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Epilepsy and recursive consciousness with special attention to Jackson's theory of consciousness.

John Hughlings Jackson's theory of consciousness has been reconsidered. The author stressed that his uniqueness as a neuroscientist lay in his keen interest in the recursive nature of human consciousness. Two clinical symptoms of interest to Jackson were discussed: recurrent utterances and mental diplopia. Recurrent utterances were believed to represent an exceptional state, in which the unconscious process in speech production, otherwise destined to be swept away automatically, became manifest and observable. Jackson regarded mental diplopia as a revelation of otherwise inaccessible duality of all healthy mental actions. Therefore, he supposed that the essence of recursive consciousness resided in a transformation of multiple, multidirectional, unconscious processes into a linear, unidirectional process.

Cognition↗

Penicillamine-induced myasthenia in rheumatoid arthritis: its clinical and genetic features.

The clinical features and genetic background of 18 patients with rheumatoid arthritis were investigated following the development of penicillamine-induced myasthenia (PIM). The initial myasthenia symptoms in all patients consisted of variable diplopia and/or ptosis with progression to a more generalized involvement in 7 of them. No clinical, humoral, or genetic factor was determined which would allow identification of individuals developing generalized as opposed to ocular myasthenia. Withdrawal of penicillamine was associated over 4-60 weeks with a slow resolution of symptoms, facilitated in 12 patients by the use of anticholinesterase agents. In 2 patients a persistent partial unilateral ptosis remains after 15 and 25 months, while in a further patient diplopia is present 42 months after resolution of the other myasthenic symptoms. The patients with PIM when compared with a healthy 'control' population had a significant increase in HLA Dr1 (p corr less than 0.005) and an absence of HLA Dr 3. A genetic susceptibility to the development of PIM, distinct from that observed in myasthenia gravis of spontaneous onset, is suggested by this abnormal distribution of HLA Dr antigens.

Adult↗

Extraocular muscle surgery for Graves' ophthalmopathy: does prior treatment influence surgical outcome?

We analysed the results of extraocular muscle surgery in 38 patients with stable Graves' ophthalmopathy. Fixed sutures were used in all patients. A useful field of binocular single vision was achieved in 27 patients (71%) after one operation and in seven patients (18%) after more than one, whereas double vision was persistent in four (11%). No recurrence of diplopia was seen during one year of follow-up. No differences were found in duration of eye disease, angle of deviation, or in prior forms of treatment between patients who responded well to a single operation and those who needed more surgical procedures or those who responded less well. We conclude that, although the individual outcome cannot be predicted, every patient with diplopia and stable Graves' ophthalmopathy has an 89% chance of binocular single vision after extraocular muscle surgery. Prior treatment does not appear to influence this outcome.

Adult↗

Serum levels of soluble Fas in patients with Graves' ophthalmopathy.

AIM: To assess levels of soluble Fas (sFas) in the sera of patients with Graves' ophthalmopathy. METHODS: The subjects in this study were 43 patients with Graves' ophthalmopathy and 11 normal subjects. Serum levels of sFas were determined by sandwich enzyme linked immunosorbent assay. In addition, serum levels of thyroid stimulating antibody (TSAB) were also measured in all the patients. RESULTS: The mean serum level of sFas was 1.35 (SD 2.03) ng/ml in patients with Graves' ophthalmopathy, and 0.93 (0.32) ng/ml in normal subjects. Serum levels of sFas in the subgroup of 24 patients with diplopia (1.98 (2.56) ng/ml) were significantly higher than those in the subgroup of 19 patients without diplopia (0.56 (0.24) ng/ml) and normal subjects (p <0.001). Serum levels of sFas in the subgroup of 27 patients with extraocular muscle hypertrophy (1.81 (2. 46) ng/ml) were significantly higher than those in the subgroup of 16 patients without extraocular muscle hypertrophy (0.58 (0.26) ng/ml) among the patients with Graves' ophthalmopathy and normal subjects (p <0.001). Serum levels of sFas were not significantly different between the subgroup of 24 patients with proptosis (1.15 (0.98) ng/ml) and the subgroup of 19 patients without proptosis (1. 61 (2.88)). In contrast, the serum levels of TSAB in the subgroup of patients with proptosis (723% (1161%)) were significantly higher than those in the subgroup of patients without proptosis (194% (122%)) (p <0.05). CONCLUSIONS: Elevated sFas levels were associated with extraocular muscle disorders but not with proptosis. On the other hand, elevated TSAB levels were associated with proptosis but not with extraocular muscle disorders, suggesting different immunological mechanisms for the extraocular muscle disorders and proptosis in Graves' ophthalmopathy. Determination of the serum levels of sFas and TSAB could provide useful markers for evaluation of the immunological processes involved in the development of Graves' ophthalmopathy.

