Sustained ventricular tachycardia responsive to verapamil in patients with hypertrophic cardiomyopathy. Clinical and electrophysiological assessment of drug efficacy

Recently, we examined 2 cases of hypertrophic cardiomyopathy (HCM) presenting with sustained ventricular tachycardia (VT). One case was a 62 year old male with midventricular hypertrophy and monomorphic sustained VT. After admission, the efficacies of procainamide, disopyramide, aprindin, flecainide, mexiletine and verapamil were evaluated by means of continuous electrocardiographic monitoring. Verapamil prevented the recurrence of sustained VT and markedly reduced the frequency and number of runs of nonsustained VT. In the electrophysiologic study, rapid VT was induced by double extrastimuli at the right ventricular apex. Intravenous verapamil at a dose of 10 mg prevented the induction of VT. The patient was discharged on verapamil and remains asymptomatic after 3 months of follow up. The other case was a 34 year old female who was a survivor of cardiac arrest. Monomorphic VT was observed on emergency admission and was converted to sinus rhythm by direct current cardioversion after resuscitation. In the electrophysiologic study, rapid VT was induced by double extrastimuli at the right ventricular outflow tract. Verapamil at a dose of 10 mg prevented the induction of VT. These 2 cases of HCM are rare in that they presented with sustained VT. It is also of interest that verapamil, which has been used conventionally in HCM, prevented VT.     

Prototype system for an MR simulation of intracavitary brachytherapy for cervical cancer: Preliminary results in six cases

We report a newly developed MR simulation system for intracavitary brachytherapy for cervical cancer and subsequent treatment results. MR simulation was performed on six patients. The spatial relationship of the tumor to the bladder, rectum, bowel, applicators, etc. was depicted well. Doses to the tumor and surrounding normal tissues were read from isodose curves superimposed on the T2-weighted sagittal image. This system promises to be useful in customizing the dose distribution.     

Evaluation of therapeutic interventions of sustained monomorphic ventricular tachycardia guided by an electrophysiologic study: a case report

Therapeutic evaluation of sustained monomorphic ventricular tachycardia (VT) using electrophysiologic study (EPS) is presented in a case of refractory VT. A 54-year-old man with a history of recurring syncope underwent coronary angiography which revealed total occlusion of the posterior descending branch of the right coronary artery. Left ventriculography showed a left ventricular aneurysm at the cardiac apex. Ejection fraction of the left ventricle was 36%. He had four VTs of different QRS morphologies in 12 lead electrocardiograms. According to our programmed ventricular stimulations, single or double, and rarely triple, extra stimuli were administered after eight basic stimuli at two basic cycle lengths. Rapid ventricular pacing, up to 210 bpm, was then added. The stimuli were delivered to two different sites in the right ventricle and to at least one site in the left ventricle. When the entire protocol could not induce VT, isoproterenol was given intravenously, and the same protocol was repeated. No drug could prevent VT attacks, even after the surgical resection of two VT foci, VT was still inducible. Postoperative drug therapy could not prevent VT induction in EPS. However, changes in the mode required for VT induction were observed. Among 47 patients with sustained monomorphic VT treated in our hospital, 24 had EPS to evaluate the efficacies of therapeutic interventions, such as drugs and surgery. In 14 patients, no VT was induced by the entire VT induction protocol. Among the remaining 10 patients, four showed changes in the VT induction mode, but VT recurred in their clinical courses even after their treatments.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of chronic necrotizing pulmonary aspergillosis]

A 70-year-old woman with diabetes mellitus who was following a therapeutic diet showed an infiltrative shadow in the right upper lung field on chest roentgenogram in April, 1986. She was diagnosed as having pneumonia and was treated for five months with several antibiotics, but the abnormal shadow on chest roentgenograms increased in size. Therefore, she was admitted to our hospital in October 1986. Although tubercle bacilli were not isolated from her sputum or from materials obtained by bronchoscopic examination, we made an initial diagnosis of pulmonary tuberculosis based on the findings of chest roentgenograms, tomographs and CT scanning. In spite of treatment with antituberculous drugs, the infiltrative shadow with cavity on chest roentgenograms continued to increase in size, and the patient developed occasional hemoptysis. Percutaneous needle biopsy was performed in February 1987 to establish a definite diagnosis, and the presence of Aspergillus fumigatus was confirmed by microscopic examination and culture. After treatment with miconazole and 5-FC for 3 to 4 months, the abnormal shadow on the chest roentgenogram gradually disappeared and was almost undetectable one year later. The clinical course of this patient was considered to be strongly indicative of chronic necrotizing pulmonary aspergillosis, which was described by Binder et al. in 1982.     

