Cardiac involvements in myasthenia gravis associated with anti-Kv1.4 antibodies.

BACKGROUND AND PURPOSE: There is no general consensus as to whether autoimmune myasthenia gravis (MG) is associated with heart diseases, despite the fact that myocarditis, a serious cardiac involvement treatable by immunotherapy, is a complication of MG. It has been observed previously that MG patients with clinically suspected myocarditis had anti-Kv1.4 antibodies. The purpose of this study was to disclose the association between anti-Kv1.4 antibodies and cardiac involvements in MG patients. METHODS: Anti-Kv1.4 antibody was detected by an immunoprecipitation assay using (35) S-labeled rhabdomyosarcome cellular extract as the antigen source. Cardiac findings including electrocardiography (ECG) and clinical features of clinically suspected myocarditis in MG patients with anti-Kv1.4 antibodies were investigated. Ultrasound echocardiography (UCG) of ex vivo chick embryos was performed to determine the suppressive effects of sera with or without anti-Kv1.4 antibodies on heart muscle functions. RESULTS: Seventy (10.8%) of 650 MG patients had anti-Kv1.4 antibodies and 60% of them had abnormal ECG findings with high frequencies of T-wave abnormality and QT prolongation. Clinically suspected myocarditis was found in eight MG patients with anti-Kv1.4 antibodies but in none of the MG patients without anti-Kv1.4 antibodies. Most patients showed rapid deterioration with lethal arrhythmias such as ventricular tachycardia, sick sinus syndrome, or complete atrial ventricular block and severe heart failure. It was concluded using UCG of ex vivo chick embryos that MG serum with anti-Kv1.4 antibodies suppressed heart muscle functions. CONCLUSION: It has been demonstrated that anti-Kv1.4 antibodies are possible markers for cardiac involvements in MG patients.

GM1/GalNAc-GD1a complex: a target for pure motor Guillain-Barre syndrome.

BACKGROUND: GM1 and GalNAc-GD1a are located on the axolemma of the motor nerves and are believed to be the antigens associated with pure motor Guillain-Barré syndrome (GBS). Furthermore, GM1 and GalNAc-GD1a may exist nearby and colocalize on the axolemma. Ganglioside complex (GSC) antigens associated with GM1 or GalNAc-GD1a can be target antigens in pure motor GBS. We investigated GBS sera for antibodies to a GSC consisting of GM1 and GalNAc-GD1a (GM1/GalNAc-GD1a) and analyzed the clinical and electrophysiologic findings of patients with antibodies to GM1/GalNAc-GD1a. METHODS: Sera from 224 patients with GBS were surveyed for antibodies to GSCs consisting of two of nine gangliosides (GM1, GM2, GM3, GD1a, GD3, GT1a, GT1b, GQ1b, and GalNAc-GD1a). We analyzed the clinical and electrophysiologic features of patients with IgG antibodies to the GM1/GalNAc-GD1a complex. RESULTS: Ten patients with GBS had IgG antibodies to the GM1/GalNAc-GD1a complex. The clinical findings of the 10 patients with GBS were characterized by preserved sensory system and infrequent cranial nerve deficits. According to the criteria established by Hadden et al., electrodiagnostic studies showed a demyelinating pattern in four patients and axonal neuropathy pattern in two. Early motor conduction block at intermediate nerve segments was found in five patients. CONCLUSIONS: GM1 and GalNAc-GD1a may form a complex in the axolemma at nodes of Ranvier or paranodes of the motor nerves, and may be a target antigen in pure motor Guillain-Barré syndrome, especially in the form of acute motor conduction block neuropathy.

GD1b-specific antibody induces ataxia in Guillain-Barre syndrome.

BACKGROUND: Rabbit ataxic neuropathy and several case reports have suggested a close association of IgG anti-GD1b antibodies with ataxia in Guillain-Barré syndrome (GBS). However, about half of the patients with GBS having IgG anti-GD1b antibodies with no reactivities against other gangliosides (GD1b-mono IgG) do not exhibit ataxia. Antibodies specific to ganglioside complexes (GSCs) containing GD1b have been found in sera from some patients with GBS. OBJECTIVE: To investigate whether the reactivities of anti-GD1b IgG to such complexes are different between ataxic and nonataxic patients. METHODS: The authors examined sera from 17 patients with GBS (9 with ataxia and 8 without ataxia) who had GD1b-mono IgG, with the use of an ELISA in which wells were coated with a mixture of GD1b and each of nine gangliosides (GM1, GM2, GM3, GD1a, GD3, GT1a, GT1b, GQ1b, and GalNAc-GD1a). The binding activities of the anti-GD1b IgG antibodies against such mixture antigens were compared between ataxic and nonataxic patients. RESULTS: The reactivities to antigens, such as GD1b combined with GD1a, GT1b, GQ1b, and GalNAc-GD1a, were significantly reduced in ataxic compared with nonataxic patients. Sera from all nonataxic patients had antibody activities to GSCs not containing GD1b. CONCLUSIONS: The addition of another ganglioside may cause conformational change of GD1b. Given the inhibition of the binding ability of the anti-GD1b IgG antibodies by such a conformational change, the anti-GD1b IgG antibodies in ataxic patients may interact closely with GD1b. IgG antibodies highly specific for GD1b may induce ataxia in Guillain-Barré syndrome.

