1/53. Neuropathology of Joubert syndrome.Very little documentation of the neuropathologic changes in Joubert syndrome exists. This paper presents a detailed postmortem neuropathologic study of a clinically and radiographically well-documented case of Joubert syndrome. In addition to aplasia of the cerebellar vermis and fragmentation of the dentate nuclei, there was marked dysplasia of structures at the pontomesencephalic junction and caudal medulla. There was abnormal decussation of the superior cerebellar peduncles and an enlarged iter (rostral 4th ventricle) with elongated tegmental nuclei (including the locus coeruleus). neurons of the basis pontis and reticular formation appeared reduced. Extensive malformations of the medulla included hypoplasia of the inferior olivary nuclei, solitary nuclei and tracts, and the nucleus and spinal tracts of trigeminal nerve (cranial nerve V). Even more striking was dysplasia of the caudal medulla at the cervicomedullary junction, which was characterized by the absence of a posterior median sulcus, neuronal swelling and axonal spheroids in the region of malformed nuclei gracilis and cuneatus, and absence of pyramidal decussation. This study suggests that, in addition to vermal agenesis, Joubert syndrome is characterized by malformation of multiple brainstem structures. The latter could explain certain clinical features of the syndrome, including episodic hyperpnea and oculomotor apraxia.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/53. Fresnel membrane prisms: clinical experience.BACKGROUND: There are few published reports on the clinical application of Fresnel membrane prisms in the treatment of diplopia in adults. The authors describe the use of these prisms in patients with fourth and sixth cranial nerve palsies, restrictive motility caused by thyroid-related orbital disease, and convergence insufficiency. methods: Of 209 patients who had been treated with Fresnel prisms, 141 were selected. The database included patients from a private practice in Montreal, seen from 1988 to 1996, and patients seen by orthoptists in the ophthalmology department of a children's hospital in Montreal between 1992 and 1996. All the patients had diplopia associated with fourth (48 patients) or sixth (43 patients) cranial nerve palsy, thyroid-related orbitopathy (18 patients) or convergence insufficiency (32 patients). After qualitative and quantitative assessment of the ocular misalignment, a Fresnel prism was selected for power and axis and for appropriate location on the spectacle lens. Ocular dominance and side of paresis or restriction were also considered in the placement of the prism. The patient's response to treatment was documented. RESULTS: The Fresnel prisms were oriented horizontally in 72 patients (51%), vertically in 55 (39%) and obliquely in 14 (10%). They were placed on the spectacle lens before the nondominant eye in 127 cases (90%), either covering the entire lens, or on the upper or lower segment or both. The patients were followed for an average of 15 (range 2 to 96) months. Of the 141 patients 113 (80%) had a successful outcome, with relief of their diplopia. Twenty-seven patients (19%) eventually had the prismatic correction ground into the lens, 70 (50%) chose to wear the Fresnel prism on a permanent basis because incorporation into the lens was not possible or because of cost, and 17 (12%) used the Fresnel prism as a temporary device before or after surgery. Most patients who converted to incorporated prisms did so when the prismatic power became stable, usually after 6 to 8 months. Eight patients (6%) stopped using the prism because of associated side effects, such as blurred vision, persistent diplopia, torsion or optical aberrations. INTERPRETATION: The Fresnel prism is an excellent device in treating diplopia in adult patients. It is a reasonable permanent option when incorporating the prism into the spectacle lens is not possible. A fused blurred image caused by a Fresnel prism placed in front of the nondominant eye is preferable to double but clear images.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/53. Lagophthalmos: an unusual manifestation of oculomotor nerve aberrant regeneration.PURPOSE: To describe a patient with unusual findings after regeneration of the oculomotor nerve. methods: Case report. RESULTS: A 35-year-old woman developed complete right third nerve paralysis after neurosurgical ligation of internal carotid-posterior communicating and internal carotid-ophthalmic artery aneurysms. Permanent ipsilateral lagophthalmos appeared as third nerve function spontaneously recovered. CONCLUSION: Lagophthalmos may rarely develop after aberrant regeneration of the oculomotor nerve, presumably caused by co-contraction of the levator and superior rectus muscles during the Bell's phenomenon.- - - - - - - - - - ranking = 4keywords = nerve (Clic here for more details about this article) |
4/53. Treatment of subtotal medial rectus myectomy complicating functional endoscopic sinus surgery.During the past 2 decades, the introduction of functional endoscopic sinus surgery (FESS) has dramatically improved the treatment of sinus disorders. However, a variety of orbital complications have been reported, including optic nerve damage, hemorrhage, infection, compromise of the lacrimal drainage apparatus, and strabismus. At least 10 cases have reported damage to the medial rectus muscle. (1-8) Treatment options for such patients have been limited, especially because most are adults at risk for anterior segment ischemia after transposition of vertical rectus muscles. We describe 2 patients whose medial rectus myectomies were repaired by using nonabsorbable "hang-back" sutures in combination with a botulinum toxin (Botox) injection of the antagonist lateral rectus muscle. Good primary position alignment was achieved in both patients, and one patient was able to regain binocular function. We recommend this surgical approach, especially in patients at increased risk for anterior segment ischemia.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
