Background Protracted febrile myalgia (PFM) is a rare and least well recognized manifestation of familial Mediterranen fever (FMF), characterized by prolonged excruciating muscle pain, tenderness, fever, and elevated levels of acute phase reactants. While the diagnosis of PFM in the setting of previously diagnosed FMF is mostly clinical, diagnosis of PFM when it is the sole manifestation of FMF might be challenging. Objectives To analyse clinical, laboratory, genetic, electrophysiologic, histopathologic, and magnetic resonance imaging (MRI) findings in a series of patients who presented to our department with myalgia and diagnosed as having PFM secondary to FMF. Methods We describe a retrospective cohort PFM patients seen at our department between 2009 and 2012. Clinical, laboratory, and imaging data were obtained by medical record review. Results Study group consisted of 20 (19 male, mean age = 20 years, SD = 3.2) patients. Four (20%) of the patients had a previous diagnosis of FMF, while PFM occurred as the presenting symptom of FMF in 40%. Mean age of disease onset was 17 ± 2.6 years, characterized by severe muscle pain in lower extremities in all patients and affecting single extremity in 90% of the cases. M694V allelic involvement was noted in 80% of the patients. Muscle enzymes were in normal range in all patients, and EMGs were normal in all studied patients (n=13), but one. None of the patients in whom muscle biopsy was available (n=8) showed features compatible with myositis. All of the patients underwent MRI of the symptomatic extremity and showed different degrees of involvement on the MR images of the affected extremities. On MR images, the muscle involvement was either patchy or diffuse, displayed with the high signal intensity on fluid sensitive and gadolinium-enhanced fat saturated T1-weighted images. Extension of the inflammation around individual muscles and muscle groups (myofascial distribution) was observed, as well as subcutaneous tissue edema. Conclusions This series of patients with PFM, to our knowledge, is the largest one reported in the literature. PFM can be the initial manifestation of FMF in some of the patients. Due to the lack of any specific abnormalities other than elevated acute phase response, diagnosis of such cases possess some difficulties. With the appropriate clinical history, detection of MRI findings compatible with myositis in the absence of other features suggestive of myositis (muscle enzymes, EMG, etc.) can help make or confirm the diagnosis of PFM, particularly in areas where the FMF is prevalent. Disclosure of Interest None Declared

Taksim Eğitim ve Araştırma Hastanesi, Nöroloji Kliniği, İstanbul, Türkiye ÖZET Herpes simpleks ensefaliti, akut ensefalitlerin en yaygın formudur. Tedavisiz %70 oranında mortal seyreder. Beyin omurilik sıvısında herpes simpleks virüs pozitifliği ve manyetik rezonans görüntüleme erken tanıda önemlidir. Nadiren olgumuzdaki gibi BOS incelemesinde HSV pozitifliği saptanmadığında tanı klinik ve radyolojik görüntülerle konur. Kırk dört yaşında erkek hasta, ateş, baş ağrısı, bilinç bulanıklığı ve jeneralize nöbet nedeniyle incelendi. Bilinci somnolans, kooperasyonu kısıtlı, ense sertliği mevcuttu. Ateşi 38°C idi. Konuşma dizartrik, kelime bulmakta zorlanıyordu. BOS incelemesinde 1-2 lökosit, 15-20 eritrosit, hafif protein artışı mevcuttu. Difüzyon MRG'de sol temporal lob anteriorunda hiperintesite izlendi. Tekrarlanan MRG'de lezyonun frontal loba yayılmasına rağmen erken ve geç dönemde tekrarlanan serum ve BOS HSV Tip1-2 IgM-IgG, HSV Tip1-2 polimeraz zincir reaksiyonu negatifti. Klinik öykü ve radyolojik görüntülerle olası herpes ensefaliti düşünülen hastamız antiviral tedavi ile sekelsiz iyileşti. Günümüzde herpes ensefaliti tanısında PCR yöntemi altın standart olarak kabul edilmektedir. Nadiren olgumuzdaki gibi PCR negatifliğinde klinik ve radyolojik görüntüleme ile herpes ensefalitinden şüphelenildiğinde tedaviye erken başlamak hayat kurtarıcıdır. (JAREM 2012; 2: 29-32) Anahtar Sözcükler: Ensefalit, herpes simpleks virüs, polimeraz zincir reaksiyonu, beyin omurilik sıvısı, görüntüleme, prognoz ABSTRACT Herpes simplex encephalitis is the most common form of acute encephalitis. Untreated Cases have a mortality of 70%. In early diagnosis, herpes simplex virus positivity in cerebrospinal fluid and magnetic resonance imaging is important. Rarely, as seen in our case, HSV positivity is not detected in CSF analysis diagnosed with clinical and radiological images. A male patient, who was 44 years old, was examined because of fever, headache, confusion and generalized seizures. He had somnolence, limited cooperation, neck stiffness, and fever of 38°C. There was dysarthria in speech and he had difficulty finding words. Examination of CSF leukocyte revealed 1-2, 15-20 red blood cells and a slight increase in protein. In diffusion MRI, hyperintensity was observed in the anterior left temporal lobe. Despite a lesion which spread to the frontal lobe on repeated MRI, repeated early and late period serum and CSF tests for HSV type 1-2 IgM-IgG and HSV type 1-2, the PCR result was negative. Clinical history and radiological images suggest probable herpes encephalitis from which the patient recovered without sequelae with antiviral therapy. Today, the PCR method is considered the gold standard in the diagnosis of herpes encephalitis. However, when the disease is suspected following clinical tests and radiological images,, even if there is PCR negativity, early treatment can be life saving. (JAREM 2012; 2: 29-32)