Adolescent↗

Anomalies of binocular function in patients with longstanding asymmetric keratoconus.

AIMS: To study binocular function in patients with longstanding asymmetric keratoconus. METHODS: In 20 adult patients with longstanding asymmetric keratoconus managed with a scleral contact lens a full clinical and orthoptic assessment was performed with and without the scleral contact lens in the poorer eye. RESULTS: All 20 patients had a corrected acuity of at least 6/9 in their better eye. With the scleral lens in situ the acuity of the poorer eye ranged from 6/6 to 6/60 and without the lens from 6/18 to hand movements. Patients were aged from 18 to 68 years and had worn a scleral contact lens for between 3 and 106 months. Without the contact lens in their poorer eye all patients had a small exotropia and all showed suppression, with the exception of one patient who had a right hypertropia with diplopia. With the scleral lens in situ 12 patients had an exophoria or esophoria, six a microexotropia, and two a manifest exotropia with suppression. CONCLUSIONS: Binocular function breaks down in some adult patients with longstanding asymmetric keratoconus. This is probably caused by longstanding unilateral visual deprivation. There are similarities to the breakdown of binocular function seen in some patients with a longstanding dense unilateral adult onset cataract who can develop intractable diplopia following cataract surgery.

Adolescent↗

One year follow up of macular translocation with 360 degree retinotomy in patients with age related macular degeneration.

AIM: To evaluate the benefits of macular translocation with 360 degree retinotomy in patients with exudative age related macular degeneration (ARMD). METHODS: A consecutive interventional case series was performed on patients who underwent macular translocation between June 1997 and January 2000 at the department of ophthalmology, University of Aachen, Germany. A retrospective pilot study was set up with a minimum follow up of 12 months in 39 consecutive patients with subfoveal choroidal neovascularisation secondary to ARMD. The surgical technique included pars plana vitrectomy, induction of retinal detachment, 360 degree retinotomy, removal of the choroidal neovascular membranes (CNVM), macular translocation, peripheral laser retinopexy, and silicone oil endotamponade. RESULTS: 18 patients showed predominantly occult CNVM, six patients had predominantly classic CNVM, and 15 showed subretinal haemorrhage. At the 12 month follow up 13 patients (33%) showed an improvement in visual acuity of more than three lines (logMAR scale), 18 patients (46%) retained stable visual acuity with a change of equal or less than three lines (logMAR scale), and eight patients (21%) showed a decrease in visual acuity of more than three lines (logMAR scale). Recurrence of CNVM was observed in three (8%) eyes at 5-11 months postoperatively. Other complications included proliferative vitreoretinopathy with retinal detachment (n=10), peripheral epiretinal membranes (n=9), macular pucker (n=2), corneal decompensation (n=2), and hypotony (n=11). 18 patients (46%) complained about persistent diplopia. CONCLUSION: Macular translocation surgery is able to maintain or improve distant vision in the majority of patients with exudative ARMD. Proliferative vitreoretinopathy and diplopia are the two major complications. A prospective randomised controlled trial comparing macular translocation with observation for patients with the occult form of exudative ARMD may be justified.

