Neurologic and other systemic manifestations in FMF: Published and own experience

Feld, Olga; Yahalom, Gilad; Livneh, Avi

doi:10.1016/j.berh.2012.01.004

Cited by 43 publications

(61 citation statements)

References 78 publications

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“…However, autoinflammatory mediated injury within the arachnoid villi and the resultant decrease in cerebrospinal fluid absorption and/or thrombotic, vasculitic obliteration of cerebral arteriolar and venous systems are among the most probable mechanisms. 3 In conclusion, to our knowledge, our patient is the first case of FMF and IIH coexistence in the literature. Although the central nervous system is rarely affected in patients with FMF, neurologic involvement might carry the risk of severe disability.…”

Section: Discussionmentioning

confidence: 50%

“…A large part of these manifestations may be directly related to FMF, its complications, associated diseases, and treatment adverse effects. 3 Ertekin et al 10 described seizures in two of 52 children with FMF. While one of the patients with seizures had electroencephalography abnormality, the other was considered as simple febrile convulsion.…”

Section: Discussionmentioning

confidence: 99%

“…Neurological involvement in FMF is rare and shows a wide spectrum, including optic neuritis, recurrent aseptic meningitis, cranial nerve lesions, IIH, ischemic stroke due to coincidental reasons, and subclinical visual evoked potentials abnormalities with demyelinating lesions. 2,3 Only a few reliable reports are present on the association of FMF and IIH in adults. 4,5 To our knowledge, there is no report involving children.…”

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confidence: 99%

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Pseudotumor Cerebri in a Child With Familial Mediterranean Fever

İncecik¹

2015

Arch Rheumatol

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Section: Discussionmentioning

confidence: 50%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Pseudotumor Cerebri in a Child With Familial Mediterranean Fever

İncecik¹

2015

Arch Rheumatol

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“…FMF can be due to tissue injury by vasculitic, amyloidotic, or thrombophilic pathways [8] . Cochlear involvement may be possible by these mechanisms.…”

Section: Discussionmentioning

confidence: 99%

Inner Ear Involvement in Children with Familial Mediterranean Fever

Bucak¹,

Ulu²,

Bükülmez³

et al. 2014

Int Adv Otol

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OBJECTIVE:Familial Mediterranean fever (FMF) is the most common and best understood disease of hereditary periodic fever syndromes. Various degrees of sensorineural hearing loss can be seen in the progression of some hereditary periodic fever syndromes. There are very limited and controversial studies in the literature with regard to hearing loss in patients diagnosed with FMF. We aimed to evaluate cochlear function in children with FMF. MATERIALS and METHODS:Forty-nine patients diagnosed with FMF and 49 age-and sex-matched controls were included in the study. All of the children had undergone a routine ear-nose-throat examination. Patients with pathological findings in the otologic examination or a history of recurrent otitis media; using proven ototoxic drugs; or having an abnormal tympanometry finding were excluded from the study. Following audiologic evaluation, tympanometry and distortion product otoacoustic emissions testing were conducted on all subjects. Pure-tone and high-frequency audiometry were carried out on 40 subjects from both groups who could cooperate in the testing. RESULTS:Hearing thresholds of FMF patients were found to be increased at all frequencies (250 to 16,000) (p<0.001). The difference between the hearing thresholds of the two groups could be seen more prominently at higher frequencies, and despite a statistically significant difference, the hearing thresholds of the two groups were within the normal range of tonal audiometry frequencies. In the otoacoustic emission evaluation, the distortion products and signal/noise ratio of children with FMF were lower in the tested frequencies from 1000 to 4000 Hz (p<0.001). CONCLUSION:Our results demonstrated that FMF disease may cause hearing loss in children with FMF. Hearing loss in children with familial Mediterranean fever has been observed, showing increased hearing thresholds at all frequencies in audiometry, together with decreased distortion products and signal: noise ratios, demonstrated by distortion product otoacoustic emissions testing. We think this might be due to cochlear involvement. Regular follow-up of auditory function in FMF children may be helpful for determining early possible hearing loss.

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“…The occurrence of nervous system manifestations in FMF is rare and their association with FMF is still uncertain (1,2). Additional neurologic disorders have been reported in FMF, such as posterior reversible encephalopathy syndrome (PRES), demyelinating lesions, and ischemic stroke (3,4,5). Attacks of FMF with clinical inflammation are only the tip of the iceberg (6).…”

Section: Introductionmentioning

confidence: 99%

Changes in Cerebral Blood Flow in Patients with Familial Mediterranean Fever

Çetin

Utku

Atilla

et al. 2017

Arch Neuropsychiatr

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INTRODUCTIONFamilial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by repeated attacks of fever, abdominal pain, pleuritic chest pain, and arthritis. The occurrence of nervous system manifestations in FMF is rare and their association with FMF is still uncertain (1,2). Additional neurologic disorders have been reported in FMF, such as posterior reversible encephalopathy syndrome (PRES), demyelinating lesions, and ischemic stroke (3,4,5). Attacks of FMF with clinical inflammation are only the tip of the iceberg (6). Increases in clinical and subclinical inflammation in FMF might lead to susceptibility to vascular comorbidities such as atherosclerosis, heart disease, and life-threatening secondary systemic amyloidosis due to endothelial dysfunction in FMF patients (7,8). The aim of this study was to assess cerebral blood flow velocities using transcranial Doppler (TCD) ultrasonography in patients with FMF. METHODSA total of 30 consecutively admitted patients aged from 20 to 50 years with a diagnosis of FMF who fulfilled the Livneh criteria for FMF were enrolled in the FMF group. The non-FMF group consisted of 30 age-and sex-matched randomly selected patients who did not have any risk factors for atherosclerosis, such as diabetes, hypertension, and coronary artery disease. All patients with FMF were using colchicine and were not in an attack period. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were measured. Consent was obtained from the local ethics committee, and all participants gave formal consent.TCD ultrasonography examination was performed with a Multidop X DWL TCD device after the patient had rested for 10 minutes in the supine position. The right and left middle cerebral arteries (MCAs) were insonated from the temporal windows at a depth of 50-65 mm with 2 MHz pulsed Doppler probes. Bilateral peak-systolic, end-diastolic, and mean blood flow velocities in the MCA, pulsatility index values, and resistance index values were recorded. The results for the FMF group were compared with the results for the non-FMF group. Statistical AnalysisThe data were analyzed using the Statistical Package for the Social Sciences 15.0 program (SPSS Inc.; Chicago, IL, USA). A paired t-test and cross-tabs tests were used for statistical analysis, and a value of p<0.05 was considered to be significant.

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Neurologic and other systemic manifestations in FMF: Published and own experience

Cited by 43 publications

References 78 publications

Pseudotumor Cerebri in a Child With Familial Mediterranean Fever

Pseudotumor Cerebri in a Child With Familial Mediterranean Fever

Inner Ear Involvement in Children with Familial Mediterranean Fever

Changes in Cerebral Blood Flow in Patients with Familial Mediterranean Fever

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