![]() A typical episode of facial palsy consisted of a gradual left-sided peripheral facial-nerve paralysis up to House-Brackmann grade 2 (slight) and lasted about 2 days in the beginning to full recovery after initiation of corticosteroid therapy. The episodes of facial weakness and spasm were nevertheless more frequent and lasted longer. The latter new symptoms all responded well to corticosteroid therapy. In 2016, in addition to the aforementioned symptoms, she developed intermittent left peripheral facial nerve palsy, left-sided facial spasm, and progressive left-sided hearing loss. The weakness in the proximal part of the legs was progressive, though not yet invalidating. In the first years, there were no significant auditory deficits, and besides an asymptomatic exophthalmia, there were no visual deficits. Genetic testing did not show a mutation in the gene encoding TGFB1.Īfter the initial diagnosis in 2005, our patient had multiple routine audiograms and ophthalmic evaluations. This specific combination of clinical and radiological manifestations raised the suspicion for a bone dysplasia, more specifically CED. There were no significant hematological or hormonal problems. There was no involvement of facial bones, clavicles, scapulae, ribs, or other short tubular bones. Radiographs showed hyperostosis of the bilateral humerus, femur, and tibia with uneven cortical thickening of diaphysis and, furthermore, a distinct sclerosis of the skull base. The initial presentation of our 27-year-old patient consisted of gait disturbances in early childhood with a progressive course, eventually leading to orthopedic consultation and radiographic evaluation of the skeleton. Another, and perhaps more important, mechanism is hyperostosis of the skull base with foraminal stenosis and secondary cranial neuropathies. Neurological symptoms can be due to hyperostosis of the skull with a decreasing size of the cranial vault and secondary intracranial hypertension. Musculoskeletal symptoms mostly consist of muscle weakness, fatigue, contractures, and pain. Symptoms can comprise musculoskeletal and/or neurological manifestations. In addition to specific clinical features and hypersclerotic radiological abnormalities, in most cases, molecular genetic testing shows a mutation in the TGFB1 gene located on chromosome 19q13. The disease mainly affects the diaphysis of the long tubular bones. It is also known as “progressive diaphyseal dysplasia,” which emphasizes the pathophysiological epifocus. ![]() 1 Camurati 2 and Engelmann 3 were, separately, among the first to describe the condition in the early 1920s. It is classified as a progressive craniodiaphyseal dysplasia within the group of sclerosing bone dysplasias. It suggests that decompression of the facial nerve can lead to improvement in motor and sensory function as well as relief of pain in some patients with combined trigeminal and facial nerve dysfunction.Camurati-Engelmann disease (CED) is a rare condition characterized by hyperostosis of the long bones and skull. This result implicates a trigeminal-facial reflex as hypothesized by others. ![]() The patient's pain and numbness resolved immediately postoperatively, and the facial paralysis improved markedly. ![]() Her fifth episode, which did not spontaneously recover, was associated with retroorbital and maxillary pain as well as sensory loss in the trigeminal distribution.Ī middle cranial fossa approach for decompression of the lateral internal auditory canal, labyrinthine segment of the facial nerve and the geniculate ganglion was performed. This case report of recovery of both trigeminal and facial neuropathy after surgical decompression of the facial nerve suggests an anatomic link.Ī case of a 39-year-old woman presenting with recurrent unilateral facial paralysis is summarized. Although a cranial nerve viral polyneuropathy has been proposed as the usual cause, in many instances the etiology remains unclear. ![]() Trigeminal sensory neuropathy is often associated with facial idiopathic nerve paralysis (Bell's palsy). ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |