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1.
Klin Monbl Augenheilkd ; 229(3): 241-5, 2012 Mar.
Article in German | MEDLINE | ID: mdl-22105538

ABSTRACT

BACKGROUND, MATERIAL AND METHODS: 1. An evaluation of medical findings and photodocumentation of 6 patients with pseudoexfoliation (PEX) material on the anterior surface of posterior chamber intraocular lenses was undertaken. Molecular genetic analysis of mutations in LOX-L1 was performed in order to confirm the association with pseudoexfoliation syndrome or glaucoma. Age of patients, the maximal intraocular pressure and perimetry with Octopus and Goldmann perimeters were documented as well as the time between implantation of the posterior chamber lens and diagnosis of pseudoexfoliation material on the anterior surface of posterior chamber lens. 2. Consecutive examinations of 35 patients with pseudoexfoliation syndrome or -glaucoma and pseudophakia were made to evaluate the frequency of patients with pseudoexfoliation material on the anterior surface of posterior chamber lenses. RESULTS: 1. The characteristic formation of stripe-shaped peripheral pseudoexfoliation material is seen in all examined patients on the anterior surface of posterior chamber lenses, but there is no central homogeneous round zone of pseudoexfoliation material in all patients. 2. the mean observation time of patients with pseudophakia and pseudoexfoliation syndrome or glaucoma is 4.4 ± 3.9 years. 5.7 % of the patients show pseudoexfoliation material in the periphery of posterior chamber lenses. The mean time between implantation of the intraocular lens and diagnosis of pseudoexfoliation material on the lenses is 3 years. CONCLUSIONS: The lack of a central homogeneous round zone of pseudoexfoliation material deposits on the anterior surface of posterior chamber lenses seems to be characteristic. The change in topography of PEX material on intraocular lenses is described here for the first time. A knowledge of this change in the topography of pseudoexfoliation material in pseudophakia is important for glaucoma screening, because pseudoexfoliation deposits can only be detected in mydriasis due to the peripheral location on the intraocular lens. Due to the old age of patients at the onset of pseudoexfoliation deposits, a pseudoexfoliation syndrome frequently is likely to develop after cataract surgery in many patients.


Subject(s)
Exfoliation Syndrome/complications , Exfoliation Syndrome/pathology , Mass Screening/methods , Ophthalmoscopy/methods , Pseudophakia/complications , Pseudophakia/diagnosis , Aged , Female , Humans , Male , Middle Aged , Reproducibility of Results , Sensitivity and Specificity
2.
Clin Genet ; 74(5): 476-80, 2008 Nov.
Article in English | MEDLINE | ID: mdl-18498376

ABSTRACT

Peters anomaly and Axenfeld-Rieger syndrome (ARS) belong to the overlapping spectrum of disorders summarized as anterior segment dysgenesis (ASD). Five patients from a family with Peters' anomaly and ARS were screened for mutations in the PITX2, CYP1B1 and FOXC1 genes by direct sequencing. All affected family members examined were heterozygous for a single nucleotide substitution, resulting in a nonsense mutation (Q120X) at a highly conserved residue of the FOXC1 gene that is essential for DNA binding. In this pedigree, all affected family members were diagnosed with ARS except for one who shows bilateral Peters' anomaly. Our findings support the role of FOXC1 mutations in the spectrum of ASD.


Subject(s)
Abnormalities, Multiple/genetics , Anterior Eye Segment/abnormalities , Eye Abnormalities/genetics , Forkhead Transcription Factors/genetics , Mutation , Aryl Hydrocarbon Hydroxylases , Base Sequence , Cytochrome P-450 CYP1B1 , Cytochrome P-450 Enzyme System/genetics , DNA Mutational Analysis , Family , Homeodomain Proteins/genetics , Humans , Iris/abnormalities , Molecular Sequence Data , Pedigree , Point Mutation , Syndrome , Transcription Factors/genetics , Homeobox Protein PITX2
3.
Ophthalmologe ; 105(7): 693-710, 2008 Jul.
Article in German | MEDLINE | ID: mdl-18592250

ABSTRACT

Optic disc drusen (ODD) are an initially symptom-free, usually bilateral, progressive type of optic neuropathy with genetic disposition. We observed visual field loss (VFL) in 64% (49 of 77) of our patients. Vascular complications such as AION are common. Initially, papilledema is often diagnosed and the diagnosis of ODD is confirmed by means of ultrasound examination. We found an average time interval of 7 years between groups of patients with preperimetric stage and onset of VFL. We also observed more advanced stages of VFL, faster progression of visual field loss and greater visibility of ODD with increasing age. VFL frequently results in loss of driving ability according to driving permission regulations. The aim of our therapy concept is to reduce intraocular pressure (IOP). In a pilot study we found that patients who underwent IOP-lowering therapy showed significantly less progression of VFL than patients who did not undergo this therapy. The present article summarizes current knowledge on epidemiology, pathogenesis, clinical presentation, diagnostics and therapy of optic disc drusen.