Aged↗

Residual binocular interactions in the striate cortex of monkeys reared with abnormal binocular vision.

We investigated the nature of residual binocular interactions in the striate cortex (V1) of monkey models for the two most common causes of visual dysfunction in young children, specifically anisometropia and strabismus. Infant rhesus monkeys were raised wearing either anisometropic spectacle lenses that optically defocused one eye or ophthalmic prisms that optically produced diplopia and binocular confusion. Earlier psychophysical investigations had demonstrated that all subjects exhibited permanent binocular vision deficits and, in some cases, amblyopia. When the monkeys were adults, the responses of individual V1 neurons were studied with the use of microelectrode recording techniques while the animals were anesthetized and paralyzed. The manner in which the signals from the two eyes were combined in individual cells was investigated by dichoptically stimulating both eyes simultaneously with drifting sine wave gratings. In both lens- and prism-reared monkeys, fewer neurons had balanced ocular dominances and greater numbers of neurons were excited by only one eye. However, many neurons that appeared to be monocular exhibited clear binocular interactions during dichoptic stimulation. For the surviving binocular neurons, the maximum binocular response amplitudes were lower than normal; fewer neurons, particularly complex cells, were sensitive to relative interocular spatial phase disparities; and the remaining disparity-sensitive neurons exhibited lower degrees of binocular interaction. In prism-reared monkeys, an unusually high proportion of complex cells exhibited binocular suppression during dichoptic stimulation. Binocular contrast summation experiments showed that for both cooperative and antagonistic binocular interactions, contrast signals from the two eyes were combined by individual neurons in a normal linear fashion in both lens- and prism-reared monkeys. The observed binocular deficits appear to reflect a reduction in functional inputs from one eye and/or spatial imprecision in the monocular receptive fields rather than an aberrant form of binocular interaction. In the prism-reared monkeys, the predominance of suppression suggests that inhibitory connections were, however, less susceptible to diplopia and confusion than excitatory connections. Overall, there were many parallels between V1 physiology in our monkey models and the residual vision of humans with anisometropia or strabismus.

Animals↗

Ancillary procedures in microscopic endonasal orbital decompression for thyroid-associated ophthalmopathy.

In 35 patients with thyroid-associated orbitopathy, nonresponsive to conservative treatment, an endonasal microscopic approach with bimural osteotomy was performed for decompression in two ways. While in group A the periorbita was resected, and fat septa were cut, in group B periorbital strips were left, and fat septa were respected. In accordance with other authors, resection of the periorbital and cutting fat septa will improve vision and proptosis, but in up to 30% of the patients de novo diplopia occurs. Our data favor the assumption that a more conservative endonasal microscopic decompression of the orbita leaving periorbital strips and fat septa achieves similar good results for reduction of proptosis and visual gain but creates less often de novo diplopia in primary gaze. Based on our experience, rehabilitation for thyroid-associated arbitopathy comprises as a first step orbital endonasal decompression with cooperation of the ophthalmologist, then if necessary as a second step strabismus surgery, and thirdly eyelid repair for scleral show.

Adult↗

Characterization of incident stroke signs and symptoms: findings from the atherosclerosis risk in communities study.

