Cases reported "Spinal Cord Diseases"

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1/17. The use of Gore-Tex membrane for adhesion prevention in tethered spinal cord surgery: technical case reports.

    OBJECTIVE: The incidence of retethering caused by postoperative adhesions at the repair site after initial tethered spinal cord surgery is not uncommon. To assess the effectiveness of a Gore-Tex membrane in preventing these adhesions, only clinical radiological and experimental animal evaluation has been reported. In this report, we describe two cases in which Gore-Tex membrane was implanted at the initial untethering surgery and in which we were able to confirm the real effectiveness of the Gore-Tex membrane during a second operation. methods: In the first patient, Gore-Tex membrane was used for dural repair in the untethering surgery of the split spinal cord malformation. Because of the suspicion of a thickened filum terminale, repeated surgery was indicated 10 months after the initial procedure. In the second patient, Gore-Tex membrane was implanted during the initial untethering surgery for a lipomyeloschisis and a dermal sinus. Because of a persistent fistula of the dermal sinus, a second operation was necessary 1 year after the first operation. RESULTS: During the repeated surgery, a thorough inspection of the implanted Gore-Tex membrane revealed no adhesions between the Gore-Tex membrane and the intradural content in both cases. CONCLUSION: We support the use of Gore-Tex membrane in the prevention of postoperative dural adhesions in the repair of spinal dysraphism.
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keywords = animal
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2/17. 'Full dose' reirradiation of human cervical spinal cord.

    With the progress of modern multimodality cancer treatment, retreatment of late recurrences or second tumors became more commonly encountered in management of patients with cancer. spinal cord retreatment with radiation is a common problem in this regard. Because radiation myelopathy may result in functional deficits, many oncologists are concerned about radiation-induced myelopathy when retreating tumors located within or immediately adjacent to the previous radiation portal. The treatment decision is complicated because it requires a pertinent assessment of prognostic factors with and without reirradiation, radiobiologic estimation of recovery of occult spinal cord damage from the previous treatment, as well as interactions because of multimodality treatment. Recent studies regarding reirradiation of spinal cord in animals using limb paralysis as an endpoint have shown substantial and almost complete recovery of spinal cord injury after a sufficient time after the initial radiotherapy. We report a case of "full" dose reirradiation of the entire cervical spinal cord in a patient who has not developed clinically detectable radiation-induced myelopathy on long-term follow-up of 17 years after the first radiotherapy and 5 years after the second radiotherapy.
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keywords = animal
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3/17. Transient oedema of the cervical spinal cord.

    Transient but very intense oedema of the cervical spinal cord was observed in two patients with obstruction of the cerebrospinal fluid (CSF) pathways. Both presented with hydrocephalus, one due to an infratentorial obstructing mass and the other due to postmeningitic adhesive obstruction of the outlet foramina of the fourth ventricle. In animal experiments with obstruction of CSF pathways (due to outlet foramina obstruction or to downward tentorial herniation) flattening and stretching of the ependymal cells along the central canal is observed, followed by disruption and splitting of the ependymal lining and then by extracellular oedema of the subependymal tissue. Without treatment, frank cavity formation develops in a fourth stage. In our two patients, however, most probably because of appropriate decompressive therapy, the oedema disappeared completely without a residual spinal cord lesion.
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keywords = animal
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4/17. Cerebellar hypoplasia in Werdnig-Hoffmann disease.

    The case of an infant with Werding-Hoffmann disease, who died at the age of 4 1/2 days, is reported. At autopsy there was severe cerebellar hypoplasia, associated with degenerative changes in the brain-stem nuclei. This case supports the concept that cerebellar hypoplasia may develop as a manifestation of the neuronal abiotrophy of Werdnig-Hoffmann disease. A similarity has been noted between the cerebellar lesion found in the child reported here and that produced by viral infection in experimental animals.
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keywords = animal
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5/17. Split cord malformation and situs inversus totalis: case report and review of the literature.

    INTRODUCTION: situs inversus is a rare condition of visceral transposition in which the spinal axis is rarely affected. CASE REPORT: The authors report a patient with situs inversus totalis and type II split cord malformation. This patient had no complaints and presented with scoliosis. CONCLUSIONS: Recent compelling evidence from animal models and human case reports has led to hypotheses that defects of the midline and laterality defects (e.g., situs inversus) are etiologically related. Confirmation from additional case reports of situs inversus and split cord malformation could prove useful in determining a genetic locus for split cord malformations or implicating various chemical agents that are known to produce situs inversus as potential causative factors in the production of split cord malformations.
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ranking = 1
keywords = animal
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6/17. Myelopathy due to copper deficiency following gastrointestinal surgery.

