Visual Impairment and Functional Classification in Children with Cerebral Palsy.

BACKGROUND: Cerebral palsy (CP) is the most common motor impairment in childhood and often accompanied by a broad spectrum of comorbidities. Data are sparse concerning visual impairment (VI) and functional classification among CP children. OBJECTIVE: The objective of this study was to analyze the prevalence of VI among children with CP and to investigate a possible association between VI and Gross Motor Function Classification System (GMFCS) and the Bimanual Fine Motor Function (BFMF). METHODS: In this hospital-based study, records of 200 children with CP aged 2 to 17 years were reviewed. RESULTS: Overall, VI was found in 59.5% of children with CP. Prevalence of VI was higher when compared with non-CP children. A correlation between GMFCS as well as BFMF and severity of VI was found. Children with severe CP were at greater risk for severe VI, especially cerebral VI compared with children with mild CP. CONCLUSION: VI is a significant problem in children with CP and is correlated with motor function. Children with CP should undergo detailed ophthalmologic and orthoptic assessment to enable early intervention.

Intraorbital plexiform neurofibroma in an NF-1-negative patient.

A 52-year-old patient presented with an orbital swelling and exophthalmos that enlarged over a period of about 40 years. The clinical examination showed massive exophthalmos and ptosis of the right eye without diplopia. The radiological investigation (MRI, CT and ultrasound) showed an unclear intraorbital mass with erosion of the orbital floor, infraorbital rim and orbital roof. The lesion was diagnosed histologically as a plexiform neurofibroma. The patient did not present any features of neurofibromatosis type 1 (NF-1) and molecular genetic analysis was unable to uncover a pathogenic sequence alteration in the NF-1 gene. Owing to the absence of clinical and ophthalmologic symptoms and the improbability of complete removal, the patient refused surgical intervention.

Use of the European preliminary criteria, the Breiman-classification tree and the American-European criteria for diagnosis of primary Sjögren's Syndrome in daily practice: a retrospective analysis.

This study was conducted to assess the use of the European preliminary criteria, the Breiman-classification tree and the American-European criteria for diagnosis of primary Sjögren's Syndrome (pSS) in daily practice. A retrospective analysis of 17 consecutive patients with pSS (European criteria) was performed evaluating the application of the Schirmer test, semiquantitative sialoscintigraphy, immunologic tests, including rheumatoid factor, antinuclear antibodies, Sjögren's syndrome autoantibodies (SS-A, SS-B) and lip biopsy. Out of the 17 patients with pSS according to the European criteria, 15 patients fulfilled the classification tree (=88.2%), and 4 patients fulfilled the American-European criteria (=23.5%, P = 0.001). In the four patients fulfilling the American-European criteria, a positive result of the sialoscintigraphy was not crucial for the diagnosis according to these criteria. In conclusion, the American-European criteria are more stringent than the European preliminary criteria. We assume the role of sialoscintigraphy to be reduced when applying the American-European criteria.

[Post-traumatic ocular motility in orbital floor fractures]. / Posttraumatische Bulbusmotilität bei Orbitabodenfrakturen.

PURPOSE: The aim of this study was to evaluate post-traumatic ocular motility in orbital floor fractures in relation to the severity of the fracture and concomitant injuries. MATERIAL AND METHODS: Over a period of 6 years, data from 435 patients with orbital floor fractures were re-evaluated. Ocular motility was divided into abduction, adduction, upgaze and downgaze movements. RESULTS: In 54.3% of the cases, there was a reduction in ocular motility. A reduced motility with a movement >20 degrees was found in most of the cases. Concomitant injuries to the eye resulted in a significant increase in the reduction of motility. This reduction depended on the severity of the orbital floor fracture. A reduced upgaze movement was the most common complication in all types of fractures. CONCLUSION: Ocular motility can act as an indication of the severity of the orbital fracture. Concomitant injuries to the eye resulted in a higher incidence of reduced ocular motility.

[Diagnosis and therapy of Graves' ophthalmopathy--consensus guidelines]. / Diagnostik und Therapie der endokrinen Orbitopathie--Konsensusrichtlinien.

On 22-09-2001 the VIth Grazer Hormonsymposion took place. Diagnosis and therapy of Graves' Ophthalmopathy was discussed in an interdisciplinary way by endocrinologists, surgeons and ophthalmologists. The results of the round-table discussion and the consensus talk are presented.

Substance P and vasoactive intestinal polypeptide in the streptozotocin-induced diabetic rat retina.