Paget's disease of the bone is a chronic, focal skeletal disease characterized by bone pain and deformity, pathological fractures and neurological symptoms such as headache, hearing loss and tinnitus, etc. The frequency of the disease increases in later ages. Viral and genetical factors play a role in the etiology. The majority of cases are asymptomatic. It is often diagnosed with an incidental finding on radiography or with an unexpected high serum alkaline phosphatase level. Bone fractures or neurological complications can negatively affect the quality of life. Early diagnosis and treatment are thus very important. Bisphosphonates are the most frequently used medication in the treatment. We present a case who sought medical help because of headache and was diagnosed as Paget's disease. Neurological complications and the diagnosis and treatment of Paget's disease are reviewed in the literature.

Sonuç: Bu çalışmada, C. pneumoniae infeksiyonunun iskemik inme için bir risk faktörü olmadığı sonucuna varıldı. Ateroskleroz ve iskemik inmedeki rolünün kesinleşmesi için geniş popülasyonlu prospektif çalışmalara ihtiyaç vardır. (JAREM 2012; 2: 15-9) Anahtar Sözcükler: İskemik inme, Chlamydia pneumoniae, ateroskleroz, infeksiyon, risk faktörleri, mikroimmünofloresan yöntemi ABSTRACT Objective: This prospective study has been carried out to investigate whether Chlamydia pneumoniae seropositivity is a risk factor for ischemic stroke. Methods:The sera of thirty patients who had acute ischemic stroke and 30 healhty participants were analysed with microimmunofluorescence (MIF) for C. pneumoniae IgG, IgM, IgA antibodies. Risk factors for stroke, clinical findings,laboratory results and radiologic examinations were recorded. Mann-Whitney U test, chi-square test and Fisher's certain probability tests were used for statistical analysis. Odds ratios were calculated.Results: Mean age of the patients was 60.43±15.87 (15 male, 15 female). In 56.7% of the patients with stroke and in 66.7% of the healthy participants, IgG seropositivity was determined in 1/32 titrations. No significant difference was determined between the two groups (p>0.05, OR=0.65). There was also no significant difference in other titrations between groups (p>0.05). IgM seropositivity was determined in 1/16 titrations within 3.3% (1/30) of both patient and healthy population (p=0.5, OR=1). There was no IgA seropositivity in ischemic stroke patients but in 1/16 titration, 3.3% (1/30) positivity was determined within the control group (p=0.50). There was no significant relation between risk factors and IgG seropositivity in the patient group (p>0.05). Stroke subtypes had significant differences with 1/16 IgG titration (p<0.05).Conclusion: This study showed that C. pneumoniae is not a risk factor for ischemic stroke. Wide population prospective studies are needed to show the relation between atherosclerosis and ischemic stroke. (JAREM 2012; 2: 15-9)