Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis that usually starts in early infancy. The disease may be associated with pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, trachea, and female genital tract. We examined two unrelated Turkish girls both suffering from ligneous conjunctivitis and occlusive hydrocephalus. Both children exhibited a severe plasminogen deficiency. Genomic DNA from both patients as well as from clinically healthy family members were screened for mutations in the plasminogen gene by polymerase chain reaction, single-strand conformation polymorphism (SSCP) analysis, and DNA sequencing. In the first girl with ligneous conjunctivitis a homozygous G → A point mutation was identified in plasminogen exon 7 at position 780 leading to an amino acid exchange (Arg216 → His). Her healthy sister and her healthy parents were heterozygous for this mutation. The second patient revealed a homozygous G → A point mutation in plasminogen exon 15 at position 1924 which leads to a stopcodon (Trp597 → Stop). The healthy parents were shown to be heterozygous for this mutation. In addition, the father's second allele revealed another mutation in the same codon (Trp597 → Cys) (compound heterozygosity). In conclusion, certain homozygous mutations in the plasminogen gene may cause ligneous conjunctivitis.
A double-blind study was performed on 212 consecutive patients (58 men, 154 women) with essential blepharospasm, who received one injection of Botox and one injection of Dysport in two separate treatment sessions (at the first session the patients randomly received one of the drugs, at the second the other drug was given. The patients' mean age was 66.4 years +/- 8.14 (range 39-86 years). The average dose of Botox per treatment was 45.4 IU +/- 13.3 (range 25-85 IU) and of Dysport 182.1 IU +/- 55.1 (range 100-340 IU). We used an empirical ratio Botox:Dysport of 1:4 (IU) in order to ensure equal doses. All patients had received botulinum toxin injections prior to the present study (mean 15.3 injections +/- 9.4; range 1-43 injections). The effect of Botox lasted 7.98 weeks +/- 3.8 (range 0-16 weeks), while the effect of Dysport lasted 8.03 weeks +/- 4.6 (range 0-22 weeks). Side effects (ptosis, tearing, blurred vision, double vision, hematoma, foreign body sensation) were observed with Botox in 36 of 212 (17.0%) of the treatment sessions and with Dysport in 51 of 212 sessions (24.1%). Ptosis was observed with Botox in 3 cases (1.4%) and with Dysport in 14 cases (6.6%). There was no statistically significant difference in the duration of the treatment effect between the two preparations (P = 0.42). The total number of side effects was lower with Botox than with Dysport; the significance of the difference was moderate (P < 0.05). However, the rate of occurrence of ptosis was significantly lower with Botox (P < 0.01). The bioequivalence, which varies between 1:3 and 1:6 (Botox:Dysport) in the literature, was found to be 1:4 in this study.
Thirty-one patients with essential blepharospasm or lid opening disorder of the levator-inhibiting type, unresponsive to treatment with botulinum toxin, underwent frontalis suspension. Twenty-eight patients received bilateral surgery (three patients with bilateral complaints of different severity were operated on the more affected side; these patients were not included in the statistical analysis). The mean age was 62.4 years +/- 8.52 (range 42-80 years). The individual improvement of complaints was assessed by the patients using a percentage scale (0% = no improvement; 100% = no complaints). Objective and subjective improvement was achieved in 26 of 28 patients. The mean subjective improvement was 57.7% +/- 31.4. In 23 cases an additional treatment with botulinum toxin was administered. During follow-up period (mean 22.1 months +/- 11.6; range 5-40 months) the effect of surgery remained stable. There were no serious complications, in a 5 of 56 operated eyes suture granuloma had developed. Unlike other surgeries for treatment of blepharospasm (excision of the orbicularis muscle, resection of facial nerve branches) frontalis suspension can be considered as a minimally invasive, but very effective and (if desired) reversible procedure. Moreover, additional treatment with botulinum toxin can bring about further improvement.
Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis that usually starts in early infancy. The disease may be associated with pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, trachea, and female genital tract. We examined two unrelated Turkish girls both suffering from ligneous conjunctivitis and occlusive hydrocephalus. Both children exhibited a severe plasminogen deficiency. Genomic DNA from both patients as well as from clinically healthy family members were screened for mutations in the plasminogen gene by polymerase chain reaction, single-strand conformation polymorphism (SSCP) analysis, and DNA sequencing. In the first girl with ligneous conjunctivitis a homozygous G → A point mutation was identified in plasminogen exon 7 at position 780 leading to an amino acid exchange (Arg216 → His). Her healthy sister and her healthy parents were heterozygous for this mutation. The second patient revealed a homozygous G → A point mutation in plasminogen exon 15 at position 1924 which leads to a stopcodon (Trp597 → Stop). The healthy parents were shown to be heterozygous for this mutation. In addition, the father's second allele revealed another mutation in the same codon (Trp597 → Cys) (compound heterozygosity). In conclusion, certain homozygous mutations in the plasminogen gene may cause ligneous conjunctivitis.
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