A Case of Lamina Lucida-Type Linear IgA Disease Complicated by Colon Polyposis and Rectal Adenocarcinoma.

Linear IgA disease (LAD) is a rare autoimmune bullous disease characterized by IgA deposition in the basement membrane zone (BMZ). A 66-year-old male was treated for myelodysplastic syndrome at our hospital for 5 years, during which his condition remained stable. He visited our department because of erythema with itching, which appeared 1 year ago and gradually exacerbated with the development of blisters and erosions. During the first visit, multiple erythemas with erosions and crusts on their periphery were observed on the trunk and lower limbs. Histopathological examination revealed subepidermal blisters with inflammatory cell infiltration, mainly constituting of neutrophils, eosinophils, and lymphocytes. Direct and indirect immunofluorescence showed linear IgA deposits in the BMZ and IgA anti-BMZ antibodies, respectively, while immunoblotting using a concentrated culture supernatant of HaCaT cells detected IgA antibodies reactive to 120-kDa LAD-1. Accordingly, the patient was diagnosed with lamina lucida-type LAD. Subsequent colonoscopy revealed multiple colorectal polyps and rectal adenocarcinoma (Tis, N0, and M0). Multigene panel test showed an ATM variant of unknown significance but did not detect any pathogenic variants associated with intestinal polyposis syndrome. The skin lesions quickly resolved with oral diaphenylsulfone 50 mg/day and resection of the colorectal polyps and adenocarcinoma. To our knowledge, this is the first reported case of LAD associated with multiple colorectal polyps and rectal adenocarcinoma. Additionally, we also analyzed reported cases of LAD associated with malignancy from the literature.

Effects of menthol on the pharmacokinetics of triazolam and phenytoin.

We have previously shown that menthol attenuates the anticoagulant effect of warfarin by increasing the expression levels of CYP3A and CYP2C in the liver. This study evaluated the effects of menthol on the pharmacokinetics of the CYP3A substrate triazolam and the CYP2C substrate phenytoin. Menthol was orally administered to mice for 7 d. Twenty-four hours after the administration of menthol, triazolam was orally administered, and the plasma concentration was measured. In addition, the CYP3A metabolic activity for triazolam and the CYP3A expression level in the liver were determined. The effects of menthol on the pharmacokinetics of phenytoin were assessed in the same manner. In the menthol-treated group, the area under the blood concentration-time curve (AUC) of triazolam was lower and its clearance was higher compared with the control group. The CYP3A metabolic activity and CYP3A expression level in the liver were significantly increased in the menthol-treated group compared with the control group. Similarly, the AUC of phenytoin was lower and the hepatic CYP2C expression level was higher in the menthol-treated group. Thus, menthol lowered the plasma concentrations of triazolam and phenytoin when concurrently administered. These effects may be attributed to an increased metabolic activity for these drugs due to the increased expression of CYP3A and CYP2C in the liver.

Auditory-pupillary responses in deaf subjects.

Pupillary dilation in response to sound stimuli is well established and is generally considered to represent a startle reflex to sound. We believe that the auditory-pupillary response represents not only a simple startle reflex to sound stimuli but also represents a reaction to stimulation of other sense organs, such as otolith organs. Eight young healthy volunteers without a history of hearing and equilibrium problems and 12 subjects with bilateral deafness participated in this study. Computer pupillography was used to analyze the auditory-pupillary responses of both eyes in all subjects. We found that auditory-pupillary responses occurred even in subjects with bilateral deafness and that this response was comparable to those of normal subjects. We propose that the auditory-pupillary response also relates to vestibular function. Thus, assessing the auditory-pupillary response may be useful for evaluating the vestibulo-autonomic response in patients with peripheral disequilibrium.

Model experiments of otoconia stability after canalith repositioning procedure of BPPV.

CONCLUSION: Postural restrictions are probably not necessary after the canalith repositioning procedure (CRP). OBJECTIVES: Epley reported the effect of CRP for benign paroxysmal positional vertigo (BPPV). After CRP, patients are often requested to restrict postural change. However, some studies suggested that CRP may work without postural restrictions. The present study aimed to determine the necessity of post-maneuver postural restriction using the frog labyrinth model. METHODS: The otoconial mass from the sacculus was placed on the utricular macular otoconia, mimicking a condition after CRP. The stability of the otoconial mass was observed by tilting the preparation, immediately, 3 min, and 5 min after it was placed on the macular otoconia. The utricular macula was maintained in the vertical plane for 10 s, during which period the behavior of the otoconial mass was observed. In experiment 1 the utricular macula was intact, in experiment 2 otoconia were partially removed, and in experiment 3 they were totally removed from the macula. RESULTS: In experiments 1 and 2, in all preparations the otoconial mass became stabilized after 3 min. Even in experiment 3, in most preparations the otoconial mass became stabilized after 5 min.

Subjective visual vertical in patients with ear surgery.

CONCLUSION: Dysequilibrium is one of the most important side effects of ear surgery. The subjective visual vertical can be used as a good indicator for the evaluation of otolithic function in patients with ear surgery. OBJECTIVE: To investigate the influence of various types of ear surgery on the otolithic organs. METHODS: Seventy-one patients underwent ear surgery. Subjective visual vertical (SVV) test was performed before and after ear surgery. We investigated the directional changes of SVV before and after the ear surgery. RESULTS: The postoperative SVV of two patients who underwent translabyrinthine removal of vestibular schwannoma shifted toward the operated side, but following other surgical procedures the SVV tended to shift toward the healthy side.

Positional and positioning down-beating nystagmus without central nervous system findings.

We report the clinical features of 4 cases with positional or positioning down-beating nystagmus in a head-hanging or supine position without any obvious central nervous system disorder. The 4 cases had some findings in common. There were no abnormal findings on neurological tests or brain MRI. They did not have gaze nystagmus. Their nystagmus was observed only in a supine or head-hanging position and it was never observed upon returning to a sitting position and never reversed. The nystagmus had no or little torsional component, had latency and tended to decrease with time. The positional DBN (p-DBN) is known to be indicative of a central nervous system disorder. Recently there were some reports that canalithiasis of the anterior semicircular canal (ASC) causes p-DBN and that patients who have p-DBN without obvious CNS dysfunction are dealt with anterior semicircular canal (ASC) benign paroxysmal positional vertigo (BPPV). There are some doubts as to the validity of making a diagnosis of ASC-BPPV in a case of p-DBN without CNS findings. It is hard to determine the cause of p-DBN in these cases.