1/129. Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa.A tonic pupil may occur in isolation or as part of a systemic disorder. We report a patient who developed tonic pupils and an abduction deficit in the setting of polyarteritis nodosa. The combination of a tonic pupil and an abduction deficit should suggest the possibility of a vasculopathic disorder, because the ciliary ganglion and lateral rectus muscle are both supplied by the lateral muscular artery. Widespread small artery and arteriolar narrowing and occlusion are the hallmarks of polyarteritis nodosa. Unusual ischemic syndromes may occur, such as this rare combination of neuro-ophthalmic signs, by involvement of both the nutrient artery and its collaterals. We are unaware of other reports of neuropathic tonic pupils in association with polyarteritis nodosa.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
2/129. Growing skull fracture of the orbital roof. Case report.Growing skull fractures are rare complications of head trauma and very rarely arise in the skull base. The clinical and radiological finding and treatment of a growing fracture of the orbital roof in a 5-year-old boy are reported, and the relevant literature is reviewed. The clinical picture was eyelid swelling. Computed tomography (CT) scan was excellent for demonstrating the bony defect in the orbital roof. Frontobasal brain injury seems to play an important role in the pathogenesis of the fracture growth. Growing skull fracture of the orbital roof should be considered in the differential diagnosis in cases of persistent ocular symptoms. craniotomy with excision of gliotic brain and granulation tissue, dural repair and cranioplasty is the treatment of choice.- - - - - - - - - - ranking = 30.381476065355keywords = brain (Clic here for more details about this article) |
3/129. 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 = 16.190738032677keywords = brain, stem (Clic here for more details about this article) |
4/129. Benign aqueductal cyst causing bilateral internuclear ophthalmoplegia after external ventricular drainage. Case report.The introduction of magnetic resonance (MR) imaging to the field of neuroimaging has allowed detection of various lesions that cause aqueductal stenosis. The authors report the case of a 3-year-old boy in whom a benign ventricular cyst developed in the aqueduct. The patient became drowsy after having complained of headache and vomiting; MR imaging revealed mild triventricular dilation and a normal-sized fourth ventricle. Repeated MR imaging performed 1 week later revealed an aqueductal cyst that had markedly enlarged during the intervening period. An external ventricular drainage system was installed, but recovery of consciousness in the child was unsatisfactory and a new bilateral internuclear ophthalmoplegia developed. Fenestration of the cyst wall and placement of a ventriculocisternostomy in the third ventricle were performed simultaneously by using a flexible neuroendoscope. By 2 weeks postsurgery, the patient's neurological symptoms had completely resolved. This case illustrates that simple rerouting of ventricular cerebrospinal fluid (CSF) can aggravate the symptoms of this rare lesion by causing severe compression of periaqueductal structures by a cyst that maintains a high intracystic pressure. Endoscopic surgery was an excellent choice of treatment to achieve both cyst fenestration and normalization of intracranial CSF pressure by creating a ventriculocisternostomy.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
5/129. Bilateral idiopathic Brown's syndrome with delayed onset in the second eye.PURPOSE: We describe 6 cases of a previously unreported variation of bilateral Brown's syndrome that presented in congenital form in one eye and developed later in the fellow eye with no underlying cause. methods: We reviewed the clinical records of 6 patients from 6 separate practices to determine whether there were any common clinical features on presentation or in their clinical courses. RESULTS: All 6 patients were diagnosed with unilateral congenital Brown's syndrome at the first ophthalmologic assessment but showed no evidence of the syndrome in the fellow eye. In 5 cases the contralateral syndrome developed in the second eye after surgery was performed on the first eye, and in 1 case it developed before any surgery was done. The ages at onset of the syndrome in the second eye ranged from 2 to 8 years. None of the children had any evidence of systemic illness or local orbital disease to explain an acquired Brown's syndrome. CONCLUSION: To our knowledge, this is the first reported series of cases of bilateral Brown's syndrome that manifested sequentially in the eyes with no known causes for an acquired syndrome in the second eye. This finding supports the premise that congenital and acquired Brown's syndrome are on a continuum with a common pathophysiology of restriction of free movement of the superior oblique tendon in the trochlea.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
6/129. Insights into the three-dimensional structure of the oculomotor nuclear complex and fascicles.The authors report the case of a patient with an ischemic lesion in the left midbrain. The patient presented with paresis of left inferior rectus, pupil, right superior rectus, convergence and transiently, of the left medial rectus. A lesion in the left dorsal midbrain close to the oculomotor nuclear complex, selectively involving the fascicles innervating the above muscles, is proposed. Fine magnetic resonance sections showed a consistent lesion in the left paramedian dorsal midbrain. A detailed, three-dimensional, schematic computer model of the oculomotor nucleus and fascicles was constructed. Using this model, the authors topographically validate the putative site of the lesion. The medial rectus subnucleus is divided into three subgroups, A, B, and C. Subgroup C is thought to be the site of the majority of neurons controlling convergence. In the above model, the putative lesion is closer to subgroup A than to C; this suggests that subgroup A, rather than subgroup C, may have a higher concentration of neurons involved in convergence.- - - - - - - - - - ranking = 3518.5199977148keywords = midbrain, brain (Clic here for more details about this article) |
