1/96. Study of the cell biology and biochemistry of cherubism.AIMS: To establish whether the multinucleate cells in lesions of patients with cherubism are also osteoclasts and if this is the case whether they were responsive to calcitonin; to carry out cytogenetic studies on two members of the same family affected by cherubism in an attempt to identify any major chromosomal defects; and to perform an in-depth modern biochemical study of four children in the same family. SUBJECTS AND methods: Four related children with cherubism were studied. Tissue taken from one of the children at elective decompression of an optic nerve was submitted to in vitro bone resorption studies. Cytogenetic studies were done on two of the children and biochemical studies on all four. RESULTS: The multinucleate cells in the cherubic lesions were shown to be osteoclasts since they synthesised tartrate resistant acid phosphatase, expressed the vitronectin receptor, and resorbed bone. bone resorption by the cultured multinucleate cells was significantly inhibited by calcitonin. High resolution cytogenetic studies failed to detect any chromosomal abnormalities in two children with cherubism. The biochemistry profile of all four children with cherubism showed that serum calcium, parathyroid hormone, parathyroid related hormone, calcitonin, and alkaline phosphatase were within normal levels. urine analysis of pyridinium and deoxypyridinium cross links, hydroxyproline, and calcium in relation to urine creatinine were measured to assess bone resorption in these children, and the values were at the upper end of the normal range in all four. CONCLUSIONS: Further studies are required to determine whether calcitonin treatment will control this grossly deforming disease until the time when the physiological changes that occur at puberty rectify the pathology. It is not recommended that biochemical markers of bone resorption are used in isolation to monitor the activity of cherubism in individuals because the results are based on a small number of children and because of reports of marked interindividual variation in the levels of these markers, particularly in children.- - - - - - - - - - ranking = 1keywords = compression (Clic here for more details about this article) |
2/96. Laminar and arch fractures with dural tear and nerve root entrapment in patients operated upon for thoracic and lumbar spine injuries.OBJECTIVE: To determine the neurological outcome in patients with laminar fractures associated with dural tears and nerve root entrapment, operated upon for thoracic and lumbar spine injuries. PATIENT population: Out of 103 patients operated upon consecutively for thoracic and lumbar spine injuries during the period 1990 to 1994 inclusive, 24 (23.3%) patients had laminar fractures out of whom 3 (2.9%) had an associated dural tear and an other 17 (16.5% or 70.8% of the total patients with laminar fractures) had an associated dural tear and nerve root entrapment. RESULTS: Twelve (70.5%) patients had injury at the thoraculumbar junction, 13 (76.5%) had Magerl's type A3 or above, 10 (58.8%) had a kyphotic angle deformity greater than 5 degrees. Seven (41.1%) had their spinal canal's sagittal diameter reduced by at least 50% and two had dislocations. Nine (52.9%) had initial neurological deficits. Four (50%) out of 8 patients with no initial neurological deficits (Frankel E) worsened to Frankel D. However, one patient among the 3 with initial Frankel A improved to Frankel C while both patients with initial Frankel C usefully improved to final Frankel grades D and E respectively. Two of the four patients with initial Frankel D improved to Frankel E, the other 2 remaining unchanged. All in all five patients neurological status improved, 4 worsened and 8 remained unchanged after neurosurgical treatment. CONCLUSIONS: Vertical laminar fractures with dural tears and nerve root entrapment represent a special group of thoracic and lumbar spine injuries that carry a poor prognosis. However, special operative precautions lead to significant improvement in some of them although a majority remain unchanged or even worsened.- - - - - - - - - - ranking = 8.0350156878873keywords = fracture (Clic here for more details about this article) |
