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Biomedical subjects

N Koçer

Publications and source records attributed to N Koçer.

16 recordsLinked to original sources

Behçet's disease: diagnostic and prognostic aspects of neurological involvement.

This study was conducted to describe clinical and prognostic aspects of neurological involvement in Behçet's disease (BD). Patients referred for neurological evaluation fulfilled the criteria of the International Study Group for Behçet's Disease. We analyzed disability and survival by the Kaplan-Meier method, using Kurtzke's Extended Disability Status Scale (modified for BD) and the prognostic effect of demographic and clinical factors by Cox regression analysis. We studied 164 patients; of the 107 diagnostic neuroimaging studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thrombosis (VST) and the others were normal. CSF studies were performed in 47 patients; all with inflammatory CSF findings (n = 18) had parenchymal involvement. An isolated increase in pressure was compatible with either VST or normal imaging. The final diagnoses were VST (12.2%), neuro-Behçet syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet syndrome (0.6%), and indefinite (11%). VST and NBS were never diagnosed together. Ten years from onset of BD 45.1% (all NBS) reached a disability level of EDSS 6 or higher, and 95.7 +/- 2.1% of the patients were still alive. Having accompanying cerebellar symptoms at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurological diagnoses in BD are NBS and VST. These are distinct in clinical, radiological, and prognostic aspects, hence suggesting a difference in pathogenesis.

Behcet Syndrome↗

Idiopathic granulomatous hypophysitis: are there reliable, constant radiological and clinical diagnostic criterias?

Idiopathic granulomatous hypophysitis is a rare inflammatory disease of unknown aetiology; few cases are reported. We review the clinical presentation and radiological characteristics of these cases and our own experience with three new surgical cases, to determine diagnostic criteria. MRI of three cases revealed sellar lesions extending into the chiasmatic cistern. Their shape varied, from dumbbell to spherical and elliptical. All were isointense with the brain on T1-weighted images and gave heterogeneously high signal on T2-weighted images. Contrast enhancement was homogeneous in one case and heterogeneous in another. The pituitary stalk could not be identified. There was no dural enhancement. The sphenoid sinus mucosa was thickened in two cases and normal in one.

Adult↗

Syringomyelia--as a late complication of tuberculous meningitis.

The aim of this paper is to demonstrate the unusual MR features of thoracic syringomyelia following TB meningitis and to discuss the neurosurgical aspect of the treatment of this rare entity. Four years after a TB meningitis episode, a 30 year-old female patient developed a progressive spastic paraparesis. MR studies revealed multiloculated syrinxes throughout the thoracic cord. She had a syringo-subarachnoid shunt with a silastic "T" tube inserted. On the first postoperative day, she showed a dramatic neurological improvement, but unfortunately her paraparesis progressed to the preoperative level within a month despite diminished size of the syrinxes on the control MRI examination. Two and a half years after the operation the patient complained of having a burning type of central pain, and further deterioration in neurological function. Thoracic spinal MRI examination demonstrated enlarged syringomyelic cavities. At the second operation syringo-peritoneal shunt insertion was performed via right T10-11 hemilaminectomy using a "T" tube. At present, 4 months after the second operation, the patient's neurological examination demonstrated decreased spasticity, and improved strength in the legs compared to the preoperative level. MRI is the first choice of investigation in detecting TB related myelopathy as it provides a greater detail of pathological changes within and around the spinal cord such as syrinx formation and arachnoiditis. The MR findings are also helpful in deciding the management and predicting the outcome. Presence of multifocal loculations and arachnoid adhesions is the likely cause of treatment failures and poor prognosis.

Adult↗

Surgical outcome of epilepsy patients evaluated with a noninvasive protocol.

Surgery is now an accepted treatment for some medically intractable epilepsies. Presurgical evaluation is particularly important for the localization of the epileptogenic zone, which may necessitate sophisticated imaging techniques and intracranial electroencephalogram (EEG) recordings. If patients are carefully selected, however, successful results can be achieved with noninvasive evaluation methods. Seventy-seven patients were operated on for intractable seizures. All patients underwent EEG, neuropsychological, psychiatric, and magnetic resonance imaging investigations. Ictal EEG-video recording was performed in all nonlesional and in some lesional cases that had discordant data. Selective amygdalo-hippocampectomy was performed on patients with mesial temporal lobe epilepsy (MTLE), an extended or a limited lesionectomy was performed on patients with structural lesions, and a lesionectomy with deafferentation was performed on two patients with West syndrome. Electrocorticography was not used. Temporal lobe directed surgery was performed in 63.6% of the cases. The pathological examinations of all cases showed hippocampal sclerosis (HS) in 43%, tumor or tumor-like lesions in 36%, and cortical dysplasia in 5% of patients. After a mean follow-up of 17 months (range, 2-53), 75% of the patients were seizure-free with or without aura and 15% had a marked improvement, whereas 10% did not benefit from surgery. Neuropsychological outcome of patients with MTLE and HS also showed worthwhile results. Our patients, who were evaluated without pre- and perioperative intracranial recordings and other sophisticated techniques, had an outcome comparable to those in other series from more experienced centers. Our experience indicates that successful results, especially for patients with MTLE-HS and lesion-related epilepsies, can be obtained at centers with limited resources if the diagnoses and evaluation procedures are performed carefully.

Adolescent↗

Siblings with cystic leukoencephalopathy and megalencephaly.