Subject(s)
Ocular Hypertension/diagnosis , Ocular Hypertension/therapy , Optic Disk Drusen/diagnosis , Optic Disk Drusen/therapy , Vision Disorders/diagnosis , Vision Disorders/prevention & control , Humans , Ocular Hypertension/complications , Optic Disk Drusen/complications , Vision Disorders/etiology
4.
Ophthalmologe ; 103(5): 393-400, 2006 May.
Article in German | MEDLINE | ID: mdl-16683168

ABSTRACT

PURPOSE: The Axenfeld-Rieger syndrome (ARS) shows genetic and morphologic heterogeneity and is associated with glaucoma in 50% of the patients. METHODS: Ocular, dental, and systemic anomalies, maximum intraocular pressure (IOPmax), frequency of ARS or glaucoma in the family history (FH), and age at diagnosis (AAD) of 26 consecutively examined patients with ARS and glaucoma or elevated IOP were evaluated retrospectively. RESULTS: In 65.4% of the patients hypoplasia of the iris was found. Almost 50% of the patients had systemic anomalies, dental anomalies being the most frequent. Of 26 patients, 12 (46.2%) had an ARS in the FH; 57.7% of the patients had a FH of glaucoma with or without ARS. No significant differences in IOPmax and frequency of iris hypoplasia and glaucoma surgery were found when patients with and without ARS in their FH and patients with and without iris hypoplasia were compared. CONCLUSION: Patients with iris hypoplasia and patients with ARS and/or glaucoma in the FH do not show a worse glaucoma prognosis than patients without iris hypoplasia or without a FH of glaucoma or ARS. ARS was diagnosed within the 1st year of life in approximately half of the patients. Therefore, children of ARS patients should be screened as soon as possible to improve early diagnosis of ARS and glaucoma and to improve glaucoma prognosis. As the dental and facial anomalies may require treatment in the first dentition, patients with ARS should be referred to a dentist or orthodontist.


Subject(s)
Abnormalities, Multiple/genetics , Glaucoma/genetics , Ocular Hypertension/genetics , Abnormalities, Multiple/diagnosis , Chromosome Aberrations , Chromosomes, Human, Pair 13 , DNA Mutational Analysis , Diagnosis, Differential , Forkhead Transcription Factors/genetics , Genes, Dominant , Genetic Testing , Glaucoma/diagnosis , Glaucoma/surgery , Homeodomain Proteins/genetics , Humans , Iris/abnormalities , Manometry , Maxillofacial Abnormalities/diagnosis , Maxillofacial Abnormalities/genetics , Ocular Hypertension/diagnosis , Ocular Hypertension/surgery , Prognosis , Retrospective Studies , Syndrome , Tooth Abnormalities/diagnosis , Tooth Abnormalities/genetics , Transcription Factors/genetics , Homeobox Protein PITX2
5.
Clin Biochem ; 20(1): 43-6, 1987 Feb.
Article in English | MEDLINE | ID: mdl-3032478

ABSTRACT

Serum carboxypeptidases N (EC. 3.4.17.3) were determined spectrophotometrically in 87 patients with disturbances of thyroid function and in 131 euthyroid individuals, including 33 women taking estrogens for contraception. Carboxypeptidases N (CN) can be subdivided into CN1 and CN2, according to variable substrate affinity. In addition, measurements of blood pressure and in vitro tests of thyroid function were performed. In euthyroid controls, CN1 was negatively correlated with age. No significant differences between CN1 and CN2 have been observed with regard to sex. In patients with hyperthyroidism, the mean values of both enzymes were elevated, but this tendency proved to be significant only for CN2. In hypothyroid patients CN1 and CN2 levels were normal. Elevations of CN1 and CN2 in the hyperthyroid state seem not to be related to underlying immunological processes but to the thyroid hormone excess itself. In euthyroid women taking estrogens for contraception CN2 was also elevated.


PIP: The serum enzymes called carboxypeptidase N that split arginine or lysine from certain peptides, here termed CN1 and CN2, were determined in patients with thyroid disorders and in women taking oral contraceptives. The assays were done spectrophotometrically using synthetic hippuryl-substrates. The normal ranges in euthyroid subjects were 13-49 Units/1 for CN1 and 71-181 for CN2. In normal subjects, CN1 was inversely correlated with age. In hyperthyroid patients, the levels of both enzymes were elevated, significant only for CN2. Mean CN2 was significantly elevated in 33 women taking oral contraceptives, compared to euthyroid controls, 148.6 u/1, but was not outside the normal range.


