primário de cone medula: relato de caso

Glioblastomas are the most common type of brain tumors; astrocytic in their origin, they are anaplastic tumors, and are located mainly in the cerebral hemispheres. Primary growth in the conus medullaris is very rare, and the assessment and prognosis of this kind of tumor are distinct and unique. We present here the case of a 39 years-old man with an intramedullary tumor of the spinal cord, with an histo pathological diagnosis of glioblastoma, along with some therapeutic considerations.

Clinical, histological and radiological responses to methylprednisolone in HIV-associated rod myopathy

Skeletal 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.

Pearls & Oy-sters: A curable myopathy manifesting as exercise intolerance and respiratory failure

Few forms of severe genetic myopathies can successfully be treated, although multiple acyl-CoA dehydrogenase deficiency (MADD) is highly responsive to riboflavin supplementation.

Facial and bulbar muscle atrophy in acetylcholine receptor antibody-positive myasthenia gravis.

A 62-year-old man presented with seven years of progressive dysphagia, dysphonia and difficulty in closing both eyes. His examination showed weakness and atrophy of facial and bulbar muscles without ocular involvement (Figure A, B and C). Single-fiber electromyography revealed increased jitter (Figure D). To evaluate for concurrent myopathy, a muscle biopsy was performed and showed angulated atrophic type II fibers, a particular finding described in patients with myasthenia gravis1,2 (Figure E). Acetylcholine receptor antibody was positive (2.2 nmol/L). Pronounced facial and tongue atrophy is uncommon in myasthenia gravis and usually associated with the muscle-specific receptor tyrosine kinase antibody, which was negative in this patient3,4.

Nemaline myopathy related to HIV infection with a good response to immunosuppression

examination, sclerotic skin and multiple erythematous indurations were observed on chest and all limbs. Range of motion of all the fingers was limited. However, neurologic examination is otherwise normal. Laboratory tests revealed peripheral eosinophilia, hypergammaglobulinemia and polyclonal gammopathy. Antinuclear antibody was marginally elevated while extractable nuclear antibodies were negative. Serum creatine kinase, aldolase and parasitic markers were within normal limits. T1-enhanced magnetic resonance imaging of thigh showed a diffuse thickening and enhancement of fasciae overlying thigh muscles bilaterally. Histopathologic findings were normal in muscle, while marked lymphocytic infiltration was present in fascia. Oral prednisolone and methotrexate relieved his symptoms remarkably leaving no limitation in joint movements or fibrosis. Although EF in childhood has been occasionally reported juvenile EF is even more uncommon. This case shows that initial combined treatment is corticosteroid and methotrexate could lead to a favorable course without residual change even marked limb at a young age of onset.