Prenatal abnormal features of the fourth ventricle in Joubert syndrome and related disorders.

Joubert syndrome and related disorders (JSRD) are characterized by absence or underdevelopment of the cerebellar vermis and a malformed brainstem. This family of disorders is a member of an emerging class of diseases called ciliopathies. We describe the abnormal features of the brain, particularly the fourth ventricle, in seven fetuses affected by JSRD.

Spontaneous recanalization of internal carotid artery occlusion.

We present a case of spontaneous recanalization of the internal carotid artery (ICA) that occurred in a 51-year-old male patient. The occlusion of the right ICA was asymptomatic and was detected incidentally during imaging of the opposite carotid artery. The patient underwent neuroradiologic analysis and a tapered, flame-like occlusion suggestive of the right ICA dissection was detected.

Early onset of carpal tunnel syndrome during pregnancy: case report.

Carpal tunnel syndrome is common during pregnancy and is considered to have a short and benign course. Carpal tunnel syndrome occurs most frequently in third trimester. Patients are usually managed conservatively; symptoms improve after delivery and do not require surgical intervention.

[Vestibular schwannoma (acoustic neurinoma). Natural history and quality of life].

Management of patients with vestibular shwannomas has gradually improved with the development of stereotaxic radiotherapy, our better understanding of these tumours' natural history, and the increasing accent placed on quality of life. The aim of this study was two-fold:--to evaluate the natural history and tumor growth in a series of 386 patients presenting with small- or medium-sized tumors amenable to conservative management; and ii) to compare quality of life in 356 patients undergoing surgery (n = 198), radiosurgery (n = 23) or conservative management (n = 135). The annual tumor growth rate was < 1 mm in 58.

Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2.

Conclusions: New technological developments will most probably improve the efficiency of auditory brainstem implantation (ABI). Meanwhile, cochlear implantation in patients who have undergone prior reductive surgery, and who have maintained a positive electric stimulation, is an excellent alternative for rehabilitating complete and bilateral hearing loss in patients with neurofibromatosis type 2 (NF2). Auditory results are far better than those reported after ABI.