Subject(s)
Carboxypeptidases/blood , Lysine Carboxypeptidase/blood , Thyroid Diseases/enzymology , Arginine/analogs & derivatives , Arginine/metabolism , Contraceptives, Oral, Hormonal , Female , Humans , Hyperthyroidism/blood , Hyperthyroidism/enzymology , Hypothyroidism/blood , Hypothyroidism/enzymology , Lysine/analogs & derivatives , Lysine/metabolism , Male , Peptidyl-Dipeptidase A/blood , Thyroid Diseases/blood
6.
Am J Clin Oncol ; 17(1): 83-5, 1994 Feb.
Article in English | MEDLINE | ID: mdl-8311015

ABSTRACT

We assessed the efficacy and toxicity of alternating non-cross-resistant chemotherapy in the treatment of advanced NSCLC. Cycles of cisplatin, methotrexate, doxorubicin, and cyclophosphamide were alternated monthly with cisplatin and etoposide. Patients had measurable disease, ECOG performance status 0-3, no previous chemotherapy, and stage II (inoperable), III, or IV disease without brain metastases. Between 1988 and 1990, 28 patients were entered in the study: 20 patients (71%) had stage IV disease, 19 (68%) were evaluable for response and toxicity; 4 (21%) responded. There were 3 partial responders (16%) and 1 complete responder (5%). The mean duration of response was 60.5 weeks (range: 32-105+ weeks), and the median time to progression was 12 weeks (range: 8-105+ weeks). The median survival time for all 28 patients was 24 weeks (range: 3-153+ weeks). The most significant toxicity was grade 3-4 leukopenia experienced by 63% of patients, but there were no episodes of sepsis and no treatment-related deaths. This regimen of alternating cycles of cisplatin-containing chemotherapy is safe, but its efficacy is not superior to other combination chemotherapy regimens.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Carcinoma, Non-Small-Cell Lung/drug therapy , Lung Neoplasms/drug therapy , Aged , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Cisplatin/administration & dosage , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Drug Administration Schedule , Etoposide/administration & dosage , Female , Humans , Male , Methotrexate/administration & dosage , Middle Aged , Survival Analysis , Treatment Outcome
7.
Ophthalmologe ; 90(6): 620-5, 1993 Dec.
Article in German | MEDLINE | ID: mdl-8124024

ABSTRACT

In earlier studies we found that visual field defects occur more frequently in the lower half of the visual field and that low systolic blood pressure occurs more frequently in low-tension glaucoma (stage II) compared to primary open-angle glaucoma (POAG) (stage II). We wanted to find out whether visual field defects in the lower half of the visual field point to insufficient perfusion of the optic nerve head due to low blood pressure. We therefore examined the visual fields of 153 eyes of 153 patients with POAG and regulated IOP with program 31 or 33 of the Octopus perimeter 201. With program Delta the loss per test point in the upper and lower hemifield was calculated. The mean systolic blood pressure was calculated for each patient from the blood pressure recordings during the observation time and over a long-term follow-up period (3-19 visual field examinations during a period of 1-8 years). With the Delta program we decided case by case whether the visual field showed a tendency to deteriorate or not. An asymmetry in the mean loss per test point between the two visual hemifields in a relation of 2:1 or more was found in 71 eyes out of 153. In the upper hemifield 50 out of 71 patients had two times greater loss per test point than in the lower hemifield. These patients showed a mean systolic blood pressure of 158 +/- 37 mmHg.(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Blood Pressure/physiology , Glaucoma, Open-Angle/physiopathology , Visual Fields/physiology , Adult , Aged , Aged, 80 and over , Female , Humans , Intraocular Pressure/physiology , Male , Middle Aged , Systole/physiology , Visual Field Tests
8.
Ophthalmologe ; 92(4): 564-73, 1995 Aug.
Article in German | MEDLINE | ID: mdl-7549349

ABSTRACT

UNLABELLED: The GG program pattern of the Humphrey Field Analyzer includes the 76 test points of the 30-2 program and an additional 52 test points in a glaucoma-specific distribution. In 18 test points in the nasal step area beyond 30 degrees the threshold is also determined. The findings obtained with the GG program and the 30-2 program were investigated by means of three questions: (1) Do the additional test points increase the sensitivity in ocular hypertension and glaucoma in stage I? For this purpose 41 eyes of 41 patients were examined. (2) Is the new grid useful for the determination of form and size of scotomas? For this purpose 46 eyes of 46 patients were examined. (3) What information is gained from a new asymmetry index, the glaucoma hemifield index (GHI), evaluated on the retinal threshold sensitivity of the GG program grid? To evaluate the information gained, the GHI of the test points in program 30-2 was compared to the GHI of the GG program in 11 eyes of 11 healthy persons, 21 eyes of 21 patients with ocular hypertension, and 32 eyes of 32 patients with primary open-angle glaucoma, stage I and II. RESULTS: (1) In ocular hypertension and glaucoma stage I, the higher grid density of the GG program leads to a higher sensitivity in comparison to program 30-2. (2) In the 46 eyes with scotomas in stages II-IV, the condensed grid allowed better assessment of form and size of them. Ninety-one percent of the eyes with glaucoma stage II-IV showed nasal scotomas out of 30 degrees excentricity, which were now detected with the GG program. (3) The GHI of program 30-2 and the GG program showed no significant difference in retinal threshold sensitivity in glaucoma stage I. The asymmetry of the visual field loss in glaucoma stage II with localized scotomas can be detected significantly better with the GHI of the GG program. At the beginning of visual field loss with diffuse sensitivity loss, the grid density of program 30-2 seems to be sufficient. In stage II with localized scotomas, the higher grid density of the GG program yield more information for the detection of hemifield asymmetries. After examination with the 30-2 program it seems to be useful to examine the 52 additional test points and combine these two with the print-out from the GG program.


