The aim of this study was to evaluate if subjective symptoms, radiographic and especially MR parameters of cervical spine involvement, can predict neurologic dysfunction in patients with severe rheumatoid arthritis (RA). Sequential radiographs, MR imaging, and neurologic examination were performed yearly in 46 consecutive RA patients with symptoms indicative of cervical spine involvement. Radiographic parameters were erosions of the dens or intervertebral joints, disc-space narrowing, horizontal and vertical atlantoaxial subluxation, subluxations below C2, and the diameter of the spinal canal. The MR features evaluated were presence of dens and atlas erosion, brainstem compression, subarachnoid space encroachment, pannus around the dens, abnormal fat body caudal to the clivus, cervicomedullary angle, and distance of the dens to the line of McRae. Muscle weakness was associated with a tenfold increased risk of neurologic dysfunction. Radiographic parameters were not associated. On MR images atlas erosion and a decreased distance of the dens to the line of McRae showed a fivefold increased risk of neurologic dysfunction. Subarachnoid space encroachment was associated with a 12-fold increased risk. Rheumatoid arthritis patients with muscle weakness and subarachnoid space encroachment of the entire cervical spine have a highly increased risk of developing neurologic dysfunction.
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http://dx.doi.org/10.1007/s003300000557 | DOI Listing |
Int J Lang Commun Disord
December 2024
Hearing, Speech & Language Center, Sheba Medical Center, Tel Hashomer, Israel.
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December 2024
Department of General Surgery, The Affiliated People's Hospital of Jiangsu University, 8 Dianli Road, Zhenjiang, 212002, Jiangsu, China.
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December 2024
Department of Endocrinology, Children's Hospital, Zhejiang University School of Medicine, National Children's Regional Medical Center, National Clinical Research Center for Child Health, 3333 Binsheng Road, Hangzhou, 310052, Zhejiang Province, China.
Williams Syndrome (WS) is a rare neurodevelopmental disorder with a prevalence of 1 in 7500 to 1 in 20,000 individuals, caused by a microdeletion in chromosome 7q11.23. Despite its distinctive clinical features, the underlying metabolic alterations remain largely unexplored.
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December 2024
Department of Neurology, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-ku, Tokyo, 105-8461, Japan.
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View Article and Find Full Text PDFWorldwide, congenital deafness and pigmentation disorders impact millions with their diverse manifestations, and among these genetic conditions, mutations in the Microphthalmia-associated transcription factor (MITF: OMIM#156845) gene are notable for their profound effects on melanocyte development and auditory functions. This study reports a novel porcupine model exhibiting spontaneous deafness and pigmentation abnormalities reminiscent of human Waardenburg Syndrome Type 2 (WS2: OMIM#193510). Through phenotypic characterization, including coat color, skin, eye morphology, and auditory brainstem response (ABR) assessments, we identified hypopigmentation and complete deafness in mutant porcupines.
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