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Biomedical subjects

G Akman-Demir

Publications and source records attributed to G Akman-Demir.

14 recordsLinked to original sources
PubMed2003

Cranial MRI in Behçet's disease: 134 examinations of 98 patients.

Two observers, blinded to the patient's neurological status, reviewed 134 MRI studies of 98 consecutive patients with Behçet's disease (BD), to define imaging patterns and to look for any relationship between the MRI findings and the timing of the examination in patients with differing courses of disease. There were 43 patients with overt parenchymal central nervous system (CNS) involvement, 22 with attacks and remissions, 15 with secondary progressive and six with primary progressive disease; 14 had raised intracranial pressure (RICP). Of the remaining 41 patients without specific neurological complaints, 16 had abnormalities on examination (silent CNS involvement) and 25 did not. During an acute CNS attack, the most common finding was a large lesion in the brain-stem or basal ganglia, extending to the diencephalon. On MRI performed after remission of an acute attack or during secondary progression, the same sites were affected, but the lesions were smaller or scattered, with less clearly defined margins. In primary progressive disease or silent CNS involvement, the cerebral white matter was most commonly involved, but almost half the MRI studies were normal. The brain parenchyma was abnormal in only one of the patients with RICP. MRI was normal in all but three patients without clinical CNS involvement, in whom it showed a few millimetric white-matter lesions. Brain-stem atrophy was seen in 15 patients examined >1 year after an initial parenchymal CNS episode, with secondary progressive cases predominating.

Adult↗
PubMed2002

Autoreactivity to myelin antigens related to HLA associations with multiple sclerosis.

Multiple sclerosis (MS) is considered as an immune process influenced by genetic and environmental factors. HLA-DR2 and -DR4 have been documented to be associated with MS. The HLA-dependent differences of immune response to myelin and other antigens might point out some relevant mechanisms in MS development The responses to myelin antigens and to PPD have been compared in 21 MS patients and 25 healthy controls (HCs) by primary proliferation and by short-term T-cell lines. There was a significantly higher response to MBP in DR2+ HCs compared to MS patients (SI: 5.9 versus 1.5, p = 0.02). In short-term T-cell lines, we observed a higher response to PLP30-49 in both DR4+ HCs and MS patients This response was significantly more frequent in DR4+ MS patients (34.6%) than both DR2+ MS patients (0%, p = 0.0001) and DR4+ HCs (7.7%, p = 0.001). The comparison between DR2+ and DR4+ MS patients has revealed that the response to MBP was also increased in DR4+ (p = 0.02). Among DR4+ groups, an increased PPD response was detected in HCs compared to MS (65.2% versus 33.3%, p = 0.01). These results may indicate that HLA-related differences to specific and recall antigens are detectable in MS and these differences may have implications in the disease pathogenesis.

Adult↗
PubMed2001

Cerebral vasculitis and ischaemic stroke in Behçet's disease: report of one case and review of the literature.

INTRODUCTION: Behçet's disease (BD) is a multisystemic, recurrent, inflammatory disorder. Neurological involvement is well-known but cerebral vasculitis and ischaemic stroke are unusual. CASE DESCRIPTION: A 43-year-old male patient presented with acute left hemiparesis, he had recurrent oral aphthae and scrotal ulcerations. Two episodes of transient brainstem ischaemia and an episode of right hemiparesis were reported in the past 2 years. Cranial magnetic resonance (MR) imaging showed a right striatocapsular infarction and multiple segmental stenosis, fusiform enlargement and beading of the arteries of the polygone of Willis were seen on angiography. Cerebro-spinal fluid (CSF) examination disclosed lymphocytic pleocytosis. Skin pathergy test was positive. A diagnosis of BD with cerebral vasculitis was made and immunosuppressive therapy was started. Some improvement of the arterial lesions on MR angiography and normalization of CSF were observed after 1 year of treatment. DISCUSSION: Low grade chronic meningo-encephalitis is the core neuropathological process in neuro-Behçet's disease. Nevertheless BD is a systemic disease known to cause vasculitis and can exceptionally lead to cerebral vasculitis and brain infarction. While BD is usually not part of the differential diagnosis of cerebral vasculitis, it should be borne in mind especially in endemic areas of the disease and in patients from these areas.