Subject(s)
Glaucoma, Open-Angle/diagnosis , Ocular Hypertension/diagnosis , Software , Visual Field Tests/instrumentation , Adult , Aged , Aged, 80 and over , Female , Glaucoma, Open-Angle/classification , Glaucoma, Open-Angle/physiopathology , Humans , Intraocular Pressure/physiology , Male , Middle Aged , Ocular Hypertension/classification , Ocular Hypertension/physiopathology , Visual Fields/physiology
9.
Ophthalmologe ; 95(9): 625-32, 1998 Sep.
Article in German | MEDLINE | ID: mdl-9793384

ABSTRACT

METHODS: In 146 eyes/patients who underwent Nd:YAG laser iridotomy after glaucomatous attack or after prophylactic iridotomy, we evaluated whether the frequency of posterior synechiae depends on (1) glaucomatous attack, (2) preoperative miotic therapy, (3) postoperative antiglaucomatous therapy or (4) mode of intraoperative laser therapy. Out of 616 eyes with YAG iridotomy between 1983 and 1987, 146 eyes/patients fulfilled the inclusion criteria: observation time of minimum 3 months after iridotomy, preoperative examination without signs of preexisting synechiae, postoperative examination at discharge and a later control examination in mydriasis to exclude posterior synechiae. RESULTS: Eyes with and without glaucomatous attack and eyes with an without pre-operative long-term miotic therapy showed no significant difference in frequency of posterior synechiae. Eyes with postoperative long-term therapy with miotics or beta-blockers showed posterior synechiae significantly more often than eyes without post-operative miotic or beta-blocker therapy. Eyes that received DPE in the early post-operative period developed posterior synechiae significantly less often. The number of laser pulses and the mean total energy used were significantly higher in eyes which later developed posterior synechiae. In the group of patients with glaucomatous attack women outnumbered men by four to one, but there was no significant difference in refraction between women and men. CONCLUSIONS: Patients with glaucomatous attack are not at a higher risk of developing posterior synechiae than those without glaucomatous attack. Post-operative antiglaucomatous therapy, the number of laser pulses and the total energy alter the frequency of postoperatively developed posterior synechiae.


Subject(s)
Eye Diseases/etiology , Glaucoma/surgery , Iris/surgery , Laser Therapy , Postoperative Complications/etiology , Adult , Aged , Aged, 80 and over , Female , Follow-Up Studies , Humans , Lenses, Intraocular , Male , Middle Aged , Risk Factors
10.
Ophthalmologe ; 93(5): 527-34, 1996 Oct.
Article in German | MEDLINE | ID: mdl-9004874

ABSTRACT

Thirty-eight eyes of 38 glaucoma patients with a defined up-down asymmetry of visual field loss (VFL) were examined to ascertain whether there was corresponding up-down asymmetry of nerve fiber layer thickness (NFLT), calculated by laser polarimetry (LP). The correspondence was found to be closer for small up-down asymmetry of VFL then for medium or large up-down asymmetry. An approximately 6% greater NFLT in the lower than in the upper retinal half was revealed found by examination of 62 eyes of 62 normals. Considering this NFLT asymmetry in normals, there was a non-significant tendency towards correspondence between the up-down asymmetry of VFL and the up-down asymmetry of NFLT for the group of glaucoma patients with large up-down asymmetry of VFL. However, in about 40% of cases there was no such correspondence, so that staging of the glaucomatous disease has to be done by perimetry and is not possible by LP (software version 06/93).


