1/67. Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation.BACKGROUND: Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia. OBJECTIVE: To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation. DESIGN: Case report. SETTING: A children's hospital. PATIENT: A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation. methods: Ammonium was measured in plasma. amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations. RESULTS: The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory. CONCLUSIONS: The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra-central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/67. Neurological deficit following spinal anaesthesia: MRI and CT evidence of spinal cord gas embolism.A 62-year-old diabetic woman developed permanent neurological deficits in the legs following spinal anaesthesia. MRI showed oedema in the spinal cord and a small intramedullary focus of signal void at the T10 level, with negative density at CT. Intramedullary gas bubbles have not been reported previously among the possible neurological complications of spinal anaesthesia; a combined ischaemic/embolic mechanism is hypothesised.- - - - - - - - - - ranking = 5.5keywords = spinal (Clic here for more details about this article) |
3/67. Intracranial dural arteriovenous fistulae with perimedullary venous drainage. Anatomical, clinical and therapeutic considerations.We report five cases of intracranial dural arteriovenous fistula (DAVF) with perimedullary venous drainage. All the patients presented with rapidly progressive myelopathy and three had autonomic disorders. The DAVF were on the tentorium cerebelli (two cases), sigmoid (one), superior petrosal (one), and cavernous sinus (one). Slow venous drainage was directed through dilated perimedullary cervical veins. The transverse sinus was occluded in two cases. MRI, performed in four cases, demonstrated high signal on T2-weighted spin-echo sequences in the medulla oblongata and upper cervical spinal cord consistent with oedema, which signal resolved after complete cure of the DAVF in three cases. Embolisation was performed in all cases. It was followed by clinical deterioration in two cases and in the dramatic improvement in the other three, with complete clinical cure in two. Extensive venous thrombosis may explain the deterioration observed in one case.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
4/67. spinal cord swelling preceding syrinx development. Case report.The pathophysiology of syrinx development is controversial. The authors report on a patient with progressive cervical myelopathy and a Chiari I malformation in whom spinal cord swelling preceded, by a few months, the development of a syrinx in the same location. The patient underwent a craniocervical decompressive procedure and duraplasty, and complete resolution of cord swelling and syringomyelia was achieved. This report is consistent with the theory that patients with Chiari I malformation have increased transmural flow of cerebrospinal fluid, which causes spinal cord swelling that later coalesces into a syrinx. The pathophysiology of syrinx development from spinal cord edema and the success of surgical decompressive treatments that do not invade the central nervous system support the prompt treatment of patients with spinal cord edema who are at risk for the development of a syrinx.- - - - - - - - - - ranking = 2.5keywords = spinal (Clic here for more details about this article) |
5/67. 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.- - - - - - - - - - ranking = 3.601284802784keywords = spinal, canal (Clic here for more details about this article) |
6/67. Cystic hygroma colli as the first echographic sign of the fetal akinesia sequence.We report first trimester cystic hygroma colli with subsequent resolution and development of a fetal akinesia deformation sequence. Neuropathological examination of the brain showed intra- and extracellular white matter edema while spinal cord, peripheral nerves and muscles were normal. Hygroma colli as the first echographic sign of subsequent severe fetal akinesia sequence without muscular dystrophy as seen in the Lethal Multiple pterygium syndrome has not been previously reported.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
7/67. Atlas hypoplasia manifesting as myelopathy in a child--case report.A 14-year-old Japanese boy presented with myelopathy due to atlas hypoplasia with complete posterior arch. Decompressive laminectomy of the atlas produced good neurological recovery, and follow-up T2-weighted magnetic resonance imaging showed disappearance of spinal cord edema. Congenital atlas stenosis may be symptomatic even in children, with no accompanying cervical spondylotic change. Such cases have previously occurred only in Asian adults. A radiological study of the patient's brother showed median cleft formation of the posterior arch of atlas, indicative of a wide spectrum of atlas anomalies and a possible genetic relationship between these anomalies.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
8/67. Bilateral subdural effusions related to disease activity in familial hemophagocytic lymphohistiocytosis in an 8-month-old infant.An 8-month-old girl had classic features of hemophagocytic lymphohistiocytosis (HLH). A presumptive diagnosis of familial hemophagocytic lymphohistiocytosis was made on the basis of her age and the presence of parental consanguinity. In view of abnormal neurologic findings at presentation, a magnetic resonance imaging scan was performed and showed bilateral proteinaceous subdural effusions. These resolved within 1 week of commencement of chemotherapy for the primary condition. These subdural effusions were the only objective documentations of central nervous system involvement, along with an increased cerebrospinal fluid protein level. We also report other radiologic findings of HLH, which are of use in strengthening this diagnosis in individuals in whom the diagnosis is strongly suspected.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
9/67. Acute spinal cord swelling in a child with Chiari II malformation.We report on a patient with Chiari II malformation who developed acute cervical myelopathy and brainstem dysfunction following a shunt failure. A brain and spine magnetic resonance image obtained immediately after admission evidenced swelling of the medulla and upper cervical spinal cord. After emergency placement of an external ventricular drain, the swelling receded, the child's respiratory pattern improved and the motor function of his upper and lower extremities progressively returned to its normal condition. The case reported here suggests that in patients with Chiari type II malformation, spinal cord swelling might have a similar pathophysiology to the periventricular edema observed in the supratentorial compartment, and thus may be independent of the early stages of syringomyelia or syringobulbia.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
10/67. False-positive magnetic resonance image in the diagnosis of small acoustic neuroma.A patient presented with sudden hearing loss on her first visit to our department. gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) of the posterior cranial fossa portrayed an intracanalicular tumour image (2-3 mm), and the pure tone average (PTA) and speech discrimination score (SDS) values were 65 dB and 60 per cent, respectively. Surgical intervention to remove the suspected tumour was scheduled by the translabyrinthine approach. Intracanalicular observations by the retrolabyrinthine approach revealed limited oedema on the inferior vestibular nerve with vascular dilation. The tumour image disappeared two years after the operation. Surgical findings and the post-operative course advocate that gadolinium-DTPA-enriched MRI image of an intracanalicular lesion such as arachnoiditis might produce a false-positive result.- - - - - - - - - - ranking = 0.3038544083519keywords = canal (Clic here for more details about this article) |
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