BACKGROUND AND PURPOSE: Although patterns of stroke occurrence and mortality have been well studied, few epidemiological data are available regarding the clinical characteristics of stroke events. METHODS: We evaluated hospitalized stroke events reported in the Atherosclerosis Risk in Communities (ARIC) Study to describe the clinical characteristics of incident stroke. Confirmed stroke cases (n=474) were evaluated for stroke symptoms (headache, vertigo, gait disturbance, convulsions) and stroke signs (hemianopia, diplopia, speech deficits, paresis, paresthesia/sensory deficits) and their univariate associations with race, sex, and stroke subtype. RESULTS: Over 9.2 years of follow-up, 402 (85%) ischemic and 72 (15%) hemorrhagic strokes occurred. Frequency of stroke symptoms (95% CIs) were as follows: headache (27.4%; 23.4% to 31.4%), gait disturbance (10.8%; 7.9% to 13.6%), convulsions (4.4%; 2.6% to 6.3%), and vertigo (2.1%; 0.8% to 3.4%). Speech deficits occurred in 24.0% (20.2% to 27.9%), hemianopia in 14.6% (11.4% to 17.7%), and diplopia in 5.5% (3.4% to 7.5%) of cases. Most cases involved paresis (81.6%; 78.1% to 85.1%), while fewer cases experienced sensory deficits (44.5%; 40.0% to 49.0%). Blacks were more likely than whites to experience paresis (85.4% versus 78.2%; P=0.044). Men were more likely than women to experience a gait disturbance (14.4% versus 6.7%; P=0.007). Persons with hemorrhagic strokes had a higher proportion of headaches (55.6% versus 22.4%; P=0.001) and convulsions (11.1% versus 3.2%; P=0.003) than those with ischemic events, while speech and sensory deficits were more common in ischemic strokes (26.1% versus 12.5%, P=0.013, and 49.0% versus 19.4%, P=0.001, respectively). CONCLUSIONS: We present epidemiological data concerning the clinical characteristics of incident stroke in a population-based cohort. Although minor differences by race, sex, and stroke subtype were observed, data from additional follow-up are required to confirm observed variations.

Arteriosclerosis↗

Orbital wall fractures: evaluation and management.

Over a 49-month period, 121 orbital wall fractures were treated and 92 were followed for a median of 6.5 months (minimum, 3 months). Associated injuries included a 17% incidence of serious globe or optic nerve injuries and 13% incidence of lacrimal drainage disruption. Diplopia occurred in 23% and dystopia in 11%. Management was by observation alone in 14% and exploration in the remainder, with layered gelfilm for defects smaller than 4 cm2, alloplastic sheeting for defects to 6 cm2, and outer cortex of parietal bone for larger dehiscences. There were no decrements in vision from operation, dystopias were corrected to within 2 mm of normal, and diplopia persisted only in those with extraocular muscle paresis. There was no benefit to exploration of orbital wall defects smaller than 2.5 cm2 or with reduction of other midfacial fractures (e.g., malar) when neither dystopia nor entrapment was present, because defects not obturated in such cases had no sequelae.

Adult↗

Medial orbital wall fractures: classification and clinical profile.

This article reports our experience and proposes a clinical classification regarding medial orbital wall fractures. After a retrospective analysis of 2741 patients with facial fractures, we were able to evaluate 273 patients with 304 medial orbital wall fractures. The male-to-female ratio was 5:1, and most injuries involved the left orbit. Most fractures were caused by personal altercations, but more complex injuries were noted with automobile accidents and falls. Fractures were divided into types based on location and severity of injury: type I (confined to the medial orbital wall), type II (medial orbital wall continuous with floor), type III (medial orbital wall with floor-malar fractures), and type IV (medial orbital wall and complex midfacial injuries). Although visual loss (2%), diplopia (41%), and enophthalmos (12%) were seen, diplopia and enophthalmos were commonly observed with type II injuries. Imaging studies showed that about 52% of the fractures were associated with prolapse of orbital fat, but only 43% could be diagnosed with plain x rays. Type I fractures were generally explored through a frontoethmoid incision; other types were treated with subciliary or transconjunctival approaches. The usual treatment consisted of repositioning the fragments and repair of the wall with polyethylene mesh or cranial bone graft. Type I and type II fractures seemed best explained by the hydraulic mechanism of injury, whereas the type III and type IV fractures best fitted the buckling theory.

Accidents, Traffic↗

Burkitt's lymphoma presenting as oculomotor palsy in an hiv-positive patient.

