1/315. Atypical herpes simplex encephalitis presenting as operculum syndrome.This case report demonstrates the course of herpes simplex virus cerebritis in a patient aged 7 years 2 months who presented with non-specific symptoms followed by an epileptic attack. Subcortical, bilateral opercular and bilateral thalamic lesions were detected, but the temporal and inferior frontal lobes were spared. The patient developed anarthria, impairment of mastication and swallowing consistent with operculum syndrome. diagnosis was made by magnetic resonance imaging and elevation of oligoclonal antibodies specific to herpes simplex virus in cerebrospinal fluid after an unexpectedly negative polymerase chain reaction test.- - - - - - - - - - ranking = 1keywords = cerebrospinal (Clic here for more details about this article) |
2/315. A distinct difference in clinical expression of two siblings with Aicardi-Goutieres syndrome.Two sibs with an encephalopathy, including intracerebral calcification and a white matter disease, are reported. In the younger sister, the cerebrospinal fluid showed chronic pleocytosis and clinically she strictly fits to the diagnosis of Aicardi-Goutieres syndrome. Both sisters were affected by a spastic tetraplegia, truncal hypotonia and dystonic posturing, but the clinical course and the neuroradiological findings were milder in the older sister and she showed no cerebrospinal fluid pleocytosis. The present cases and recent reports of intrafamilial variability of Aicardi-Goutieres syndrome may raise interesting aspects as to the limits and criteria of this syndrome.- - - - - - - - - - ranking = 2keywords = cerebrospinal (Clic here for more details about this article) |
3/315. plasma exchange in Rasmussen's encephalitis.The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease.- - - - - - - - - - ranking = 1keywords = cerebrospinal (Clic here for more details about this article) |
4/315. Upper respiratory problems in the yellow nail syndrome.A case of the yellow nail syndrome is described. The characteristic nail changes were associated with pleural effusions, lymphoedema of the face and legs and upper respiratory symptoms. The finding of extremely hard ear wax has not been reported previously. A satisfactory response to combination diuretic therapy was seen. The case of recognition of the Syndrome is emphasized together with the danger of excessive investigation if the nails are not inspected. Possible mechanisms for the formation of extremely hard cerumen are discussed.- - - - - - - - - - ranking = 2.1566226235342keywords = effusion (Clic here for more details about this article) |
5/315. MRI findings in lumbar puncture headache syndrome: abnormal dural-meningeal and dural venous sinus enhancement.intracranial hypotension (IH) is a treatable cause of persistent headaches. Persistent cerebrospinal fluid (CSF) leak at a lumbar puncture (LP) site may cause IH. We present postcontrast MRI of a patient with post-lumbar-puncture headache (LPHA) showing abnormal, intense, diffuse, symmetric, contiguous dural-meningeal (pachymeningeal) enhancement of the supratentorial and infratentorial intracranial dura, including convexities, interhemispheric fissure, tentorium, and falx. MRI also showed abnormal dural venous sinus enhancement, a new finding in LPHA, suggesting compensatory venous expansion. Thus, IH and venodilatation may play a role in the development of LPHA.- - - - - - - - - - ranking = 1keywords = cerebrospinal (Clic here for more details about this article) |
6/315. Positron emission tomography in juvenile alexander disease.A 13-year-old boy with cervical kyphosis was diagnosed as having juvenile alexander disease because of the typical MRI findings, abnormally elevated alphaB-crystallin and heat shock protein 27 in the cerebrospinal fluid. Positron emission tomography with 18F-fluorodeoxyglucose demonstrated hypometabolism in the frontal white matter corresponding to the areas with leukodystrophy. However, the overlying gray matter preserved normal glucose metabolism.- - - - - - - - - - ranking = 1keywords = cerebrospinal (Clic here for more details about this article) |
7/315. Polyangiitis overlap syndrome with eosinophilia associated with an elevated serum level of major basic protein.Polyangiitis overlap syndrome is a new disease entity and the reported cases in the literature are still limited. We describe a female patient presenting with finger ulcers, skin eruptions, pleural effusion, interstitial pneumonia and eosinophilia. skin biopsy showed systemic small-sized angiitis and thrombosis. She was diagnosed as having polyangiitis overlap syndrome and was successfully then treated with corticosteroid. It is also of interest that the disease activity was correlated with the number of eosinophils in peripheral blood. The measurement of the serum level of major basic protein released from eosinophils functioning as a coagulant indicated the possible association of eosinophilia with thrombosis and polyangiitis.- - - - - - - - - - ranking = 2.1566226235342keywords = effusion (Clic here for more details about this article) |
8/315. The syndrome of pericarditis, arthritis, and camptodactyly: an under-recognized cause of pericardial constriction in children?The syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. pericardiectomy is the treatment of choice.- - - - - - - - - - ranking = 2.1566226235342keywords = effusion (Clic here for more details about this article) |
9/315. Sclerotomy in uveal effusion syndrome.PURPOSE: To report visual and anatomic outcomes after modified scleral surgery in patients with uveal effusion syndrome with retinal and choroidal detachment. methods: In five eyes of four patients with retinal and choroidal detachment and uveal effusion syndrome due to nanophthalmos, we performed pars plana full-thickness unsutured sclerotomies without sclerectomy. RESULTS: In all cases, uveal effusion with choroidal and retinal detachment resolved within 3 weeks, and all patients showed improved vision. The functional and anatomic results remained stable for over 2 years. CONCLUSIONS: Uveal effusion syndrome that is refractory to medical treatment (high-dose systemic corticosteroids) can be managed effectively by pars plana full-thickness unsutured sclerotomy without sclerectomy.- - - - - - - - - - ranking = 17.252980988273keywords = effusion (Clic here for more details about this article) |
10/315. Jugular foramen syndrome caused by varicella zoster virus infection in a patient with ipsilateral hypoplasia of the jugular foramen.We report a patient with acute cranial polyneuropathy with unilateral involvement of the ninth, tenth, and eleventh cranial nerves. Although this patient lacked a typical cutaneous herpetic manifestation, elevated levels of IgM and IgG antibodies to varicella zoster virus (VZV) in both the serum and cerebrospinal fluid confirmed the clinical diagnosis of VZV infection and zoster sine herpete. Coexisting hypoplasia of the ipsilateral jugular foramen was detected using three-dimensional, surface-rendering displays reconstructed from the cranial helical CT scan. The patient recovered almost completely following treatment with an anti-inflammatory corticosteroid. Anatomical narrowing of the jugular foramen in this patient may have contributed to entrapment of the affected nerves at their passage through the foramen.- - - - - - - - - - ranking = 1keywords = cerebrospinal (Clic here for more details about this article) |
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