Gamma loop dysfunction in the quadriceps femoris of patients who underwent anterior cruciate ligament reconstruction remains bilaterally.

Purpose of this study was to investigate the effects of anterior cruciate ligament (ACL) repair on the gamma loop of the bilateral quadriceps femoris (QF). Maximal voluntary contraction (MVC) of knee extension and integrated electromyogram (I-EMG) of vastus medialis (VM), vastus lateralis (VL), and rectus femoris (RF) were examined in uninjured and injured limbs of 18 patients and 10 normal subjects, before and after 20-min vibration applied to the QF. Mean percentage changes were calculated as: (pre-vibration value-post-vibration value)/pre-vibration value x 100. Patients were divided into two groups: short-term-group (tested /=18 months after ACL repair, n=10). Mean percentage changes of the four groups were compared with those of controls. Results indicated that changes of MVC and I-EMG on the uninjured and injured sides in short-term-group in response to vibration were significantly different from those of controls. There were no significant differences between uninjured sides in long-term and control groups. MVC and I-EMG of VM and RF of injured side in patients in the long-term-group in response to vibration were not different from those of controls. From these results, we concluded that this abnormality of the gamma loop in both injured and uninjured sides did not recover despite ACL reconstruction. However, the abnormality in uninjured side might recover >/=18 months after repair.

Seasonal variation of multiple sclerosis exacerbations in Japan.

Several reports have described the seasonal variation of multiple sclerosis (MS) attacks in the European countries and in the US. Some have insisted that attacks occurred more frequently in winter or spring. We investigated the possibility of a seasonal variation in the frequency of MS attacks among patients in Japan. A total of 172 MS exacerbations in 34 MS patients were analyzed retrospectively. Attacks were divided into two groups: opticospinal type and brain type. The 12 months of the year were assigned to 6 groups based on average monthly temperature. Of the 172 MS exacerbations, 123 were opticospinal type and 49 were brain type of attacks. The total number of attacks was significantly more frequent in the warmest (July and August) and coldest (January and February) months. The heat of summer in warmer, low latitude areas may be a risk factor for MS attacks.

Acute unilateral corneal immunoprotein deposition in IgM monoclonal gammopathy.

A healthy 43-year-old officer of a merchant ship at sea developed pain, redness, and photophobia in his right eye. During the next 2 weeks, he noted the presence of a band of opacity spreading from his temporal limbus toward his central cornea. His episcleral vessels were engorged in a distribution contiguous with the peripheral, sectorial, fleck-like corneal opacities. The opacity had progressed during topical and systemic antibiotic therapy, but halted with use of topical corticosteroids. Systemic evaluation showed mild IgM monoclonal gammopathy. Transmission electron microscopy of a corneal biopsy specimen revealed electron-dense fibrils identified as immunoprotein. To our knowledge, this is the first report of a case of acute unilateral deposition of corneal immunoprotein in a patient with monoclonal gammopathy. Clinicians should begin with a broad differential diagnosis when evaluating patients with corneal opacity.

The iris cerclage suture for permanent mydriasis: a running suture technique.

Permanent mydriasis can be a disabling condition. The author describes an iris cerclage running suture technique for the treatment of permanent mydriasis caused by diffuse iris sphincter damage. A long, curved transchamber needle on 10-0 polypropylene is passed in and out of the anterior chamber through limbal paracentesis openings while weaving the needle through the iris near the pupillary margin to form the cerclage. The technique minimizes stretching and distortion of the iris and creates a pupil that is precisely sized and rather round.

Radial keratotomy clear zone diameter errors.

