ABSTRACT
Background: Neuro-cranio-vertebral syndrome (NCVS) includes a set of idiopathic diseases: Arnold-Chiari syndrome type 0,1 and 1.5, idiopathic scoliosis, and idiopathic syringomyelia. It is caused by the pathological traction transmitted by the filum terminale on the neuraxis. Considering that the filum terminale is inserted at sacrococcygeal level, it is logical to think that an alteration of this segment's anatomy, such as an anterior coccyx dislocation, can increase the tension exerted by the filum terminale on the neuraxis, which in turn triggers NCVS. Methods: We collected data from 372 patients with NCVS and 15 patients with coccygeal dislocation and NCVS from our database. We analyzed the relationship between the sacrococcygeal and intercoccygeal angles with signs, symptoms and associated diagnoses. T-tests for independent samples and linear regression were used for analysis (p <0.05). We describe the MRI findings and clinical features of NCVS caused by coccygeal dislocation and compare its prevalence with that of a sample without coccygeal dislocation. Results: 65% of the signs, symptoms, and imaging features were present in similar proportions in both samples. There was a similar prevalence of cerebellar tonsillar descent and scoliosis in both groups, whereas the incidence of syringomyelia differed. A sacrococcygeal angle between 89-110° prevails in cases of syringomyelia while an intercoccygeal angle between 90-140° prevails in cases with cerebellar tonsillar descent. Conclusions: The high clinical and radiological prevalence of coccygeal dislocation in patients with Neuro-cranio-vertebral syndrome suggests an association between them. The mechanism involved in coccygeal dislocation can directly influence the development of Neuro-cranio-vertebral syndrome.
ABSTRACT
BACKGROUND: We propose two new concepts, the Filum Disease (FD) and the Neuro-cranio-vertebral syndrome (NCVS), that group together conditions thus far considered idiopathic, such as Arnold-Chiari Syndrome Type I (ACSI), Idiopathic Syringomyelia (ISM), Idiopathic Scoliosis (IS), Basilar Impression (BI), Platybasia (PTB) Retroflexed Odontoid (RO) and Brainstem Kinking (BSK). METHOD: We describe the symptomatology, the clinical course and the neurological signs of the new nosological entities as well as the changes visible on imaging studies in a series of 373 patients. RESULTS: Our series included 72% women with a mean age of 33.66 years; 48% of the patients had an interval from onset to diagnosis longer than 10 years and 64% had a progressive clinical course. The commonest symptoms were: headache 84%, lumbosacral pain 72%, cervical pain 72%, balance alteration 72% and paresthesias 70%. The commonest neurological signs were: altered deep tendon reflexes in upper extremities 86%, altered deep tendon reflexes in lower extremities 82%, altered plantar reflexes 73%, decreased grip strength 70%, altered sensibility to temperature 69%, altered abdominal reflexes 68%, positive Mingazzini's test 66%, altered sensibility to touch 65% and deviation of the uvula and/or tongue 64%. The imaging features most often seen were: altered position of cerebellar tonsils 93%, low-lying Conus medullaris below the T12L1 disc 88%, idiopathic scoliosis 76%, multiple disc disease 72% and syringomyelic cavities 52%. CONCLUSIONS: This is a paradigm shift that opens up new paths for research and broadens the range of therapeutics available to these patients.
