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The diagnosis of myasthenia gravis (MG) can usually be made on the basis of the characteristic clinical history and signs, improvement by the use of anticholinesterase drugs, decremental responses in repetitive nerve stimulations, and assay of anti-acetylcholine receptor (AchR) antibody titers. We, however, have difficulty to make diagnosis of ocular MG patients with mild symptoms because muscular weakness is minimal and ancillary tests are negative. In the present communication, we report clinical usefulness of a hot test to provoke ptosis by warming the eyelid in ocular MG patients with minimal fatigability. Patient 1, a 27-year-old housewife, developed drooping of the right upper eyelid in May 1985. The ptosis was absent in the morning, but became apparent and worsened later. Neurological examination carried out 3 months after the onset revealed mild right ptosis, but fatigability of the levator palpebrae superioris could not be elicited by the provocative procedures such as sustained upward gaze or repeated opening and closing of eyelids. Both Tensilon and cold tests yielded negative responses. Repetitive nerve stimulations produced no decremental responses. Titers of anti-AChR antibody and antistriational antibody were within normal limits. In order to find a possible neuro-muscular blockade, we warmed the right upper eyelid by applying hot water of about 45 degrees C in a vinyl bag for 3 minutes. The hot test worsened the right ptosis and induced mild left ptosis. Tensilon administration reversed the eyelids to the previous position. Patient 2 was a 12-year-old boy with a typical history and clinical signs of ocular MG. His symptoms remitted spontaneously without any medication 3 weeks after the onset.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Diagnostic usefulness of a hot test in patients with mild ocular myasthenia gravis]. 342 58

A case of aortic dissection associated with aortic regurgitation which was induced by diastolic prolapse of an intimal flap into the left ventricular outflow tract was reported. This 57-year-old man, referred for evaluation of sudden onset of chest oppression and a heart murmur, was hypertensive for several years. His admission blood pressure was 184/44 mmHg, and a systolic ejection murmur and a diastolic decrescendo murmur were audible along the left sternal border. Two-dimensional echocardiography revealed an intimal flap in the markedly enlarged aortic root. The intimal flap moved posteriorly in systole and anteriorly in diastole, and prolapsed into the left ventricular outflow tract during diastole. Associated with the movement of the intimal flap, an aortic cusp was shifted from its original position to the left ventricular outflow tract in diastole. Aortography disclosed type I aortic dissection and severe aortic regurgitation. After medical treatment for four months, the patient underwent a Bentall surgical procedure and recovered. Impaired coaptation of the aortic valve induced by diastolic prolapse of the intimal flap into the left ventricular outflow tract is a newly encountered echocardiographic finding in proximal aortic dissection.
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PMID:[Aortic dissection presenting aortic regurgitation induced by diastolic prolapse of an intimal flap into the left ventricle: a case report]. 383 75

A 27-year-old man was bitten by a coral snake in Mexico. Within 24 hours he had ptosis, dysphonia, difficulty chewing, and limb weakness. His symptoms peaked at 72 hours with loss of ambulation. Neurologic examination was consistent with severe myasthenia. Repetitive stimulation of the median nerve showed a postsynaptic defect that was not corrected by edrophonium. He was monitored in an intensive care unit, but received no antivenom globulin or acetylcholinesterase inhibitors. The syndrome abated in 3 weeks.
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PMID:Neurologic complications of a coral snake bite. 398 52

A 21 year old patient was operated for bilateral ptosis and external ophthalmoplegia at 13 years of age. At this time there were no signs of retinitis pigmentosa or atrioventricular block, features of the Kearns and Sayre Syndrome (1958) which were detected five years later. His bundle recording showed an intrahisian block (1 degree proximal and a complete distal block) with a trifascicular block, the latter persisting alone during a brief return to sinus rhythm. This is one of the rare cases of the Kearns and Sayre Syndrome with documented His bundle recordings and the only reported case with intrahisian block. The patient also suffered from bilateral neural deafness. The patient's condition remains stable after implantation of an isotopic cardiac pacemaker and he now leads a normal life. A review of 52 previously published cases shows that this rare condition appears to be caused by a mitochondrial abnormality, which, for an unknown reason, affects only the neuromuscular and cardiac conduction systems. The prognosis is poor when swallowing and respiration are affected, but this does not occur in all cases. As cardiac conduction abnormalities are the other life-threatening complication, cardiac pacing has greatly improved the prognosis of these patients.
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PMID:[Auriculo-ventricular block in the Kearns-Sayre syndrome. Apropos of a case]. 640 30