3/96. blindness from bad bones.Progressive visual loss is the most common neurologic finding in osteopetrosis. Several mechanisms may explain this phenomenon, including compression of the optic nerves caused by bony overgrowth of the optic canals and retinal degeneration. We report a child with osteopetrosis and progressive visual loss, even though patent optic canals were demonstrated by computed tomography and digital holography. This patient's visual loss was caused by increased intracranial pressure secondary, to obstruction of cerebral venous outflow at the jugular foramen. This case points to the importance of a full evaluation of the skull base foramina in the diagnostic workup of visual loss in patients with osteopetrosis.- - - - - - - - - - ranking = 1keywords = compression (Clic here for more details about this article) |
4/96. Rapid enlargement and recurrence of a preexisting intrasellar craniopharyngioma during the course of two pregnancies. Case report.Enlargement of preexisting pituitary adenomas during pregnancy is well documented, but this phenomenon is unusual for nonendocrine pituitary tumors such as craniopharyngiomas. Only six cases of craniopharyngioma have been reported as presenting during pregnancy. The authors describe a 19-year-old woman who presented with amenorrhea and galactorrhea caused by an intrasellar mass. Seven months later, when she was 20 weeks pregnant, the patient developed sudden visual dysfunction. Emergency transsphenoidal surgery was performed to restore visual function, and the tumor was found to be a craniopharyngioma. The patient had spontaneous labor and delivered a healthy infant at term. The tumor recurred 4 years later, during her second pregnancy, and was again entirely removed via a second transsphenoidal approach. She again had a normal term delivery. During the 5-year follow-up period she has demonstrated no endocrinological or visual dysfunction. Control magnetic resonance images have revealed no recurrence of the tumor. The transsphenoidal approach seems to be the safest procedure to use during pregnancy to achieve an immediate optic nerve decompression and to preserve pituitary function.- - - - - - - - - - ranking = 1keywords = compression (Clic here for more details about this article) |
5/96. Delayed diffuse upper motor neuron syndrome after compressive thoracic myelopathy.A 54-year-old man developed progressive spastic paraparesis beginning 2 weeks after a back injury caused by a subacute compressive thoracic myelopathy attributable to a post-traumatic arachnoid cyst. Three to 18 months after surgical decompression of the thoracic arachnoid cyst, the patient developed a diffuse predominantly upper motor neuron syndrome characterized by spastic quadriparesis, pseudobulbar paresis, and pseudobulbar affect. Retrograde corticospinal tract degeneration and upper motor neuron death after spinal cord injury is recognized. This case suggests that focal upper motor neuron injury can occasionally precipitate diffuse upper motor neuron dysfunction.- - - - - - - - - - ranking = 1keywords = compression (Clic here for more details about this article) |
6/96. Development of IgG lambda multiple myeloma in a patient with cutaneous CD30 anaplastic T-cell lymphoma.We report a patient with an epidermotropic cutaneous T-cell lymphoma which transformed into an anaplastic cutaneous CD30 T-cell lymphoma. Repeated relapses required prolonged systemic puva therapy. Two years after diagnosis, the patient had several episodes of infections of the respiratory tract. serum electrophoresis now revealed significantly reduced polyclonal immunglobulin production and an additional band in the gamma fraction corresponding to IgG lambda monoclonal gammopathy. Thereafter, the patient suffered a pathologic fracture of the dorsolateral 5th rib on the right side and an accumulation of monoclonal plasma cells in the bone marrow confirmed the diagnosis of multiple myeloma (IgG lambda). Accordingly, 6 cycles of cytoreductive chemotherapy (alkeran, decortin) were given. After one year of steady state disease the patient lost weight and bone pain increased while only a few papular eruptions were detectable. radiography showed multiple small osteolytic areas. A few months later he died with signs of bone marrow insufficiency.- - - - - - - - - - ranking = 1.0043769609859keywords = fracture (Clic here for more details about this article) |