Adult↗
PubMed2001

Paroxysmal attacks in multiple sclerosis.

Paroxysmal attacks are short, frequent and stereotyped symptoms that can be observed in multiple sclerosis (MS) patients. We evaluated retrospectively the clinical and neuroradiological findings of patients, who developed any paroxysmal attacks during the course of their disease. EDSS scores, MS clinical types and disease durations of 59 patients with paroxysmal attacks (Group 1) were compared to 586 consecutive patients without paroxysmal attacks recorded in our MS database (Group 2). Anatomical and clinical correlation was attempted in 31 of the patients who had MRI examinations performed within 6 months of the paroxysmal attacks. EDSS scores of patients in Group 1 at the time of the paroxysmal attacks were significantly lower than the scores at their last clinic visit whereas there were no significant differences for other parameters. Our results demonstrate that paroxysmal attacks occur early in the course of the disease, when there is little or no neurologic disability and associated tissue damage.

Brain↗
PubMed2000

Anti-alpha B-crystallin immunoreactivity in inflammatory nervous system diseases.

alpha B-Crystallin, a small heat shock protein, is an immunodominant antigen with increased tissue expression in demyelination. To investigate the humoral response against alpha B-crystallin, the sera and CSF samples of patients with multiple sclerosis (MS), Guillain-Barré syndrome (GBS), neuro-Behçet's disease (NBD) and other non-inflammatory neurological diseases (NIND) were screened by enzyme-linked immunosorbent assay for anti-alpha B-crystallin IgG and IgM antibodies. Serum and CSF IgG antibody responses to alpha B-crystallin were significantly elevated only in NBD patients (serum IgG, NBD 1.29 +/- 0.49 vs. NIND 0.95 +/- 0.39, P = 0.01; CSF IgG, NBD 1.22 +/- 0.64 vs. NIND 0.81 +/- 0.35, P = 0.01). Similarly, high serum IgM antibody titres were also detected in NBD (1.83 +/- 0.72 vs. 1.16 +/- 0.49, P = 0.0005) and in MS (1.57 +/- 1.07, P = 0.046), whereas elevated CSF IgM responses were observed only in GBS (2.09 +/- 1.09 vs. 1.41 +/- 0.7, P = 0.007). Humoral responses against alpha B-crystallin are increased in NBD and GBS, which may implicate this central nervous system antigen in the causation and pathogenesis of these inflammatory nervous system disorders.

Adult↗
PubMed1999

Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet's disease from other central nervous system diseases? [corrected].

Two neuroradiologists reviewed MRI studies of 34 patients with neuro-Behcet's disease (NBD), 22 with multiple sclerosis (MS) and 7 with systemic lupus erythematosus (SLE) with central nervous system involvement, masked to the clinical diagnosis, age and sex of the patients. Of the patients with NBD 12 were in an acute attack; the others had chronic disease. MRI was assessed using a set of criteria, looking at atrophy, the site of discrete parenchymal lesions, regions of predominant involvement and the extent of the lesion(s). The observers also made a guess at the clinical diagnosis. The brain stem and/or basal ganglia were the most predominantly involved sites in all patients with acute NBD; 75% of these lesions were large and confluent, mainly extending from the brain stem to the diencephalon and basal ganglia. However, in chronic cases, the predominant involvement was in the brain stem and/or basal ganglia in only 36%, and in cerebral hemisphere white matter in another 36%; 27% of these patients showed no parenchymal lesion. Hemisphere white-matter lesions were equally distributed between periventricular and other areas in NBD, while in MS more were periventricular, and in SLE more were nonperiventricular. Brain-stem atrophy was seen in 21% of patients with NBD, with a specificity of 96.5%. In the absence of cortical atrophy, its specificity was 100%. The attempt at making a radiological diagnosis was successful in all cases of acute NBD and 95.5% of patients with MS, but in only 40% of patients with chronic NBD. Most of this latter groups MRI studies were interpreted as MS. An extensive lesion involving the brain stem and basal ganglia seemed to be diagnostic of acute NBD. However, hemisphere white-matter lesions could not be differentiated from those in MS.