PURPOSE: To report a case of Burkitt's lymphoma (BL) in an HIV-positive patient presenting as complete third nerve palsy. METHODS: Interventional case report. A 34-year-old man presented with headache, left eye pain, diplopia, and complete ptosis of several hours' duration. Left eye examination disclosed complete third nerve palsy with pupillary involvement. Significant hepatomegalia was noted at physical examination and computed tomography (CT) scan of the abdomen showed multiple solid lesions. RESULTS: Liver biopsy was consistent with Burkitt's lymphoma. Bone marrow biopsy was normal. Brain imaging was normal. Work-up also revealed positivity for human immunodeficiency virus. Chemotherapy treatment was started. Two months later, ocular motility examination was normal. CONCLUSIONS: Although BL affects the central nervous system very rarely, BL should be considered in any immunosuppressed patient presenting with diplopia or ophthalmoparesis.

Adult↗

Vertigo and Parinaud's syndrome as presentation of Churg-Strauss syndrome.

Intracranial complications are rare in Churg-Strauss syndrome (CSS). Cerebral infarctions are the most common intracranial presentation, usually after the clinical diagnosis of CSS had been established. We present a case of vertigo and Parinaud's syndrome as presentation of CSS in an asthmatic patient. Clinical examination revealed upward gaze limitation and bilateral midriasis. A cranial computed tomography scan showed a small round hypoattenuating lesion located in the right thalamic-mesencephalic region, which was later confirmed by magnetic resonance imaging. There was eosinophilia of more than 50%, and p-ANCA were positive. After steroid treatment was started, vertigo and diplopia resolved, and eosinophilia was reduced. After 24 months follow-up, the patient remains stable, with negative p-ANCA, taking 20 mg prednisone daily. Further magnetic resonance exams have shown findings that were similar to those of previous studies. This case shows how vertigo and transient diplopia may be the first symptoms of neurological complications in patients with CSS.

Adult↗

Botulinum toxin treatment of supranuclear ocular motility disorders.

We treated one patient with bilateral internuclear ophthalmoplegia and another with skew deviation with extraocular muscle botulinum toxin injection. Both patients had pre-injection symptomatic diplopia in primary position, one for 1 month and the other for 12 months. Resolution of diplopia and complete and permanent binocular fusion in primary position was obtained within 3 to 4 days in both cases.

Aged↗

Role of anticardiolipin antibodies in young persons with migraine and transient focal neurologic events: a prospective study.

Anticardiolipin antibodies (aCL) are a risk factor for cerebral ischemia. In migraine, the association is controversial, with widely varying results in different small series. The controversy in part may be due to the inherent difficulty in distinguishing the transient focal neurologic events (TFNE) of migraine from TIA. To assess the frequency of aCL in migraine, we prospectively evaluated consecutive adults under 60 years of age with migraine without aura and with recent TFNE (<24-hour duration) clinically suggestive of either migraine with aura or TIA. We concomitantly enrolled persons with no CNS disease. Each person was interviewed and had blood drawn for solid-phase ELISA with IgG and IgM aCL isotyping. Neuroradiologic studies were reviewed. Patients with TFNE were followed every 6 months for the duration of the 3-year study. The frequency of aCL positivity (IgG >20, IgG >40, IgM >7.5) for the 645 patients with TFNE (8.8, 3.1, 4.2%), the 518 persons in the TFNE subgroup with migraine with aura (8.9, 3.3, 4.1%), the 497 persons with migraine without aura (7.0, 2.0, 3.6%), and the 366 control subjects (9.3, 3.6, 3.9%) did not differ significantly between groups. In TFNE patients with elevated aCL titer, the association was positive with diabetes mellitus, TFNE duration <15 minutes, and diplopia and was negative with hemiparesis, tinnitus, and family history of stroke. Findings on imaging consistent with cerebral ischemia were more frequent in aCL-positive persons. The short-term risk of stroke was uniformly low. In young persons, aCL is not associated with migraine or with TFNE, although diabetes mellitus, negative family history of stroke, and brief duration of symptoms (including diplopia) may predict immunoreactivity. Imaging studies suggest an ischemic etiology of TFNE in this cohort.

