Kader Karlı-Oğuz

An unusual central nervous system involvement in rheumatoid arthritis: combination of pachymeningitis and cerebral vasculitis

Severe primary central nervous system (CNS) involvement such as vasculitis and pachymeningitis can rarely occur in rheumatoid arthritis (RA) even in the absence of systemic disease activation. The authors illustrate a female patient with well-controlled RA who presented with headaches, encephalopathy, seizures and relapsing focal neurological deficits. Primary rheumatoid cerebral vasculitis and pachymeningitis were diagnosed based on suggestive brain magnetic resonance (MR) imaging, MR angiography, cerebrospinal fluid analysis and cerebral angiography. MR showed abnormal leptomeningeal enhancement and hyperintense FLAIR signal in the cortical subarachnoid spaces consistent with pachymeningitis. Cerebral angiography findings were consistent with vasculitis. Aggressive treatment resulted in significant clinicoradiological resolution. Cerebral vasculitis is a rare but certain manifestation of RA. This complication can be diagnosed in the presence of suggestive angiographic and CSF findings. The condition may be steroid resistant, and needs to be treated more aggressively.

Congenital infiltrating lipomatosis of the face with ipsilateral hemimegalencephaly

Abstract. An extremely rare case of congenital infiltrating lipomatosis of the face (CILF) associated with ipsilateral hemimegalencephaly is reported in a 3-month-old boy. MRI not only thoroughly evaluated the extent of the lesion, but also demonstrated the cerebral anomaly. MRI is indispensable in the evaluation of patients suspected of having CILF.

A clinically isolated syndrome: a challenging entity: multiple sclerosis or collagen tissue disorders: clues for differentiation.

Acute isolated neurological syndromes, such as optic neuropathy or transverse myelopathy, may cause diagnostic problems since they can be the first presentations of a number of diseases such as multiple sclerosis (MS) and collageneous tissue disorders. In the present study, particular systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) patients, who were followed up with the initial diagnosis of possible MS with no evidence of collagen tissue disorders for several years, are described. Five patients with the final diagnosis of SLE and five pSS patients are evaluated with their neurologic, systemic and radiologic findings.Over several years, all developed some systemic symptoms like arthritis, arthralgia, headache, dry mouth and eyes unexpected in MS. During the regular and close follow-up laboratory evaluations of vasculitic markers revealed positivity, leading to the final definite diagnosis of SLE or pSS. Patients with atypical neurological presentation of MS, a relapsing remitting clinical profile, or lack of response to the regular MS treatment should be evaluated for the presence of a connective tissue disease. Various laboratory tests, such as cerebrospinal fluid findings, autoantibodies profile, markers, cranial and spinal magnetic resonance imaging, can be helpful for the differential diagnosis. Lack of response to the regular multiple sclerosis treatment, even increasing rate of relapses can force the clinician for the differential diagnosis. In particular cases an accurate diagnosis can only be made after close follow-up.

Radiologic aspects of abdominal hydatidosis in children A study of 31 cases in Turkey

OBJECTIVE To determine the location and radiological characteristics in children with abdominal hydatid disease (HD). MATERIALS AND METHODS Thirty-one children (average age: 7.2 years) with abdominal HD were studied. The number, location, diameter and internal architecture of the cysts were assessed with abdominal ultrasonography (US) and computed tomography (CT). Density measurements and enhancement patterns were determined on CT. RESULTS Twenty-one children had hepatic HD. The remaining 10 children had both hepatic and extrahepatic cysts. There were splenic cysts in five children, peritoneal cysts in two children and combined splenic and peritoneal cysts in three children. The most common site of the cysts was the liver (64%), followed by the spleen (20%) and the peritoneal cavity (16%). The seven intraabdominal cysts, which were not detected by US, were 20 mm or less in diameter. CONCLUSION CT may demonstrate additional small intrahepatic or unsuspected extrahepatic cysts. Although rare, splenic or peritoneal hydatidosis should be included in the differential diagnosis of a cystic splenic or peritoneal lesion. Familiarity with atypical locations of HD may be helpful in making a prompt, accurate diagnosis. We think that in particular patients, especially those who had diagnostic problem and who are under surgical planning, CT should be performed additionally.

Teaching NeuroImages: A neurosyphilis case presenting with atypical neuroradiologic findings

A 51-year-old man presented with memory loss, behavioral changes, and generalized seizures for 4 months. Mini-Mental State Examination (MMSE) score was 11. MRI showed hyperintensities in multiple cortical areas and thalami (figure, A–C). Diffusion was restricted in certain areas (figure, D and E). Treponemal antibody tests were positive in serum and CSF. He was treated with penicillin. Six months later, his behavior and memory improved considerably (MMSE 20).

DEFAULT-MODE NETWORK FUNCTIONAL CONNECTIVITY CHANGES IN ALZHEIMER'S DISEASE

and p 181 -tau (R1⁄4 0.48; p1⁄4 0.017), but not A b 42. Lactate was not correlated with any CSF biomarker. The Figure depicts estimatedmarginal means and standard errors from the mixed model.Conclusions: Patients with early AD have higher glucose concentration in an area susceptible to AD pathology compared to controls, perhaps as a result of lower glucose utilization due to neurogeneration. In addition, lactate concentration is higher, suggesting higher regional metabolic reliance on glycolysis. These results motivate the longitudinal study of MRS glucose and lactate as novel AD biomarkers. Acknowledgement: This research was supported entirely by the Intramural Research Program of the NIA/NIH.