7/96. Progressive bone resorption after pathological fracture of the femoral neck in Hunter's syndrome.We report a case of Hunter's syndrome associated with a transverse fracture of the left femoral neck after minor trauma, followed by progressive resorption of the femoral head at 12 years of age and a stress fracture of the right femoral neck at 16 years of age. MRI performed at 15 years of age revealed intra-articular low intensity on T1-weighted and T2-weighted images of both hip joints. The MR finding may represent fibrous synovial thickening, which caused pressure erosion of the femoral neck, resultant pathological and/or stress fractures, and subsequent osteonecrosis with rapid absorption of the femoral head.- - - - - - - - - - ranking = 7.0306387269014keywords = fracture (Clic here for more details about this article) |
8/96. On the natural history of trigeminal neuralgia.OBJECTIVE: trigeminal neuralgia is usually considered a separate entity from atypical trigeminal neuralgia. The exact relationship among these two and several other syndromes of facial pain remains unknown. There is no long-term prospective study of the natural history of trigeminal neuralgia nor any explanation for the existence of different, albeit somewhat similar, facial pain syndromes. DESCRIPTION OF CONCEPT: On the basis of our clinical experience, we propose a theory that may explain different facial pain syndromes as sequential stages of the same disease process. Typical trigeminal neuralgia caused by microvascular compression of the trigeminal nerve root in the posterior fossa may become transformed over time into atypical trigeminal neuralgia, if left untreated. This transformation involves change in the character of pain and development of sensory impairment. Two representative cases are presented to support this theory. CONCLUSION: If the theory of progressive change in character of pain and degree of sensory impairment in the course of otherwise typical trigeminal neuralgia is correct, trigeminal neuralgia, atypical neuralgia, and trigeminal neuropathic pain may represent different degrees of injury to the trigeminal nerve, therefore comprising a continuous spectrum rather than discrete diagnoses.- - - - - - - - - - ranking = 1keywords = compression (Clic here for more details about this article) |
9/96. Progressive visual loss and ophthalmoplegia.A 51-year-old woman with hyperthyroidism presented with a 4-week history of bilateral progressive visual loss despite treatment with oral prednisone. Her visual function improved after bilateral orbital decompression. The indications for and advantages and disadvantages of radiation therapy and orbital decompression in TAO are discussed. The management of intraocular pressure, strabismus, and lid abnormalities in TAO is also addressed.- - - - - - - - - - ranking = 2keywords = compression (Clic here for more details about this article) |
10/96. Lesions of the internal auditory canal and cerebellopontine angle in an only hearing ear: is surgery ever advisable?OBJECTIVE: To define the indications for surgery in lesions of the internal auditory canal (IAC) and cerebellopontine angle (CPA) in an only hearing ear. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. patients: Seven patients with lesions of the IAC and CPA who were deaf on the side opposite the lesion. Five patients had vestibular schwannoma (VS), and one each had meningioma and progressive osseous stenosis of the IAC, respectively. The opposite ear was deaf from three different causes: VS (neurofibromatosis type 2 [NF2]), sudden sensorineural hearing loss, idiopathic IAC stenosis. INTERVENTION(S): Middle fossa removal of VS in five, retrosigmoid resection of meningioma in one, and middle fossa IAC osseous decompression in one. MAIN OUTCOME MEASURE: Hearing as measured on pure-tone and speech audiometry. RESULTS: Preoperative hearing was class A in four patients, class B in two, and class C in one. Postoperative hearing was class A in three patients, class B in one, class C in two, and class D in one. CONCLUSIONS: Although the vast majority of neurotologic lesions in an only hearing ear are best managed nonoperatively, in highly selected cases surgical intervention is warranted. Surgical intervention should be considered when one or more of the following circumstances is present: (1) predicted natural history of the disease is relatively rapid loss of the remaining hearing, (2) substantial brainstem compression has evolved (e.g., large acoustic neuroma), and/or (3) operative intervention may result in improvement of hearing or carries relatively low risk of hearing loss (e.g., CPA meningioma).- - - - - - - - - - ranking = 2keywords = compression (Clic here for more details about this article) |
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