Subject(s)
Glaucoma, Open-Angle/diagnosis , Glaucoma/diagnosis , Nerve Fibers/pathology , Ophthalmoscopy , Retina/pathology , Adolescent , Adult , Aged , Female , Glaucoma/classification , Glaucoma, Open-Angle/classification , Humans , Male , Middle Aged , Optic Disk/pathology , Reference Values , Visual Fields/physiology
11.
Ophthalmologe ; 92(4): 506-10, 1995 Aug.
Article in German | MEDLINE | ID: mdl-7549337

ABSTRACT

UNLABELLED: Does the disc parameter "mean pallor value" suggest a risk for further deterioration of visual field defects? PATIENTS: In 10 patients with ocular hypertension and 23 patients with primary open-angle glaucoma (Octopus 201, Humphrey perimeter) disc pallor was calculated by double examinations with the ONHA in 1987-1988, and a long-term follow-up of the visual field was performed. In all patients we also acquired information about the IOP level and visual field during the observation time from the ophthalmologists. Only one eye per patient was evaluated after randomized selection. RESULTS: In ocular hypertensive patients only 1 of 10 eyes showed deterioration of the visual field. In the glaucoma group 4 eyes out of 23 showed an increase in visual field loss. The eyes with a tendency to deteriorate had a higher mean pallor value compared with the mean value of the corresponding group [ocular hypertensives: 0.40 compared to 0.31 +/- 0.07 (0.02); POAG: 0.50 +/- 0.06 (0.03) compared to 0.33 +/- 0.08 (0.02); mean value +/- SE (SEM)]. The difference was statistically significant in the glaucoma group; Wilcoxon, Mann and Whitney U-test; P < 0.005. CONCLUSIONS: Taking into consideration all risk factors, the mean pallor value might help additionally to prognosticate the risk of deterioration of the visual field as far as 5-year follow-up allows this preliminary conclusion. The pallor value alone is not suitable for the follow-up of glaucoma patients because of the influence of increasing lens opacities and changes in the video system of the ONHA over years.


Subject(s)
Glaucoma, Open-Angle/diagnosis , Image Processing, Computer-Assisted/instrumentation , Ocular Hypertension/diagnosis , Ophthalmoscopes , Optic Disk/physiopathology , Visual Fields/physiology , Follow-Up Studies , Glaucoma, Open-Angle/physiopathology , Humans , Intraocular Pressure/physiology , Ocular Hypertension/physiopathology , Pilot Projects , Prognosis , Visual Field Tests/instrumentation
12.
Ophthalmologe ; 92(4): 521-5, 1995 Aug.
Article in German | MEDLINE | ID: mdl-7549340

ABSTRACT

In order to verify whether nerve fiber bundle defects can be detected by the confocal technique of the Heidelberg Retina Tomograph (HRT), we compared the localization of localized visual field defects with the localization of the expected corresponding nerve fiber bundle defect. We examined 105 patients: 49 with primary open-angle glaucoma (POAG), 26 with low-tension glaucoma (LTG) and 10 with ocular hypertension (OH), and as a control-group 20 healthy eyes. The glaucoma stage had been defined by examination of the central visual field with the Octopus Perimeter 201 program 31 or 32 and/or the Humphrey Perimeter GG program. In healthy eyes, eyes with OH and POAG stage I (n = 19), no nerve fiber bundle defect could be found. In stage II (n = 15) and stage III (n = 14) we found nerve fiber bundle defects in all patients with POAG and LTG (except for one patient with POAG stage III). In stage IV (n = 27) no localized nerve fiber bundle defects were detected in eyes with POAG, but in 4 of 7 patients with LTG, defects could be seen. In glaucoma stages II and III, nerve fiber bundle defects can be detected with the HRT. The control group shows, that the appearance of a nerve fiber bundle defect is no artifact caused by the HRT. However, examination of eyes with OH and POAG stage I also shows that this method is not sensitive enough to be useful for early diagnosis of glaucoma. Nevertheless, it may be possible to develop the confocal HRT method further as a nerve-fiber diagnostic method for glaucoma.


Subject(s)
Glaucoma/diagnosis , Lasers , Nerve Fibers/pathology , Optic Disk/pathology , Retina/pathology , Tomography/instrumentation , Visual Fields/physiology , Glaucoma/classification , Glaucoma/pathology , Glaucoma, Open-Angle/classification , Glaucoma, Open-Angle/diagnosis , Glaucoma, Open-Angle/pathology , Humans , Intraocular Pressure/physiology , Ocular Hypertension/classification , Ocular Hypertension/diagnosis , Ocular Hypertension/pathology , Reference Values , Sensitivity and Specificity
13.
Ophthalmologe ; 96(6): 364-9, 1999 Jun.
Article in German | MEDLINE | ID: mdl-10429493

ABSTRACT

PURPOSE: To verify whether nerve fiber bundle defects (NFBD) can be detected by the polarization technique of the Nerve Fiber Analyzer I (NFA I), we compared the localization of localized visual field (VF) defects with the localization of the expected corresponding NFBD. METHODS: We examined 25 eyes of 25 glaucoma patients. All examined glaucomatous eyes had localized VF defects stage II and III. The glaucoma stage was defined by examination of the central 30 degrees VF using computer perimetry. NFBD were determined by masked examination of NFA I images by three examiners. As controls 22 eyes of 22 normals were examined. RESULTS: In 18 (72%) of 25 glaucomatous eyes the three examiners agreed in their findings. In 15 of 25 glaucomatous eyes (60%) all three examiners found a NFBD, which corresponded to a localized VF defect. In 18 of 22 healthy eyes all three examiners found no NFBD. The first examiner had no false-positive results, the second three, and the third four. CONCLUSIONS: The three examiners often disagreed in their findings of NFBD, which indicates high subjective variability. Examiner-dependent specificity and sensitivity show that detection of glaucomatous NFBD is often not possible by laser polarimetry (software version 06/93).