Peripheral nerves are involved in hypomyelinating leukodystrophy-3 caused by a homozygous AIMP1 variant

IntroductionAminoacyl-tRNA synthetase-interacting multifunctional protein 1 (AIMP1) is a non-catalytic component of the multi-tRNA synthetase complex that catalyzes the ligation of amino acids to their correct tRNAs. Bi-allelic truncating variants in the AIMP1 gene have been associated with hypomyelinating leukodystrophy-3 (HLD3; MIM 260600), which is characterized by hypomyelination, microcephaly, seizures and decreased life expectancy. Although peripheral nerve involvement has been assumed for HLD3, no compelling evidence is available to date.Case reportThe case was a first-born Filipino male. He showed profound developmental delay, failure to thrive, and spasticity in his limbs. At three months of age he developed refractory epilepsy. Serial magnetic resonance imaging (MRIs) showed profound myelination delay and progressive cerebral atrophy. He showed abnormal nerve conduction studies. Genetic testing revealed a homozygous pathogenic variant in the AIMP1 gene (NM_004757.3: c.115C > T: p.Gln39*). The parents were heterozygous for the same variant.ConclusionHere, we report a patient with a homozygous nonsense AIMP1 variant showing peripheral neuropathy as well as HLD3. Our case suggests that AIMP1 plays a pivotal role in the peripheral nerve as well as the central nervous system.     

Emphysematous cystitis developed in a patient with an eating disorder and schizophrenia

A 27-year-old female patient with an emphysematous cystitis associated with an eating disorder and schizophrenia is described. To our knowledge, this is the first case report of an emphysematous cystitis developed in those kinds of psychiatric disorders.     

Ontogeny, distribution and amine/peptide content of chromogranin A- and B-immunoreactive endocrine cells in the small and large intestine of the chicken

Chromogranin A-(CgA-) and chromogranin B-(CgB-)-immunoreactive endocrine cells were investigated in the chicken intestine during embryonic and post-hatching life. CgA- and CgB-immunoreactive cells first appeared in the intestinal tract at various embryonic ages from day 10 in the cloaca to day 16 in the distal ileum and colon. To identify the CgA- and CgB-immunoreactive cells, each tissue section was double-immunostained using a panel of polyclonal antibodies raised against gut amine/peptides. Almost all the serotonin-immunoreactive cells co-localised CgA and CgB along the entire intestinal mucosa and at all ages examined. In contrast, substance P-, peptide tyrosine tyrosine-, neurotensin- and secretin-immunoreactive cells displayed heterogeneous co-localisation patterns. For example, either all or only some cells of a given endocrine type co-stored Cg; they did so variously-only in the embryo, only after hatching, or at both stages, and co-localizing cells were sometimes located within the mucosa only in the villi and not in the glands, and sometimes vice versa. All the CgA/CgB-immunoreactive cells also displayed argyrophilia.     

Case of immunoblastic sarcoma (IBS)

A patient with immunoblastic lymphadenopaty which evolved into immunoblastic sarcoma is reported. A 48-year-old female was admitted to our department because of cough and fever. A diagnosis of immunoblastic lymphadenopathy had been made two years before the present admission. Physical examination revealed generalized lymphadenopathy. Chest radiograms showed a left hilar mass. The pulmonary tumor and enlarged lymphnodes were treated by irradiation. Although there was marked improvement at first, she experienced several relapses. One year after the admission, chest radiograms showed multiple pulmonary lesions. She developed pancytopenia and bone metastases. At autopsy, the lymphnodes showed histological evidence of immunoblastic sarcoma; a pulmonary lesion showed immunoblastic lymphadenopathy.     

Elimination of EKG artifacts from EEGs recorded with balanced non-cephalic reference electrode method

The EKG artifacts contaminating EEGs recorded by the balanced non-cephalic reference electrode system were removed from EEGs by using a device composed of a one channel averager and simple hardware. This device has the following advantages to the others which have been previously proposed: (1) it is possible by averaging of a few (i.e., 5) EKG artifacts in EEG records successfully with only one channel averaged EEG derived from an earlobe; (2) the averaged EKG pattern is able to follow rapid EKG pattern change due to altered body position; (3) the possibility of being affected by another kind of artifact was decreased, because the time of averaging process to obtain averaged EKG became shorter. A remaining problem in achieving a complete removal of EKG artifacts (maximally of 100 micro V) was beat-by-beat small changes in EKG wave forms due to the respiratory cycle. Therefore, it was felt that our method was sufficiently accurate for clinical examination of EEG.