5/53. Remission of superior oblique myokymia after microvascular decompression.Superior oblique myokymia (SOM) is an ocular motility disorder characterized by oscillopsia, vertical or torsional diplopia, sometimes combined with pressure sensation. Although the pathophysiological basis is unclear, isolated case reports have documented its association with intracranial pathological processes. We present a case of SOM associated with a vascular compression of the fourth nerve at the root exit zone. Following microneurosurgical decompression, SOM completely resolved and paralysis of the fourth nerve occurred. This was less disturbing.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
6/53. July 2000: A 70 year old with rigidity, decreased ocular movements, and dementia.The July Case of the Month (COM): A 70 year old male presented with a four year history of cognitive decline, difficulty expressing himself, and an increasingly unsteady gait with numerous falls. At presentation he was wheel-chair bound. Examination showed some slowing of speech, mild memory impairment, but normal cranial nerves. Spastic weakness and brisk reflexes were also noted, with bilateral ankle clonus. MRI scans were normal. Four years later he was admitted with a urinary tract infection and was mute with severely impaired ocular motility. He died 18 months later and autopsy showed the classic neuropathological findings of typical Progressive supranuclear palsy, including tau-positive glial inclusions.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
7/53. Major orbital complications of endoscopic sinus surgery.BACKGROUND: The paranasal sinuses are intimately related to the orbit and consequently sinus disease or surgery may cause severe orbital complications. Complications are rare but can result in serious morbidity, the most devastating of which is severe visual loss. methods: A retrospective review was undertaken of four cases of severe orbital trauma during endoscopic sinus surgery. RESULTS: All the cases suffered medial rectus damage, one had additional injury to the inferior rectus and oblique, and two patients were blinded as a result of direct damage to the optic nerve or its blood supply. CONCLUSION: Some ophthalmic complications of endoscopic sinus surgery are highlighted, the mechanisms responsible are discussed, and recommendations for prevention, early recognition, and management are proposed.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
8/53. Monocular elevator paresis in neurofibromatosis type 2.A retrospective review of 29 consecutive unselected patients referred for neuro-ophthalmic evaluation after the diagnosis of neurofibromatosis type 2 (NF2) showed that four of them had a monocular elevator paresis. In two of the four MRI demonstrated lesions, presumed to be schwannomas, of the third nerve. These findings indicate that monocular elevator paresis is a common neuro-ophthalmic finding in NF2, which the authors suspect is probably a sign of third nerve infiltration or compression by a schwannoma.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
9/53. Neuro-ophthalmological manifestations of systemic lupus erythematosus in Asian patients.PURPOSE: To report eight patients with diagnosed systemic lupus erythematosus (SLE) who presented with a variety of neuro-ophthalmological complications and outcomes. methods: A review of the follow up, subsequent management and outcomes of the neuro-ophthalmological complications of eight patients with SLE. RESULTS: The patients presented with a myriad of symptoms of varying severities. The most common manifestations were that of optic neuropathy and eye movement abnormalities.The outcome was variable ranging from complete recovery to optic atrophy with navigational visual acuity. Treatment was often empirical, although early treatment with corticosteroids has been tried with varying success, CONCLUSIONS: The pathophysiology in the neuro-ophthalmological manifestations of SLE is thought be due to vaso-occlusive disease of the small vessels of the nerve and brain, secondary to an autoimmune process. Ophthalmologists may need to consider the diagnosis of SLE in young women who present with a recent onset of neuro-ophthalmological symptoms and signs.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
10/53. Elevation of one eye during tooth brushing.PURPOSE: To describe a patient with congenital fibrosis of the extraocular muscles (CFEOM) and elevation of one eye during tooth brushing. methods: A 9-month-old girl underwent full ophthalmologic examination. Exonic and flanking intronic sequences of the CFEOM2 ARIX gene were sequenced. RESULTS: The patient's clinical findings were consistent with classic CFEOM including chin elevation, bilateral ptosis, infraducted globes, no voluntary ocular elevation beyond midline, and convergence on attempted upgaze. During tooth brushing her right eye elevated beyond midline. This was elicited only by touching her teeth or gums but not by mouth movements. No ARIX mutations were detected. CONCLUSION: This is the first case described with CFEOM and aberrant regeneration between the nerve to the superior rectus and the trigeminal nerve. This aberrant innervation supports a primary developmental abnormality of the cranial nerves in CFEOM.- - - - - - - - - - ranking = 1.5keywords = nerve (Clic here for more details about this article) |
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