Are Chiari Malformation and Basilar Invagination Associated with Jugular Foramen Stenosis?

OBJECTIVE: We compared the diameter of the jugular foramen (JF) between normal individuals and those with a diagnosis of craniovertebral junction abnormalities, such as Chiari malformation type I (CMI) and/or basilar invagination (BI). METHODS: This cross-sectional analytical study measured the diameter of the right and left JF on magnetic resonance imaging scans of patients divided into 4 groups: 68 with combined CMI and BI (CMI+BI), 42 with isolated BI, 45 with isolated CMI, and 102 control cases. The t test determined sex differences, and univariate analysis of variance with Tukey's post hoc test assessed JF differences between groups. RESULTS: Considering all combinations of laterality and sex, the CMI+BI group had a JF narrower than their respective control groups. The JF was bilaterally narrower in the BI group than in the control group only in the women. Regardless of laterality, the CMI group had a wider JF than did the CMI+BI group in men and did not differ from that of the control group. In the female patients with CMI, the right JF did not differ from that of the controls and was wider than that in the CMI+BI group. Also, the left JF was narrower than that in the control group and did not differ from that in the CMI+BI group. CONCLUSIONS: JF narrowing is associated with CMI and BI when combined in the same patient. When CMI or BI are detected separately, they do not imply evident changes in JF morphometry. It is expected that these findings could bring empiric substrate to support theories that claim a possible role of intracranial pressure disturbances as a triggering factor for CMI.

Sjögren: unique surname, two men, four syndromes and one disease.

Henrik and Torsten Sjögren (/'ʃoʊɡrən/ or SHOH-grən) were two Swedish physicians living in the same period, but completely unrelated, except for their notable contributions to Medicine. The first one described keratoconjunctivitis sicca, afterward called Sjögren's syndrome, and a fishing net aspect retinal pigmentation affecting visual acuity, nowadays known as Sjögren reticular dystrophy. The last one contributed to the understanding of Spielmeyer-Sjögren disease, Marinesco-Sjögren, and Sjögren-Larsson syndromes, all related to genetic disorders and neurological symptoms. In this paper, we aim to describe each disorder, in order to avoid any misunderstanding in diagnosis and for historical record.

Henrik e Torsten Sjögren (/ˈʃoʊɡrən/ ou SHOH-grən) foram dois médicos suecos que viveram na mesma época, mas não tinham nenhuma relação entre si, exceto por suas notáveis contribuições à medicina. O primeiro descreveu a ceratoconjuntivite sicca, posteriormente chamada de síndrome de Sjögren, e uma pigmentação da retina com aspecto de rede de pesca que afeta a acuidade visual, hoje conhecida como distrofia reticular de Sjögren. O último contribuiu para a compreensão da doença de Spielmeyer-Sjögren, das síndromes de Marinesco-Sjögren e Sjögren-Larsson, todas relacionadas a distúrbios genéticos e sintomas neurológicos. Neste artigo, pretendemos descrever cada desordem, a fim de evitar qualquer mal-entendido no diagnóstico e para registro histórico.