7/129. Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism.The case of a 38-year-old patient with rapidly progressing motor neuron disease, complicated by major dysfunction of the extrapyramidal system and of vertical gaze is described. Neuropathological examination revealed a degenerative process that severely affected the lower motor neurons, as well as the neurons of the pars compacta of the substantia nigra, the nucleus of Darkschewitsch, the nucleus interstitialis of Cajal, the colliculi superiores, and the pallidum. The long tracts were unaffected at all levels of the brain stem and spinal cord. There was no convincing evidence for the presence of a multiple system atrophy or progressive supranuclear palsy; the results rather revealed a pattern of vulnerability characteristic of a variant of motor neuron disease.- - - - - - - - - - ranking = 88.955314280596keywords = brain stem, brain, stem (Clic here for more details about this article) |
8/129. Fixation dysfunction with intermittent saccadic intrusions managed by yoked prisms: a case report.BACKGROUND: A 44-year-old woman came to us with a chief symptom of "jumping letters side-to-side, which is most noticeable while reading." The onset occurred after she had experienced a closed head traumatic brain injury 3 years earlier. Several neuro-ophthalmologists diagnosed a fixational instability secondary to saccadic intrusions and prescribed Gabapentin, which provided minimal relief. methods: The term saccadic intrusions refers to an inappropriate saccade with a disrupting effect on fixation. Our examination revealed a myopic anisometropia. Motility testing confirmed saccadic intrusions that lessened on occlusion of either eye and superior gaze. A plano spectacle with six-prism diopter-yoked base down was used to position the eyes in the superior null point. Electro-oculography, using the Visagraph II, demonstrated pre and post changes with the prism. The uncorrected anisometropia allowed the patient to be monocular under binocular viewing conditions. CASE REPORT: The case report focuses on fixational problems that may occur secondary to traumatic brain injuries. There is evidence that the origin of the problem may be from uninhibited brain stem circuits. Pharmacological treatment may only offer transient improvement. The responsibility of a functional cure is often placed on the optometrist. CONCLUSION: This case demonstrates how an alternative use of prism and prescription application can play an important role in the management of fixation dysfunctions.- - - - - - - - - - ranking = 117.33679034595keywords = brain stem, brain, stem (Clic here for more details about this article) |
9/129. A case of superior oblique myokymia observed by an image-analysis system.Superior oblique myokymia is a microtremor of the eye that causes monocular torsional oscillopsia. A modified Harada-Ito procedure was used to treat a case of the disease in a 20-year-old woman. The authors used video-image analysis pre- and postoperatively to evaluate the effect of the surgery on abnormal torsional eye movements. This analysis revealed that before surgery, the abnormal torsional movement had a very regular cycle (duration of attack, 8.0 /- 0.5 s; time interval between attacks, 18.7 /- 3.2 s; n = 9). After the surgery, amplitude of the abnormal torsional eye movement was reduced, and the oscillopsia had subjectively improved, although the movement cycle remained unchanged. The authors' video-image analysis, which used iris striation, proved to be a useful method for clinical measurement of torsional eye movements.- - - - - - - - - - ranking = 4keywords = stem (Clic here for more details about this article) |
10/129. Extramedullary plasmacytoma of the orbit.PURPOSE: To report a case of extramedullary plasmacytoma of the orbit. methods: A 71-year-old patient presented with diplopia eyelid fullness and limitation of ocular motility in the left eye. visual acuity was counting fingers, intraocular pressure 34 mmHg and fundus eye examination showed choroidal folds in the involved eye. RESULTS: CT scan showed a mass filling the superior and external left orbit without bone destruction. A biopsy was performed revealing that the tumour was composed of plasmacytoid cells positive with immunohistochemical stains for Kappa light chains and epithelial membrane antigen. Systemic work up was negative. The diagnosis of extramedullary orbital plasmacytoma was made. The patient was treated with external beam radiotherapy (40 Gy) and has remained disease free for four years (49 months). CONCLUSION: Extramedullary plasmacytomas of the orbit are extremely rare tumours. Accurate and early diagnosis is essential for the therapeutic approach. Extensive medical work up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving the orbit is needed when the diagnosis of orbital extramedullary plasmacytoma is suspected because treatment and prognosis are very different.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
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