Subject(s)
Glaucoma/diagnosis , Microscopy, Polarization/instrumentation , Nerve Fibers/pathology , Ophthalmoscopes , Optic Disk/pathology , Adult , Aged , Aged, 80 and over , Female , Glaucoma/pathology , Humans , Infrared Rays , Lasers , Male , Middle Aged , Sensitivity and Specificity , Visual Fields/physiology
14.
Ophthalmologe ; 106(12): 1116-20, 2009 Dec.
Article in German | MEDLINE | ID: mdl-19326122

ABSTRACT

BACKGROUND: Myotonic dystrophy (DM type 1) is the most common type of muscular dystrophy in adults, accompanied by myotonia, iridescent multicoloured posterior capsular lens opacities ("Christmas tree cataract", CTC), and, for example, cardiac arrhythmias and respiratory failure. A further feature is anticipation, which is the occurrence of increasing disease severity and decreasing age of onset in successive generations, depending on inherited unstable DNA sequences that become larger with each generation. AIM: To evaluate the frequency of clinically manifest DM in patients with CTC and the age at diagnosis of CTC. METHODS: Retrospective analysis of ophthalmologic, neurologic, and internal findings in 18 consecutively diagnosed patients with CTC. RESULTS: Three of 18 CTC patients showed clinical signs of DM. The mean age at diagnosis was 72,8+/-16,5 years (range 36-94 years). DISCUSSION: A relationship existed between CTC and manifest DM in 16.7% of the patients with CTC. A hypothesis states that the other 83.3% might have premutations in the DMPK gene, which might lead to a complete mutation after transmission through subsequent generations. In the meantime, molecular genetic DM tests are available. Because of clinical heterogeneity and the anticipation phenomenon in DM, confirmation with such molecular genetic DM tests should be obtained.


Subject(s)
Cataract/complications , Cataract/diagnosis , Myotonic Dystrophy/complications , Myotonic Dystrophy/diagnosis , Adult , Female , Humans , Male , Middle Aged , Risk Assessment , Risk Factors
15.
Klin Monbl Augenheilkd ; 191(3): 184-98, 1987 Sep.
Article in German | MEDLINE | ID: mdl-3682683

ABSTRACT

Visual field defects of stages I-IV, in 451 eyes of 451 glaucoma patients, were examined with the Octopus 201 perimeter: 83 patients with low-tension glaucoma (LTG), 316 patients with primary open-angle glaucoma (POAG), and 52 patients with pigmentary glaucoma (PG). Program 31 or 33 was used, with an eccentricity range of up to 30 degrees, 73 test points, and a 6 degrees grid. The mean total field loss (TL) as well as the mean loss per test point (TL/TP) in the 30 degrees field, in the field quadrants, and in the eccentricity ranges from 0-10 degrees, 10-20 degrees, and 20-30 degrees were calculated for each patient with Program Delta. In addition, for each visual field the quotient of total loss and of the number of disturbed test points was calculated, providing a measurement of the mean depth of the field defects. For definition and comparison of visual fields in the different glaucomas at identical stages of the disease according to the amount of TL, the 3 patient groups were subdivided into 4 stages of sensitivity loss: Stage 1: TL less than or equal to 100 dB; Stage 2: TL 101-400 dB; Stage 3: TL 401-800 dB; Stage 4: 801-1600 dB. The results of a comparison of eyes with LTG, POAG, and PG at the same stage of disease were as follows: I. Location of scotomas: 1. Defects more frequent in the lower field in LTG as compared to POAG in stage 2. 2. Defects more frequent in the upper than in the lower field in POAG. 3. Almost equal number of defects in the upper and lower halves in PG. 4. In all 3 glaucoma types the defects are more frequently found in the nasal than in the temporal half of the visual field. 5. In LTG and POAG of all stages the defects are most frequently found in the upper nasal and most seldom in the lower temporal quadrant. 6. In all glaucoma types the defects occur on average most frequently between 10 degrees and 20 degrees of eccentricity. 7. With increasing IOP in POAG the scotomas tend to be equally distributed in the upper and lower quadrants. II. Depth of scotomas: 1. Scotomas are deepest in LTG; they are less deep in POAG, and least deep in PG. 2. In PG the mean scotoma depth depends significantly on maximum IOP.(ABSTRACT TRUNCATED AT 400 WORDS)