Adolescent↗

Long-term follow-up and recent observations on 305 cases of orbital decompression for dysthyroid orbitopathy.

Dysthyroid exophthalmopathy (orbitopathy) results from an enlargement of extraglobal orbital structures, producing ocular proptosis, optic nerve compression, and corneal exposure. Treatment with corticosteroids and radiation may be beneficial; refractory cases require surgical decompression of the orbit. Transantral orbital decompression was described by Walsh and Ogura and has been performed in over 350 patients at this institution. A review of 305 patients with long-term follow-up was performed. Visual acuity improved or was maintained at preoperative levels in over 95% of the patients, with ocular recession ranging from 1 to 12 mm (average: 4 mm). Postoperative ocular balance of relative exophthalmos was to within 1 mm in 76% of the patients and to within 2 mm in approximately 90% of the patients. Normal postoperative extraocular muscle balance was present in 99 patients. Immediate postoperative diplopia was noted in 206 patients. Long-term follow-up revealed that in 137 of these patients, diplopia resolved or responded to conservative management. Extraocular muscle surgery was required for correction in 69 patients. Twenty-seven patients had postoperative complications. These included 16 patients with hypesthesia of the infraorbital nerve, 5 patients with sinusitis, 3 patients who had incomplete decompression, 2 patients with oral antral fistulae, and 1 patient who had CSF rhinorrhea. Five patients, despite surgery, radiation, and steroid therapy progressed to blindness. We conclude that this procedure is effective and carries few complications. Orbital imaging, using computed tomography or magnetic resonance sequence with reconstructive capabilities, permits early diagnosis and treatment of dysthyroid compression optic neuropathy.

Adolescent↗

[Myasthenia gravis. Clinical evaluation of 153 patients].

We have retrospectively analysed the records of patients diagnosed as having myasthenia gravis and followed up in our department from February 1973 to March 1995. The main clinical findings were ptosis, diplopia, dysphagia, dysphonia or dysarthria, mastigatory impairment, dyspnea, asthenia, weakness of the cervical muscles and of the extremities, as well as findings of the physical and neurological examination. Based on the information collected, the patients were classified clinically according the modified Osserman-Gerkins scale, but considering the non-dynamic aspects of that scale, we used the modified functional scale of Niakan and classified the patients clinically as follows: remission, controlled, stable, partially controlled, poorly controlled, no response. We collected 153 patients, 104 (68.0%) females and 49 (32.0%) males producing a female/male ratio of 2.2:1. The duration of the disease varied from seven days to 27 years, mean 6.26 years (+/- 5.44). Age at the first symptoms varied between 24 hours to 80 years, mean of 32.13 years (+/- 19.48). We had 30 patients ranging from 0 to 15 years of age, 91 patients were observed between 15 and 50 years and 32 patients after the age of 50 years. Above the age of 60 years, the disease aflicts males more than females with a ratio of 1.5:1. The acquired autoimmune form with generalized weakness was the most frequent presentation. However, ocular muscle weakness with ptosis and diplopia were the most common clinical presentation in our series.

Adolescent↗

Surgical treatment of endocrine exophthalmos by removal of orbital fat: clinical experience.

PURPOSE: To report a series of 73 patients with endocrine exophthalmos treated by removal of orbital fat using the transpalpebral approach during the period 1989 to 1999. METHODS: The operation was performed according to the technique described by Olivari. Aesthetic analysis was done preoperatively and postoperatively (more than 6 months after surgery). The number of complications was also observed. RESULTS: The average volume of resected fat was approximately 7.6 mL per orbit. No major complication was observed. In 9 patients with epiphora, all improved. One patient developed postoperative diplopia and 5 complained of temporary diplopia. Appearance improved in 62 patients (85%). CONCLUSION: Surgical removal of orbital fat associated with endocrine exophthalmos provides consistent improvement in appearance with a low rate of complications. Additional procedures may be indicated to improve the cosmetic outcome.

Adipose Tissue↗