Machado-Joseph disease (MJD) is an autosomal dominant neurodegenerative disease characterized by cerebellar ataxia associated to varying degrees with pyramidal signs, extrapyramidal signs, or peripheral amyotrophy. It is caused by unstable expansion of the CAG repeat in the MJD1 gene on chromosome 14q32.1. To determine how the neurodegenerative process in the central nervous system of patients with MJD correlates with the size of expanded CAG repeats in the MJD1 gene and other factors, we performed detailed quantitative analyses of findings of magnetic resonance imaging of the central nervous system of 21 patients with MJD of various ages and with various sizes of expanded CAG repeats. We found that atrophy of the brainstem and cerebellar vermis in MJD patients is closely correlated not only with the size of expanded CAG repeat in the MJD1 gene but also with patient age, which suggests that the neurodegenerative process in MJD is regulated by the size of expanded CAG repeats as well as by the patient age.
The present study was performed to investigate how condylar bony changes related to craniofacial morphology. Twenty‐nine subjects (an average age of 18.8) with condylar bony changes were selected from orthodontic patients who had undergone both helical computed tomography (CT) and magnetic resonance imaging (MRI) to evaluate suspected temporomandibular joint (TMJ) internal derangement and condylar bony changes. Craniofacial morphology was assessed with lateral and frontal cephalograms. For each patient, six linear and five angular measurements were compared with an age‐ and sex‐matched population from the Japanese standard.
In the bilateral condylar bony change group (bilateral group), osteophyte formation and erosion were the common bony change and were present in adult as well as juvenile subjects. In the unilateral condylar bony change group (unilateral group), flattening was the most common feature, erosion was only present in subjects below 19 years.
Disk displacement without reduction was seen in 90.6% of the bilateral group, and in 76.9% of the unilateral group.
In the bilateral group, retrognathic mandible was shown. In the unilateral group, all subjects exhibited a lateral shift of the menton to the side with condylar bony changes.
These findings suggested that condylar bony changes might be progressive and unstable in adults of the bilateral group as well as juveniles of the both groups. It is concluded that condylar bony changes may be related to a lateral shift of the mandible and a retrognathic mandible in orthodontic patients with temporomandibular disorders (TMD) symptoms.
A traumatic fistula occurring between a lacerated middle meningeal artery and a diploic vein was treated conservatively, and was no longer demonstrable at follow-up examination about 1 1/2 years after the injury.
Imaging studies have reported on the relationship between temporomandibular joint (TMJ) degeneration and facial deformity. These studies have suggested that mandibular growth is affected by TMJ degeneration, resulting in altered skeletal structure as mandibular retrusion. However, there are very few longitudinal case reports on TMJ osteoarthrosis (OA). Progressive open bite occurred in an adolescent patient with TMJ OA. Cephalometric analysis showed a downward and backward rotated mandible, and a labial inclination of the upper incisor. Magnetic resonance imaging showed internal derangement without reduction and erosion in the right and the left condyles. Although the cause of open bite is unclear in this case, tongue thrusting, and internal derangements in the temporomandibular joint were suspected as causes of the open bite.
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