Subject(s)
Computers , Glaucoma, Open-Angle/diagnosis , Glaucoma/diagnosis , Microcomputers , Software , Visual Field Tests/instrumentation , Visual Fields , Humans , Intraocular Pressure , Ocular Hypertension/diagnosis , Scotoma/diagnosis
16.
Int Ophthalmol ; 13(1-2): 113-7, 1989 Jan.
Article in English | MEDLINE | ID: mdl-2744939

ABSTRACT

UNLABELLED: 126 eyes with open-angle glaucoma and defects of the visual field were studied over five years (+/-1.3) by repeated perimetries with Octopus Perimeter, Program 31, after the IOP had been normalized from 26.49 mm Hg to 19 mm Hg. All patients had suffered visual field defects in the period with increased pressure preceding our study and had many perimetric examinations before. The aim of the study was to check the behaviour of the visual field defects after pressure normalization. For evaluation of the visual fields, the upper most line of the test-points and the 6 test-points surrounding the blind spot were disregarded. The evaluation of each field and the comparison with preceding fields (no change, better, or worse) were done by the symmetry test of Bowker instead of the Delta Program, taking into account each of the 61 test-points with a significance level of 5%. In each patient the eye with the heavier field loss was evaluated. RESULTS: 67% of all eyes had no change during the observation time, 12% became better, and 21% worse. Improvement of the visual fields occurred even in old age: 10% became better in the age-group over 70 years. The total loss decreased in the 15 eyes which improved by 269 dB. In eyes which deteriorated, the total loss increased by 282 dB. The good news of this study is that normalization of IOP can stop the further decay of the visual-field in 67% and an improvement of the field loss is possible even in old age of with heavy field loss.(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Glaucoma, Open-Angle/drug therapy , Intraocular Pressure , Visual Fields , Age Factors , Aged , Electronic Data Processing , Female , Humans , Longitudinal Studies , Male , Middle Aged , Prognosis , Visual Field Tests
17.
Int Ophthalmol ; 13(1-2): 3-13, 1989 Jan.
Article in English | MEDLINE | ID: mdl-2663747

ABSTRACT

The Optic Nerve Head Analyzer (ONHA) calculates by means of computer-assisted analysis of stereo images different parameters of the optic disc: disc diameter, disc size, cup/disc ratio (CDR), neuroretinal rim area, and excavation volume for the disc quadrants and for the total disc. To obtain first indications of the clinical value of ONHA measurements for diagnosis and follow-up in glaucoma we examined the reproducibility of measurement results for different diseases. Furthermore, we studied the mean values of the different disc parameters in healthy eyes. It was investigated: 1) whether the reproducibility is different in the different disc parameters; 2) whether the reproducibility in different eye diseases is different as compared to healthy eyes; 3) by which criteria the reproducibility is influenced; 4) whether there is a correlation between disc size and size of rim area. The reproducibility was studied in 178 eyes of 178 patients, who were all examined twice with the ONHA. The mean difference between the results of first and second measurement was calculated for the different disc parameters. Differences were found in the reproducibility of the parameters: e.g., the mean difference between first and second measurement was 1.8 percent for the disc size, and 5.6 percent for the neuroretinal rim area. For the disc quadrants, the reproducibility of values was worse than for the total disc. No marked differences of reproducibility of disc parameters were found for different diseases. Reproducibility depends, for instance, upon correct determination of the disc margin. In healthy eyes, a significant correlation was found between rim area and disc size: larger discs have a larger neuroretinal rim area than smaller discs. Thus, the rim areas of different eyes are only comparable for equally sized discs. Relative values, as for instance, cup/disc ratio, or the quotient of rim area and disc size, are therefore better suited for comparison of different eyes than absolute values. The recent developments in automatic disc analysis equipment and the clinical relevance of the results for diagnosis and follow-up in glaucoma are discussed.


Subject(s)
Diagnosis, Computer-Assisted , Glaucoma/diagnosis , Optic Disk/anatomy & histology , Eye/embryology , Humans , Pallor/complications , Vision Tests
18.
Curr Opin Ophthalmol ; 6(2): 78-88, 1995 Apr.
Article in English | MEDLINE | ID: mdl-10150862

ABSTRACT

This article briefly overviews risk factors involved in the multifactorial glaucomatous disease such as hypotension, preexisting field damage, location of scotomas, intraocular pressure, vasospasm, vasoactive agents, and less connective tissue of the optic disc. We summarize some of these risk factors in a formula allowing the calculation of a glaucoma progression risk index. This index helps to identify patients who are at risk for further visual field deterioration already at the time of the initial perimetric examination. Diagnostic and therapeutic aspects are highlighted.


