Cases reported "Disease Progression"

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1/135. Primary brainstem injury: benign course and improved survival.

    Primary brainstem injury following head injury is a rare event. The victims often have features of supratentorial injury, and a primary isolated injury to the brainstem occurring due to shearing stresses or to injury from the tentorial edge is extremely rare. In the presence of supratentorial injury, these patients may have altered sensorium. Isolated brainstem injury may manifest itself as internuclear ophthalmoplegia, anisocoria, rigidity and cerebellar tremor. Such injuries are now being diagnosed more often due to improved imaging techniques. We treated nine such cases who had sustained primary brainstem injury in road traffic accidents, all but one of whom were subsequently independent. Primary brainstem injuries need not be associated with poor prognosis and mortality and may run a benign course with good quality of survival.
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2/135. Spontaneous disappearance of temporo-frontal arachnoid cyst in a child.

    We report a child with a large temporo-frontal arachnoid cyst which resolved spontaneously. There was no history of a head injury. The patient was a boy aged 1.6 years. Though a large head was pointed out ( 2SD), no therapeutic intervention was made because the relationship of the head circumference and the cyst was not established. No change in cyst size was visualized on the follow-up CTs at the age of 2.5 years and 6 years. At the age of 7 years, the arachnoid cyst had completely disappeared on CT. In order not to overlook a minute change in cyst size, the volumetry of the cyst and the intracranial cavity was performed, using the Photoshop, Macintosh. Both the cyst volume and the volume ratio of the cyst to the intracranial cavity slightly decreased and then increased. It is speculated that the cyst spontaneously ruptured by factors such as extreme breath holding and crying on the presence of the higher intracystic tension which might become a factor to accelerate spontaneous rupture of the cyst. Since a number of paediatric cases of symptomatic arachnoid cysts in need of surgical intervention has been larger than that of adult cases, we can speculate that a large arachnoid cyst might spontaneously resolve more frequently than we had expected. This case demonstrates that the surgical treatment of asymptomatic arachnoid cyst in the middle cranial fossa is not necessarily indicated in children.
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3/135. 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.
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4/135. Progression of maxillofacial squamous cell carcinoma evaluated using computer graphics with spiral computed tomography.

    This report describes the appearance of maxillofacial squamous cell carcinoma in multiplanar (MPR) and three-dimensional (3D) images reconstructed from spiral CT using computer graphics and the importance of the computer software tools in permitting better visualization of the lesion. Two oral and maxillofacial radiologists and one neuroradiologist analysed the images. The usefulness of interactive reconstruction in the diagnosis, surgical planning and treatment evaluation, especially in the localization of the extent of these maxillofacial tumors, was demonstrated. Spiral CT-based computer graphics for displaying MPR and 3D images are an important adjunct in the evaluation of squamous cell carcinoma.
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keywords = soft
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5/135. Natural course of combined limb and palatal tremor caused by cerebellar-brain stem infarction.

    After infarction of the left superior cerebellar peduncle and dentate nucleus, a patient developed tremor of the left upper limb beginning on the twelfth day followed by palatal tremor appearing 10 months after infarction. Surface electromyogram revealed a difference in the frequency of the tremor in the upper limb and soft palate. When the palatal tremor appeared, brain magnetic resonance T2-weighted images revealed high signal intensity of the contralateral, right inferior olivary nucleus. Subsequently, when the amplitude of palatal tremor became less severe, the high olivary signal intensity subsided whereas the hypertrophy of the nucleus remained. This patient provides useful information on the pathogenesis of skeletal and palatal tremor with brain stem or cerebellar lesions based on the differences in the onset and frequency of tremors and morphologic changes in the inferior olive.
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6/135. 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.
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7/135. Mobility challenges and solutions for fibrodysplasia ossificans progressiva.

    Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive soft tissue ossification. Although signs may be present at birth, the first appearance of ectopic bone typically occurs in early childhood. The primary target is the axial musculature. Eventually ectopic bone also occurs in ligaments, fascia, aponeurosis, tendons, and joint capsules of the appendicular skeleton with a proximal to distal predilection. As the disease advances, mobility becomes restricted, and affected individuals are typically limited to bed or chair by their early 30s. This report describes a 30-year-old woman with advanced FOP. She had a fused spine and a fixed pelvis, with hips and knees locked in flexion and feet in plantarflexion. Her upper limb mobility was similarly restricted. She was not able to stand upright or sit independently. The modification of a commercially available power wheelchair that allowed the patient to maintain her employment as a preschool teacher and custom shoes are described. Creative physiatric intervention is essential to liberate human potential for people with FOP.
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8/135. Dedifferentiation of neoplastic cells in medullary thyroid carcinoma: report of a case.

    We report herein the unusual case of a man who was diagnosed as having sporadic medullary thyroid carcinoma (MTC) at the age of 29 years, and subsequently followed up for a period of 18 years. A total thyroidectomy with radical neck dissection was initially performed, followed by a stable interval of 16 years with regional metastases. He then developed widely disseminated metastases resulting in death within 2 years at the age of 47 years. While the neoplastic tissue from localized metastases in the soft tissue of the neck expressed strong immunohistochemical positivity to calcitonin (CT), calcitonin gene-related peptide, carcinoembryonic antigen, neuron-specific enolase, and chromogranin a during the stable interval, extremely weakened immunoreactivity to those markers was observed in samples from the disseminated metastases in the subcutaneous tissue after his clinical deterioration. Furthermore, only a few neoplastic cells in specimens obtained at postmortem sampling exhibited a weak response to CT. Ultrastructurally, the characteristic secretory granules in the neoplastic cells decreased remarkably in number, consistent with the immunohistochemical findings. These granules also diminished in diameter and intracytoplasmic small lumina and intercellular clefts with microvilli, interpreted as an attribute of anaplastic thyroid carcinomas, were frequently observed in tissues obtained after his clinical deterioration or at postmortem sampling. These cytological changes might represent dedifferentiation of the neoplastic cells or the anaplastic transformation of MTC.
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9/135. osteoradionecrosis of the cervical vertebrae and occipital bone: a case report and brief review of the literature.

    osteoradionecrosis (ORN) is a common complication of radiation therapy. We present the first case reported in the literature of ORN involving the first and second cervical vertebrae and occipital bone in a patient who was treated with surgery and radiation therapy 9 years prior for a TxN3M0 squamous cell carcinoma of the left neck arising from an unknown primary origin. A brief review of the pathophysiology and treatment of this pathological process is also presented. Although the mandible is the most commonly affected site in the head and neck, ORN may develop in an unusual location without any preceding trauma and display an insidious but rapidly progressive course. The pathophysiology of ORN is believed to be a complex metabolic and homeostatic deficiency created by radiation-induced cellular injury and fibrosis, which is characterized by the formation of hypoxic, hypovascular, and hypocellular tissue. The irradiated bone loses its capability to increase the metabolic requirements and nutrient supply required to replace normal collagen and cellular components lost through routine wear. This results in tissue breakdown and the formation of a chronic nonhealing wound. infection plays only a contaminant role, with trauma being a possible initiating factor. diagnosis of ORN begins with a complete physical examination, including fiberoptic examination and biopsy of any suspicious lesion to eliminate the possibility of recurrent tumor. Treatment of ORN commonly requires the debridement of necrotic bone and hyperbaric oxygen therapy. The head and neck surgeon must possess a high degree of suspicion to promptly diagnose ORN and initiate early treatment. Because of similarities in clinical presentation, the most important step in the initial management of suspected ORN is to eliminate the possibility of tumor recurrence or a new primary.
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10/135. 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.
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