A 2-year-old boy with psychomotor retardation, congenital unilateral ptosis, bilateral adducted thumbs, weakness of upper limbs, and Hirschsprung's disease (aganglionosis), with complete agenesis of the corpus callosum and hypoplasia of the inferior vermis and cerebellum is reported. His 24-year-old maternal uncle, with severe psychomotor retardation but none of the other physical problems, also has agenesis of the corpus callosum demonstrated by CT scan. The implications for antenatal diagnosis are discussed.
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PMID:X linked recessive inheritance of agenesis of the corpus callosum. 668 47

Diagnosis of ventricular arrhythmias is generally easy. The differentiation between ventricular tachycardia (VT) and supraventricular tachycardia (ST) with aberrant intraventricular conduction deserves some comments. Frequently the distinction can be made on the electrocardiogram alone. VT is characterized by: a) QRS wider than 0.14 sec., b) left axis deviation (a and b criteria are no longer reliable when a preexisting bundle branch block is present), c) mono or biphasic V1 configuration (biphasic rSR' in ST), d) multiformity, e) A-V ratio different from 1:1 in spontaneous conditions or during vagal or pharmacological (ATP, verapamil) stimulation, f) presence of fusion or capture beats. None of these criteria has an absolute diagnostic value. His bundle potential (H) recording confirms the diagnosis when bundle of His is activated during or after QRS complex or when H-V interval is shorter than during sinus rhythm. Special cases are discussed, particularly the diagnosis of rapid supraventricular arrhythmias with anterograde conduction through an anomalous pathway. Characteristics of "slow", "bidirectional" and "iterative" VT are reported. "iterative" VT are reported. Electrocardiographic aspects of "torsades de pointe" are obtained in relation to the presence or the absence of a QT interval prolongation (multiform VT). The torsade is the result of rapid variations in depolarizing wavefront direction due to a high degree of electrical heterogeneity of ventricular myocardium; it has a severe prognostic significance regardless of the QT interval duration. However a long QT interval, when present, is the mark of the pathophysiological mechanism of the arrhythmia and a major indicator for therapeutical decisions. Finally the malignant aspects of unsustained ventricular arrhythmias in different cardiac diseases (mitral prolapse, obstructive cardiomyopathy, ischemic cardiac disease) and in apparently normal subjects are taken into consideration. The concept that the prognostic significance of the arrhythmias is in a large part dependent upon the type and severity of the underlying cardiac disease is stressed. The risk related to R/T premature ventricular beats is confirmed.
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PMID:[Malignant ventricular hyperkinetic arrhythmias: diagnostic aspects]. 688 68

Myotonia atrophica, a neuromuscular disease marked by autosomal dominant transmission and delayed relaxation of skeletal muscle, has been associated with cardiac failure, conduction abnormality and mitral prolapse (MVP). In order to determine the relaxation rate of cardiac muscle, left ventricular (LV) size and function, and the presence of MVP, 30 patients with myotonia atrophica were studied using digitized M-mode echocardiography (MME). Intracardiac conduction intervals were determined by noninvasive His bundle recording (HBR) from surface electrodes using a high-resolution, R-wave triggered, signal averaging computer. Neurologically unaffected first-degree relatives of the patients with myotonia atrophica were also studied to determine if cardiac abnormalities may be present in the absence of neurologic manifestations of the disease. Peak normalized diastolic endocardial velocity in patients with myotonia atrophica (3.7 +/- 0.8 sec-1) did not differ from unaffected first-degree relatives (3.8 +/- 0.8 sec-1) or normal subjects (3.6 +/- 0.8 sec-1). Systolic LV function and LV dimensions on MME were normal in both groups. However, MVP was present in 7 of 24 (29%) of patients who could be evaluated, but not in unaffected first-degree relatives. Despite normal LV systolic and diastolic function, infranodal intracardiac conduction was prolonged in patients with myotonia atrophica (average HV interval 50 +/- 5 SD msec) but not in neurologically unaffected relatives (average HV interval 40 +/- 5 msec). Delay in proximal intracardiac conduction was also found in patients with myotonia atrophica (average PH interval 140 +/- 20 msec) but not in neurologically unaffected relatives (average PH interval 115 +/- 6 msec). Hence cardiac findings in myotonia atrophica include proximal and distal conduction delay by external HBR even in the absence of abnormality of the standard 12-lead ECG. There may also be an increased frequency of MVP; however, early diastolic relaxation of the LV is unimpaired, and cardiac manifestations of myotonia are not transmitted independently of neurologic abnormality.
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PMID:Left ventricular relaxation, mitral valve prolapse, and intracardiac conduction in myotonia atrophica: assessment by digitized echocardiography and noninvasive His bundle recording. 709 Sep 87