Cystic leukoencephalopathy with megalencephaly is a newly described entity with mild clinical involvement. Patients suffer from developmental problems and seizures in childhood. Progression is gradual into adulthood. Typical magnetic resonance imaging findings include subcortical cysts and diffuse leukoencephalopathy. The etiology is unknown with possibly autosomal-recessive inheritance. We present two pairs of siblings with this disease and emphasize the characteristic and variable patterns even within the same family.

Brain↗

Extradural balloon obliteration of the empty sella report of three cases (intrasellar balloon obliteration).

Empty sella syndrome is an anatomical and clinical entity composed of intrasellar reposition of the CSF and compression of the pituitary tissue, resulting in a clinical picture of headache, visual field defect, CSF rhinorrhea and some mild endocrinological disturbances. While some cases are primary with no appreciable aetiology, secondary cases are associated with prior operation or radiotherapy of the region. In our series, 3 patients with primary empty sella syndrome were treated by the current approach of extradural filling of the sellar cavity. This technique was first described by Guiot and widely accepted thereafter. We used a detachable silicon balloon filled with HEMA or liquid silicone for obliteration of the sellar cavity and obtained clinically satisfactory results without complications. Visual symptoms regressed and headache disappeared. But at long term follow-up all the balloons were found to be deflated. Despite the facility and efficacy of the technique we do not recommend it in the treatment of the empty sella because the filling of the sella is only transient and relapses may occur.

Adult↗

MRI and MRS in HMG-CoA lyase deficiency.

3-Hydroxy-3-Methylglutaryl coenzyme A lyase (HMG-CoA) deficiency is a rare inborn error of leucine catabolism. The disease is characterized by recurrent episodes of metabolic acidosis, hyperammonemia without ketosis, hypoglycemia, lethargy, hepatomegaly, and seizures. This study has evaluated the magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) findings of three patients with HMG-CoA deficiency. The common findings on all of the MRI scans were multiple, coalescent, marked lesions in periventricular white matter and arcuate fibers, most prominently in frontal or periatrial regions that were superimposed on diffuse, slightly hyperintense subcortical white matter signal. Involvement of the caudate nucleus and the dentate nucleus were observed in the reported patients. MRS studies by both STEAM and PRESS spectra of all patients revealed a decrease in N-acetylaspartate and elevation in both myoinositol and choline. A pathologic peak at 1.33 ppm, which is compatible with lactate, and a particular peak at 2.42 ppm in all patients were also found. The combination of both MRI and MRS findings could be considered as being specific in patients with HMG-CoA lyase deficiency.

Amino Acid Metabolism, Inborn Errors↗

Biliary pseudolithiasis in childhood: a case report.

Cholelithiasis is uncommon in childhood and usually associated with any predisposing factors such as congenital abnormalities of biliary tract, hemolytic diseases, TPN administration and diseases of terminal ileum. Recent studies demonstrated ceftriaxone inducing reversible precipitations in gallbladder that mimic cholelithiasis. This complication is termed "biliary pseudolithiasis" or "reversible cholelithiasis". In this paper we describe a patient who developed biliary pseudolithiasis after six days of ceftriaxone therapy which completely resolved eleven days after the end of the treatment, and discuss the indication for cholecystectomy.

Adolescent↗

Iniencephaly: neuroradiological and surgical features. Case report and review of the literature.

Iniencephaly is a rare congenital anomaly characterized by spina bifida of the cervical vertebrae, fixed retroflexion of the head on the cervical spine, and occipital bone defect. There are only five reports of surviving patients with iniencephaly. The authors report the case of a newborn who presented with iniencephaly and an encephalocele that were surgically treated in our service. Neurological examination of the patient yielded normal results except for a moderate psychomotor retardation. The neuroradiological and surgical findings of the case suggested that the trigger of the anomaly was the occipital bone defect and rachischisis of the posterior vertebral arches.

Brain↗

Primary myxoid-type mediastinal liposarcoma.

In this paper we present a case of primary mediastinal liposarcoma and describe CT findings of this malignant tumor. In light of the literature we also discussed the mediastinal fatty masses.

Aged↗

CNS involvement in neuro-Behçet syndrome: an MR study.

BACKGROUND AND PURPOSE: Behçet disease (BD) is a multisystem vasculitis of unknown origin in which neurologic involvement has been reported in the range of 5% to 10% in large series. Reports on clinical and radiologic aspects of neuro-Behçet syndrome (NBS) are in general limited in number. Our purpose was to determine the MR patterns in patients with NBS who had neural parenchymal involvement and to correlate our findings with possible vascular pathophysiology. METHODS: The MR images of 65 patients with NBS and neural parenchymal involvement were reviewed. In a subgroup of patients who had serial MR studies, we evaluated the anatomic-radiologic location and distribution of the lesions and whether they corresponded to any vascular territory, and studied their extension, enhancement patterns, and temporal course. RESULTS: The most common imaging finding in NBS patients who had neural parenchymal involvement was a mesodiencephalic junction lesion with edema extending along certain long tracts in the brain stem and diencephalon in 46% of the patients. The next most common location of involvement was the pontobulbar region, seen in 40% of the cases. Three primary cervical spinal cord lesions and one case of isolated optic nerve involvement were observed. CONCLUSION: The parenchymal distribution of lesions in NBS appears to support the hypothesis of small-vessel vasculitis; mainly, venular involvement. The anatomic distribution of intraaxial veins of the CNS explains the predominant involvement of the brain stem structures observed in our patients. This pattern of lesion distribution might help to differentiate NBS from other vasculitides as well as from the inflammatory-demyelinating diseases of the CNS, such as multiple sclerosis.

Adult↗