Acute Disease↗
PubMed1999

Neuropsychological follow-up of 12 patients with neuro-Behçet disease.

We analyzed the data obtained from neuropsychological evaluations of 12 neuro-Behcet Disease (NBD) patients who had been followed up for 35.6+/-23.7 months with successive neuropsychological testing by a comprehensive battery. Memory impairment, which seems to stem basically from a retrieval deficit, was the major finding in this series. The most severely affected memory process was delayed recall, being impaired in all of the patients in the verbal and/or visual modalities. This was followed closely by an impairment in the process of acquisition and storage. In addition to the memory impairment, a "clinical impression of personality change" toward either disinhibition or apathy was seen in 8 of the 12 patients. Attention deficit was of the third highest frequency and was present in 7 patients, followed by deficits of executive functions of frontal system which were present in 5. Other cognitive domains were rarely involved. Neuropsychological status deteriorated insidiously, regardless of the neurological attacks during the follow-up period in most of the patients. Furthermore, our observations also showed the presence of cognitive decline prior to detectable lesions on CT or MRI, emphasizing the need for neuropsychological testing in NBD patients. The late stages of the disease seem to be reflected in MRI as an enlargement of the third ventricle and atrophy of the upper brainstem, which could be compatible with memory loss. Our series, a rather selected group, suggests that NBD can be associated with a special pattern of cognitive deficit, especially memory loss and personality change. The designation of any specific neurobehavioral syndrome for NBD, however, awaits further study.

Adult↗
PubMed1999

Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group.

In order to define the patterns of neurological involvement in Behçet's disease and to assess prognostic factors, 558 files of the neuro-Behçet out-patient clinic were reviewed. Those patients without any evidence of objective neurological involvement as well as the patients with other possible explanations for the neurological picture, and cases not fulfilling the criteria for Behçet's disease were excluded. The remaining 200 cases (155 male, 45 female) were evaluated: 162 had parenchymal CNS involvement (brainstem or 'brainstem +' involvement in 51%, spinal cord involvement in 14%, hemispheric involvement in 15% and isolated pyramidal signs in 19%) while 38 had secondary or non-parenchymal CNS involvement. In the first group the most common findings were pyramidal signs, hemiparesis, behavioural changes and sphincter disturbance, whereas in the second group the syndrome of raised intracranial pressure due to dural sinus thrombosis was the main clinical manifestation. In 60% of the cases with parenchymal involvement, CSF was hypercellular and/or had an elevated protein level, whereas in cases with non-parenchymal involvement the CSF was usually normal except for the elevated pressure. In more than half of the patients with parenchymal involvement, MRI showed brainstem and/or basal ganglion lesions. Forty-one per cent of the cases had a course with at least one attack and remission, another 28% also had attack(s) but showed secondary progression, 10% had primary progression and 21% had silent neurological involvement. Survival analysis was performed in patients who had at least a 3-year duration of neurological disease. Parenchymal involvement, elevated protein and/or pleocytosis in the CSF, 'brainstem +' type involvement, primary or secondary progressive course and relapse during steroid tapering were all associated with a poorer prognosis.

Adult↗
PubMed1998

Humoral immune response to mycobacterial heat shock protein (hsp)65 in the cerebrospinal fluid of neuro-Behçet patients.

Although systemic immune reactivity to 65-kD mycobacterial hsp65 (m-hsp65) has been shown previously in Behçet's disease (BD), local immune response was not investigated. We studied anti-m-hsp65 IgG, IgM and IgA antibodies in the serum and cerebrospinal fluid (CSF) of 25 BD patients with cerebral parenchymal involvement (p-NBD), seven BD patients with intracranial hypertension (ih-NBD), eight BD patients without central nervous system (CNS) involvement, 30 patients with multiple sclerosis (MS) and 24 patients with non-inflammatory CNS disorders (NIC). Significantly higher CSF IgG responses were detected in p-NBD patients (ELISA ratio 1.3 +/- 0.9) compared with NIC (0.7 +/- 0.4, P < 0.01). In p-NBD patients' IgG, IgM or IgA CSF anti-m-hsp65 positivity rate was 48% (12/25); this was significantly higher when compared with MS (3/30; P < 0.03) and NIC (3/24; P < 0.01). CSF anti-m-hsp65 IgG ratios correlated with the duration of BD (r = 0.4, P < 0.04) but not with the duration of neurological involvement. Serum IgM and IgA responses were elevated in ih-NBD, suggesting a different type of involvement than p-NBD. These results implicate an increased local humoral response to m-hsp65 in the CSF of p-NBD patients, which might be related to the pathogenesis of neurological involvement.