The Marcus Gunn Syndrome is discussed. It is postulated that an atypical oculocardiac reflex is easily initiated in these patients so that precautions must be taken for its prevention and early detection. Marcus Gunn first described the syndrome in 1883. His patient had a congenital ptosis of the left upper eyelid. Paradoxical lid retraction was evoked by movement of the jaw. Consequently the syndrome also became known as the 'jaw-winking' syndrome. Other varieties of the Marcus Gunn syndrome have also been described. The pathogenesis of this syndrome seems to be the presence of abnormal central connexions between the trigeminal and oculomotor cranial nerves which supply the external pterygoid and the levator palpebrae superioris muscles. The importance of recognition of the oculocardiac reflex in patients undergoing eye surgery under anesthesia is well known to anaesthetists and to eye surgeons. The occurrence of this reflex in patients undergoing surgery on the eyelids has been reported previously. This paper describes a patient with the Marcus Gunn Syndrome who underwent three operations on the left upper eyelid and who exhibited unusual manifestations of the oculocardiac reflex.
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PMID:Marcus Gunn Syndrome associated with an unusual oculo-cardiac reflex. 739 12

We present a rare case of thalamic germinoma with crossed aphasia in a dextral. A patient, 17-year-old righat-handed male, was admitted to Nippon Medical School Hospital with chief complaints of headache, abnormality of visual field and speech disturbance. There were pigmentations on the back of hand, foot and the perineum. Neurological examination revealed left homonymous hemianopsia, right slight degree of ptosis, left facial palsy, a mild paresis of the left upper extremity and motor aphasia. Right carotid angiography showed marked unrolling and midline shift of right anterior cerebral artery. CT scan revealed ring-like high density area in the right thalamic region, which was enhanced after constant infusion. Brain scintigraphy also showed an abnormal accumulation at the same site. The hen-egg sized tumor of 40 g. weight was almost totally removed by the right fronto-parietal craniotomy. The tumor was characterized histologically by the so-called two cell pattern with teratomatous components. As postoperative treatment local injection of adriamycine, irradiation and immunotherapy with picibanil were performed, and then left hemiparesis was markedly improved without sign of recurrence. Language evaluation was performed after operation. There were dysarthria, remarkable word amnesia, paraphasia and perseveration. Repetition was also impaired. His speech function was concluded to be a mixed type aphasia mainly composed of Broca's aphasia. The speech function of thalamus and crossed aphasia with dextrales were discussed.
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PMID:[A case of thalamic germinoma with crossed aphasia in a dextral (author's transl)]. 743 99

A 71-year-old man suffered from left-side throbbing headache over the region of the fifth cranial nerve first division, followed by left ptosis and ophthalmoplegia. Under tentative diagnosis of Tolosa-Hunt syndrome (THS), oral prednisolone 60 mg was given daily. His headache and ophthalmoplegia dramatically improved within 24 hours. Magnetic resonance (MR) imaging and computed tomography (CT) of the head were negative. However, irregularity in the siphon of the left internal carotid artery was disclosed by angiography. This case illustrates that angiography may be useful in diagnosing THS, when CT and MR imaging are negative.
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PMID:Angiographic findings in Tolosa-Hunt syndrome: a case report. 764 Nov 28


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