Subject(s)
Glaucoma, Open-Angle/etiology , Glaucoma, Open-Angle/physiopathology , Glaucoma, Open-Angle/therapy , Humans , Hypotension/complications , Hypotension/therapy , Intraocular Pressure , Optic Disk/pathology , Risk Factors , Scotoma , Sickness Impact Profile , Visual Fields
19.
Klin Monbl Augenheilkd ; 211(4): 257-62, 1997 Oct.
Article in German | MEDLINE | ID: mdl-9445914

ABSTRACT

BACKGROUND: Poor compliance can be dangerous to successful medical treatment of glaucoma. Among other things one reason for non-compliance represents the inability of the patients to place drops in the eye appropriately. Therefore information regarding the patients ability to administer an eyedrop safely are a prerequisite to determining a therapy scheme which ensures the compliance of glaucoma patients. PATIENTS AND METHODS: 100 glaucoma patients on medical therapy who first presented in the outpatient glaucoma clinic of the University Eye Hospital of Würzburg were examined by standardised questionnaire and ability tests. We evaluated: 1. Can the eyedrop administration of glaucoma patients be improved by a standardised instruction? 2. Can the eyedrop administration be improved by the use of a drop aid (Autodrop)? 3. Can the accuracy of aiming and the manual ability be evaluated with a target-test on a sheet of paper with a series of concentric circles? 4. How do patients on combined therapy distinguish between their different bottles and where is the dosage regimen noted? 5. What kind of distinguishing marks of eyedrop-bottles do the patients prefer? RESULTS: 1. Before verbal instruction 76% of the patients applied the eyedrops appropriately, after instruction 94% (p < 0.001) were capable. Touching the eye with the tip of the dropper was reduced significantly. Touching the eye before instruction was found in 63% of the patients, after instruction it was found in 41% (p < 0.001). 2. When patients used the drop aid 81% were able to place a drop in the eye appropriately. Only by 46% of the patients the Autodrop was welcomed. 3. 16% of the patients were not able to place a drop within 1.5 cm of the center of the target (according to the size of an eye). 4. 47% of the patients who use more than one eyedrop bottle admitted problems in distinguishing the bottles, only 38% of the patients read the labels. 5. 76% of the patients would prefer markable stickers of different colours for the bottom of the bottles to improve distinguishing the bottles in combined therapy. CONCLUSION: Instruction improves eyedrop administration in 18% of our patients. In combined therapy with several drugs new distinguishing marks are requested by the patients. To improve compliance combination preparations should be administered if available.


Subject(s)
Drug Delivery Systems/instrumentation , Glaucoma/drug therapy , Miotics/administration & dosage , Parasympathomimetics/administration & dosage , Patient Compliance , Self Administration/instrumentation , Adolescent , Adult , Aged , Aged, 80 and over , Drug Therapy, Combination , Equipment Design , Female , Glaucoma/psychology , Humans , Male , Middle Aged , Ophthalmic Solutions , Patient Acceptance of Health Care , Self Administration/psychology
20.
Curr Opin Ophthalmol ; 9(2): 77-87, 1998 Apr.
Article in English | MEDLINE | ID: mdl-10180519

ABSTRACT

The possibilities and limitations of methods for indirect and direct nerve fiber layer thickness (NFLT) measurements, and a summary of our own clinical studies using Optic Nerve Head Analyzer (Rodenstock, Munich, Germany) (ONHA), Laser Tomographic Scanner (Heidelberg Engineering, Heidelberg, Germany) (LTS), Heidelberg Retinal Tomograph (Heidelberg Engineering, Heidelberg, Germany) (HRT), Nerve Fiber Analyzer (Laser Diagnostic Technologies, San Diego, CA) (NFA) and Optical Coherence Tomography (OCT), together with the literature of the review period, are highlighted. A retinal hemifield test with the new parameters, retinal asymmetry difference and retinal asymmetry index, is introduced. These relative nerve fiber layer thickness (NFLT) measurement values are found to be independent of age, reference plane, and disc size. This reduces the interindividual variation of the measurements and may improve screening possibilities for glaucoma. The detection probability for nerve fiber bundle defects was found to be stage-dependent using HRT and NFA. For laser polarimetry, a decreasing correlation between NFLT and visual field loss with advanced glaucomatous disease was found. In the lower retina, a 6.9% higher NFLT was evident compared with the upper peripapillary NFLT in healthy eyes using NFA and OCT. This is not in correlation with perimetric retinal sensitivity, which was found to be 3.9% higher in the lower hemifield compared with the upper hemifield. In general, quantitative NFLT measures (HRT, NFA, OCT) were correlated with visual field loss in glaucoma. The interindividual variability of NFLT measurements, among other things, induces limitations on distinguishing beginning glaucoma from normals. Clinical and scientific significance and future directions of NFLT measurements are discussed.


Subject(s)
Nerve Fibers/pathology , Optic Disk/pathology , Retina/pathology , Diagnostic Imaging , Glaucoma/pathology , Humans , Image Processing, Computer-Assisted , Lasers , Tomography/methods
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