PURPOSE: Little knowledge exists about how neurotransmitters behave in the diabetic retina. In this study, the authors measured the concentration of two neuropeptides, substance P and vasoactive intestinal polypeptide, in the streptozotocin-induced diabetic rat retina in a time-dependent manner. METHODS: The retinas of 1-, 3-, 5-, 8-, and 12-week diabetic rats were processed using a highly sensitive radioimmunoassay for both substance P and vasoactive intestinal polypeptide. Furthermore, the peptide-immunoreactivities were characterized by high-pressure liquid chromatography. RESULTS: Substance P and vasoactive intestinal polypeptide were found to be significantly reduced with a maximum decrease of 28.6% (+/-6.7) and 64.5% (+/-10.7) after 5 weeks, respectively. The peptide-immunoreactivities were found in a major peak coeluting with the synthetic peptides indicating that the quantitative values measured by radioimmunoassay represent the authentic peptides. CONCLUSIONS: The reduction of substance P and vasoactive intestinal polypeptide is in clear contrast to the amino acid transmitters GABA and glycine, which have been shown to be elevated in this early stage of diabetic retinopathy. This finding is important for three reasons: First, the decrease may result in reduced excitability of inner retinal neurons, as both peptides are known to modulate the excitability of these neurons; second, the decrease may be the consequence of a depressing and/or damaging effect by excitotoxins; and third, it may help explain why neovascularizations do not occur in this animal model, although VEGF is massively upregulated, as substance P is a very potent vascular growth factor.

Laser stimulation of acupuncture point P6 reduces postoperative vomiting in children undergoing strabismus surgery.

We conducted a double-blind, randomized, placebo-controlled study to investigate the effectiveness of P6 acupuncture on postoperative vomiting in children undergoing strabismus surgery. Acupuncture was performed by laser stimulation with a low-level laser. Laser stimulation of P6 was administered 15 min before induction of anaesthesia and 15 min after arriving in the recovery room. In the laser stimulation group, the incidence of vomiting was significantly lower (25%) than that in the placebo group (85%).

Evaluation of immunological mechanisms mediating thyroid-associated ophthalmopathy by radionuclide imaging using the somatostatin analog 111In-octreotide.

Thyroid associated ophthalmopathy (TAO) is a disorder involving the soft tissues and extraocular muscles of the orbit seen mainly in cases of Graves' disease. Although an immunogenic pathogenesis has been proposed, the actual mechanisms of the in vivo retrobulbar involvement are not well defined. The recent introduction of the 111In-labeled somatostatin analog, octreotide, which can bind in vivo to the cell membrane of activated lymphocytes expressing somatostatin receptors, has provided a new investigational tool for diseases with a presumed immunological background. Based on this property, octreotide scans can be expected to be positive in cases of immunological disease showing tracer accumulation within affected sites. The aim of this study was to evaluate the utility of scintigraphic imaging with octreotide of the retrobulbar space in cases of TAO, including sequential studies of patients undergoing immunosuppressive therapy. We studied a series of 51 patients who had Graves' disease with varying degrees of TAO. Nine patients had received immunosuppressive therapy. The degree of orbital inflammation was classified according to the clinical activity score of Mourits. Both planar and tomographic imaging of the orbit were carried out using 111 MBq of the 111In-labeled octreotide (OctreoScan) 2 h after tracer injection. A significant tracer accumulation in the retrobulbar space was seen in all 20 patients with a high activity score, in 8 of 16 cases with a negative score, and in 11 of 20 cases with an intermediate Mourits' score. In cases of persistent eye disease in spite of immunosuppressive therapy, the octreotide scan remained positive. Successful therapy either with prednisolone, external radiation, or i.v. immunoglobulins showed a significant diminution of tracer uptake after finishing the therapeutic regime. Three-dimensional reconstruction of the images also revealed a significant tracer accumulation in the areas of the lacrimal gland, the nasal region, and the pituitary. Controls cases (n = 30) showed no uptake in the orbital region. We conclude that 111In octreotide scintigraphy is an objective method that identifies patients with active inflammatory eye disease, i.e., having significant tracer uptake in the retrobulbar space. This uptake appears to reflect an immunological process, since immunosuppressive therapy will significantly decrease tracer accumulation.

Endocrine ophthalmopathy in a patient under continuous immunosuppressive therapy after cardiac transplantation.

We present the case of a female patient who has been on immunosuppressive therapy consisting of cyclosporin A and prednisolone for 9 years because of heterotopic (auxiliary) heart transplantation in 1984. In 1992 the patient developed Graves' disease followed by endocrine ophthalmopathy class IV 1 year later. To our knowledge this is the first report on Graves' disease with subsequent severe endocrine ophthalmopathy in a patient under immunosuppressive treatment with cyclosporin A and prednisolone in doses that effectively prevent heart transplant rejection. Prednisolone, which is used as a first line treatment of endocrine ophthalmopathy, and cyclosporin A, both inhibit T cell function. However, in this patient they were not effective in preventing the development of Graves' disease with subsequent endocrine ophthalmopathy, both of which are autoimmune diseases.

A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate.

We report a family with six patients suffering from a sphingomyelinase-deficient form of Niemann-Pick disease, all presenting with a visceral course of the disease. Retinal changes classified as macular halos in four members indicated neuronal storage and therefore an intermediate type of the disease. For further classification of the biochemical type, [choline-methyl-14C]sphingomyelin degradation studies were carried out in fibroblast cultures of all six members. The low degradation rates measured were similar to those usually found in the neuronopathic form (type A) of Niemann-Pick disease. This family illustrates the broad heterogeneity within the sphingomyelinase deficiency group of the Niemann-Pick disease. Apparently the finding of a low sphingomyelin degradation rate in fibroblast cultures does not necessarily imply a typical serious and lethal course of the disease.