PURPOSE: To examine the variability of achieved clear zone diameters following radial keratotomy and to determine if refractive outcomes are correlated to clear zone diameter errors. METHODS: Twenty-six patients with radial keratotomy (51 eyes) were studied retrospectively. The central clear zone diameter for each radial incision meridian was measured using a slit-lamp and a photograph. The achieved clear zone diameters were statistically compared to the intended clear zone sizes and to the refractive outcomes. RESULTS: Twenty-nine of the 51 eyes met various exclusion criteria. Ninety individual slit-lamp clear zone measurements were obtained for the remaining 22 eyes. Sixty-two of 90 (69%) achieved clear zone diameters were more than 0.25 mm different than the intended clear zone size. A maximum difference of +/- 0.80 mm from the intended clear zone marker size was observed. The average clear zone diameter error did not statistically differ between the three refractive outcome groups (undercorrection, emmetropia, and overcorrection). CONCLUSIONS: In selected cases of centripetal radial keratotomy, the achieved clear zone diameter can vary widely from the intended clear zone size despite extensive surgeon experience.

Single wound, in situ tying technique for iris repair.

The authors describe a technique for placing and tying interrupted iris sutures in virtually any area of the iris using only a single limbal paracentesis wound. The tying is performed with conventional knot-forming techniques outside the eye before the throws are pulled into the anterior chamber and tightened with the desired 180 degrees tension on the suture ends. During suture tying, the iris stays in situ rather than being stretched and distorted.

Measurement of radial keratotomy clear zone diameters.

BACKGROUND: To compare the accuracy of photographic measurements to slit-lamp measurements of radial keratotomy clear zone diameters in order to develop an independent, objective, unbiased, and reproducible method of verifying clinical observations. METHODS: Twenty-five patients (48 eyes) following radial keratotomy had matched slit-lamp and photographic measurements of the diameter of the central clear cornea between the ends of opposite radial incision pairs. Matched slit-beam, photographic, and pathologic observations were compared statistically. RESULTS: Two hundred fifty-four slit-lamp clear zone diameter measurements were obtained. In twenty instances (8%), the radial incision end-point could not be identified on the photograph. For the remaining 234 measurements, the mean slit-lamp clear zone diameter was statistically smaller than the photographic measurement by 0.03 mm (t-test, p = 2.2 x 10(-5)). The slit-lamp and photographic clear zone measurements were strongly positively correlated (Pearson r = 0.99, p < 1.0 x 10(-15)). Matched slit-lamp, photographic, and pathologic clear zone measurements (N=8) in one excised corneal button were not statistically different. CONCLUSIONS: For radial keratotomy clear zone diameters, slit-beam measurements and photographic caliper measurements yield indistinguishable results that are highly concordant over a wide range of diameters. Both slit-beam and photographic measurements yield accurate and reliable results that reflect the true pathologic achieved clear zone diameter following radial keratotomy surgery.

Screening for glaucoma with stereo disc photography.

PURPOSE: To determine the value of taking simultaneous stereo photographs of the optic nerve head as a basis for identification of patients with glaucoma. METHODS: Two hundred fifty-eight patients received complete ophthalmological examinations and were ranked on a scale of 1-5 regarding the likelihood of their having glaucoma. Each eye was also photographed using the NIDEK camera, providing stereo pairs of the optic nerve head. The same patients were reclassified by two independent masked observers on the same scale of 1-5, based solely on examination of the photographs. RESULTS: Examination of stereo photographs alone provided maximum sensitivity of 75% and specificity of 95% in identification of glaucoma patients when photographic readings were compared with all available clinical information. CONCLUSIONS: Stereo photographs of the optic nerve head can be used for glaucoma detection with an accuracy that is significantly greater than simple tonometry and with a sensitivity that is equivalent to screening with computerized perimetry.

Increasing pharmacy productivity by expanding the role of pharmacy technicians.

Efforts to meet growing clinical and distributive demands without increasing pharmacy staff are described. Real and expected increases in demands for services led pharmacists at a cancer center to seek ways of accommodating those demands within budgetary limits. Growth in the distributive workload was interfering with clinical consultation work. Research studies by the medical staff were resulting in complex dosage calculations and time-consuming compounding. Increasing requests for clinical services had to be met without compromising distributive services and teaching responsibilities and without raising costs. A plan of action was approved that included the use of a written test and a training manual to allow the hiring and retaining of skilled pharmacy technicians qualified to assume greater responsibilities. Technicians were assigned to enter drug orders into the computer, check other technicians, and dispense certain drugs. Greater use was made of commercially prepared i.v. solutions, and the floor stock was expanded. A comprehensive quality control program was concurrently put in place. The larger role for technicians not only enabled the pharmacy department to increase its distributive workload dramatically but reduced pharmacy medication errors and provided more time for clinical pharmacy practice. The number of pharmacist and technician full-time equivalents increased by only 1.5 in each category between 1985 and 1990. By making more use of pharmacy technicians, a pharmacy department was able to meet escalating demands for services with only a minor increase in personnel.