    BACKGROUND: Ataxic myelopathy due to copper deficiency has been described in ruminant animals and is called swayback. Neurological manifestations due to inherited copper deficiency secondary to the failure of intestinal copper absorption is well recognized as Menkes disease. The neurological consequences of acquired copper deficiency in humans are not well described. OBJECTIVE: To report 2 cases where patients developed a myelopathy with copper deficiency after gastrointestinal surgery.patients Two patients developed a myelopathy many years after gastrointestinal surgery. Both had severe copper deficiency, which was the likely cause of the myelopathy. CONCLUSIONS: Acquired copper deficiency may present as a myelopathy. Gastrointestinal surgery and resulting decreased copper absorption may be causative.
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ranking = 1
keywords = animal
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7/17. Split cord malformation and situs inversus totalis: case report and review of the literature.

    INTRODUCTION: situs inversus is a rare condition of visceral transposition in which the spinal axis is rarely affected. CASE REPORT: The authors report a patient with situs inversus totalis and Type II split cord malformation. The patient had no symptoms and presented with scoliosis. CONCLUSIONS: Recent compelling evidence from animal models and human case reports has lead to hypotheses that defects of the midline and laterality defects (e.g., situs inversus) are etiologically related. Confirmation from additional case reports of situs inversus and split cord malformation could prove useful in determining a genetic locus for split cord malformations or implicating various chemical agents that are known to produce situs inversus as potential causative factors in the production of split cord malformations.
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ranking = 1
keywords = animal
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8/17. copper deficiency myelopathy.

    BACKGROUND: In humans, Menkes disease is the well-recognized neurological disorder due to inherited copper deficiency. Myelopathy due to acquired copper deficiency is not a well-recognized entity in humans, although myelopathy due to copper deficiency is well documented in some animal species. patients: We describe 3 patients who developed a progressive spastic-ataxic gait with proprioceptive deficits. All patients had a severe reduction in serum ceruloplasmin and copper levels. RESULTS: All patients had evidence of posterior column dysfunction clinically and on somatosensory evoked potential studies. Two had a signal change in the posterior column on magnetic resonance imaging of the spinal cord. CONCLUSION: patients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.
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ranking = 1
keywords = animal
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9/17. Central neuropathic itch from spinal-cord cavernous hemangioma: a human case, a possible animal model, and hypotheses about pathogenesis.

    Cavernous hemangiomas (cavernomas) of the spinal cord are rare congenital malformations that comprise less than 5% of all intramedullary lesions. Despite this rarity, we describe the third case of central neuropathic itch associated with intramedullary cavernoma. Since fewer than 10 cases of central spinal itch from all causes have been published, this concurrence suggests the possibility of a specific association. A middle-aged man developed chronic disabling neuropathic itch and pain affecting his left shoulder and arm after frank hemorrhage of a midcervical cavernoma. We hypothesize that the relatively rostro-dorsal location of his lesion increased its likelihood of causing itch as well as pain. The microscopic pathology of cavernomas, specifically their gliotic rim containing hemosiderin-laden phagocytes, fosters ectopic firing of nearby neurons and makes cranial cavernomas highly epileptogenic. We hypothesize that these pathological features predispose cavernomas to cause central itch if they are located near, but spare, the central itch projection neurons in lamina I of the dorsal horn. Quisqualate injections into the deeper layers (neck) of the dorsal horns of rats produce pathologically similar lesions. Such rats develop unilateral dermatomal hyperalgesia and self-injurious scratching and biting (autotomy). Although this pathological grooming is currently interpreted as a response to chronic pain, we propose that it more likely models scratching provoked by central neuropathic itch, as seen in our patient and others. Study of quisqualate-injected rats may provide leads towards new treatments for neuropathic itch.
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ranking = 4
keywords = animal
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10/17. Spinal shock--comparative aspects and clinical relevance.

    Spinal shock is the loss of muscle tone and segmental spinal reflexes that develops caudal to a severe spinal cord injury. Although little recognized in the veterinary literature, spinal shock occurs in animals and is important in both accurate lesion localization and case management. In this review, we consider why spinal shock occurs and discuss the subsequent physiologic alterations, including the development of reflex hyperactivity. We also discuss differences in the expression of spinal shock between species and suggest why recovery of reflexes is relatively rapid in animals compared with humans. Finally, the implications for clinicians dealing with animals in the period immediately after severe spinal injury are considered.
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ranking = 3
keywords = animal
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