Adult↗
PubMed1996

Seven-year follow-up of neurologic involvement in Behçet syndrome.

OBJECTIVE: To determine the long-term prognosis of neurologic involvement in Behçet syndrome. DESIGN: Forty-six patients with Behçet syndrome, who had been the subjects of a previous report with short-term follow-up, were reexamined 7 years later; 42 of them could be reexamined neurologically. Neuropsychological testing, magnetic resonance imaging, electromyography, and evoked potential studies were performed when available. RESULTS: Of the 27 patients who had had headaches without any neurologic symptoms or signs previously, 2 had developed an acute neurologic attack. In addition, 7 patients in this group showed minor abnormalities on neurologic examination and/or other laboratory investigations, without history of any attacks. Among the previous neuro-Behçet group (n = 15), as defined by the presence of neurologic signs or symptoms, other than headache, 7 had a stationary course, while 8 had been progressive. Three of the latter group had died. Patients with progressive course had had abnormal cerebrospinal fluid findings at the time of the previous report, whereas patients with a stationary course had not. CONCLUSIONS: Silent neurologic involvement may occur in Behçet syndrome. Patients should undergo periodic neurologic evaluation. The long-term prognosis in neuro-Behçet syndrome does not seem to be as favorable as we observed in short-term follow-up. Cerebrospinal fluid findings may predict prognosis.

Adult↗
PubMed1996

A controlled study of reliability and validity of MRI findings in neuro-Behçet's disease.

Our aim was to test the reliability of interpreting MRI studies in neuro-Behçet's disease (NBD) and to determine the sensitivity and specificity of different MRI findings. We prospectively studied 50 patients: 24 had chronic NBD, 12 multiple sclerosis, 5 vasculitis other than Behçet's disease (BD) and 9 patients had BD without neurological involvement. MRI studies were performed according to a standard protocol with a 0.2 T imager. Two neuroradiologists, blinded to the diagnosis, age and sex of the subjects, reviewed the films independently, twice. Separate assessments were made for a set of items: dural sinus pathology, widening of ventricles and sulci, brain stem atrophy, lesions of the cerebral cortex, discrete lesions of deep white matter, basal ganglia, brain stem and cerebellum and the presence of smooth periventricular high-signal foci. Intraobserver agreement was substantial or better, and interobserver agreement moderate to substantial for most items. In these patients with chronic NBD we found low sensitivity on all assessed items. Dural sinus pathology or brain stem atrophy were highly specific, but parenchymal lesions in different sites had uniformly low specificity.

Behcet Syndrome↗
PubMed1996

MRI in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease.

Adolescent↗
PubMed1993

Spontaneous dissection of the extracranial vertebral artery with spinal subarachnoid haemorrhage in a patient with Behçet's disease.

A 40-year-old man with known definite Behçet's disease (BD) was admitted with confusional state which had started 4 days before admission with an acute headache and vomiting. Neurological examination revealed confusion, stiff neck, right facial weakness, left hemiparesis, dysartria and truncal ataxia. CSF was haemorrhagic and xanthochromic. Cranial CT scans were negative, but MRI showed a right pontine hyperintense lesion on T2-weighted images. Bilateral carotid angiograms were normal. Right vertebral angiogram showed findings consistent with a dissection at the V2 segment of the artery. At the level of the fifth cervical vertebra, a radiculomedullary branch of the vertebral artery with an aneurysmal dilatation in its intradural portion was notable. This case shows that, in BD, aneurysm formation can also occur in a spinal artery and spontaneous vertebral artery dissection can be seen.

Adult↗