Cases reported "Neuromuscular Diseases"

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1/16. The 'MW' sacropelvic construct: an enhanced fixation of the lumbosacral junction in neuromuscular pelvic obliquity.

    Fixation to the lumbosacral spine to correct pelvic obliquity in neuromuscular scoliosis has always remained a surgical challenge. The strongest fixation of the lumbosacral junction has been achieved with either a Galveston technique with rods or screws or with iliosacral screws. We have devised a new fixation system, in which iliosacral screws are combined with iliac screws. This is made possible by using the AO Universal spine System with side opening hooks above and below the iliosacral screws and iliac screws below it. The whole sacropelvis is thus encompassed by a maximum width (MW) fixation, which gives an 'M' appearance on the pelvic radiographs and a 'W' appearance in the axial plane. We report on our surgical technique and the early results where such a technique was used. We feel that this new means of fixation (by combining the strongest fixation systems) is extremely solid and should be included in the wide armamentarium of sacropelvic fixation.
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2/16. Atrial standstill in a case of Kugelberg-Welander syndrome with cardiac involvement: an electrophysiologic study.

    A patient with Kugelberg-Welander syndrome associated with junctional rhythm and restrictive cardiomyopathy is presented. An electrophysiologic study was performed. Persistent atrial standstill was demonstrated by detailed right atrium and coronary sinus mapping and failure of capture of both right atrium and coronary sinus pacing. A prolonged junctional recovery time, normal HV interval and normal pacing threshold of the right ventricle were also noted. The patient was successfully treated with cardiac pacing and diuretics.
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3/16. Progressive myelopathy caused by dural arteriovenous fistula at the craniocervical junction--case report.

    A 68-year-old male presented an unusual dural arteriovenous fistula (AVF) located at the craniocervical junction. magnetic resonance imaging revealed dilated perimedullary veins around the spinal cord at C-1 and C-2 levels, as well as high intensity signals in the spinal cord on T2-weighted images. Vertebral angiography identified an AVF at the point where the right vertebral artery penetrates the dura. The fistula was a single and direct communication between the vertebral artery and the spinal vein. Surgical interruption of the fistula at its venous side resulted in prompt improvement of both motor and sensory signs and symptoms.
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4/16. Cardiac involvement in the Kugelbert-Welander syndrome.

    Two cases of the Kugelberg-Welander syndrome (juvenile form of progressive spinal muscular atrophy) associated with cardiomyopathy and cardiomegaly are presented. The first patient, a 24 year old man, had atrial flutter with complete atrioventricular (A-V) block due to A-H block. echocardiography revealed an increase in the left atrial and right ventricular dimensions. The second patient was a 26 year old man whose electrocardiogram revealed an A-V junctional rhythm, deep Q wave in leads I, aVL and V5 to V6 and an RS pattern in lead V1. Histologic examination of the myocardium in Case 2 showed slight interstitial fibrosis. review of previously reported cases shows that (1) the atrium, the ventricular myocardium and A-V conducting tissue may be involved, and (2) atrial arrhythmias, A-V conduction disturbances and congestive heart failure may occur in the Kugelberg-Welander syndrome.
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5/16. Prolonged neurogenic weakness in patients requiring mechanical ventilation for acute airflow limitation.

    We describe three patients who required mechanical ventilation for severe acute exacerbations of obstructive airways disease. When treatment with sedatives and muscle relaxants was withdrawn, they exhibited profound generalized weakness and consequently required prolonged ventilation despite resolution of the airway obstruction. Clinical features were variable, but none of the patients developed failure of other organs and infection was confined to the lungs. All had electrophysiologic evidence of a predominantly motor axonal syndrome. One patient in whom sensory action potentials were abnormal may represent an unusually severe case of critical illness neuropathy occurring in the absence of systemic sepsis and multiple organ failure. In the other two cases, this diagnosis is made less likely by the complete absence of sensory involvement and in these patients the lesion appeared to be either in the most distal portion of the motor neuron or at the neuromuscular junction. In all three patients, resolution was slow but eventually complete. The etiology of the condition is not clear, but it seems to be distinct from the acute myopathy previously described in asthmatics who had received mechanical ventilation. It is important to recognize this phenomenon to avoid erroneous conclusions about the likelihood of the patient recovering from ventilator dependence. A prolonged weaning period is to be expected in such cases.
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ranking = 1560.5313712274
keywords = neuromuscular junction, junction
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6/16. Monoclonal IgM with unique specificity to gangliosides GM1 and GD1b and to lacto-N-tetraose associated with human motor neuron disease.

    IgM lambda monoclonal antibodies in two patients with motor neuron disease showed the same unique antigenic specificity. They bound to gangliosides GM1 and GD1b and to lacto-N-tetraose-BSA. By immunofluorescence microscopy they bound to central and peripheral nerve tissue and to motor end-plates at the neuromuscular junction. Sera from control subjects did not contain antibodies of similar specificity. Monoclonal IgMs with the same unique specificity could be responsible for motor neuron disease in some patients with monoclonal gammopathies.
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7/16. Neuromuscular, autonomic and central cholinergic hyperactivity associated with thymoma and acetylcholine receptor-binding antibody.

    Two cases of a neuromuscular hyperactivity syndrome associated with a proliferative thymoma and high serum titres of acetylcholine receptor (AChR) antibody with no signs of myasthenia are reported. The clinical and electrodiagnostic findings indicated generalized cholinergic hyperactivity at the neuromuscular junction and in the autonomic and central nervous system, resulting in generalized myokymia, excessive sweating and intermittent psychotic behaviour. The association with thymoma and raised AChR antibody suggests that this syndrome represents a unique type of autoimmune disease, in which antibodies against the AChR facilitate rather than inhibit cholinergic action. This conclusion is supported by the remission of symptoms after thymectomy and with immunosuppressive therapy in one case.
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ranking = 1560.5313712274
keywords = neuromuscular junction, junction
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8/16. Malignant thymoma with dysautonomia and disordered neuromuscular transmission.

    A 42-year-old man had prominent dysautonomia accompanied by clinical and electrophysiological features of both myasthenia gravis and the lambert-eaton myasthenic syndrome. Antiacetylcholine receptor antibodies were present in high titer. Invasive thymoma was found at thymectomy; later, a solitary metastasis to the spleen required a splenectomy. Complete remission followed surgery. There was evidence of antibody activity directed against postsynaptic acetylcholine receptors, presynaptic somatic motor terminals, and autonomic effector junctions. To our knowledge, the association of thymoma with a myasthenia gravis-Lambert-Eaton overlap syndrome has not been reported previously.
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ranking = 0.2
keywords = junction
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9/16. Muscle fiber hypotrophy with intact neuromuscular junctions. A study of a patient with congenital neuromuscular disease and ophthalmoplegia.

    An infant born with severe but nonprogressive somatic and cranial muscle weakness including bilateral external ophthalmoplegia was studied with a motor-point muscle biopsy. There was a strinking generalized decrease in the size of muscle fibers (hypotrophy), most marked in the type I fibers. Many of the small fibers were immature, resembling myotubes. Neuromuscular junctions on severely hypotrophic fibers were normal with esterase staining and by ultrastructural criteria. Although these are unusual clinical and biopsy characteristics, this infant's condition bears a resemblance to two other congenital nonprogressive neuromuscular diseases:myotubular myopathy and congenital fiber type disproportion. In these conditions and in our patient, there is no primary degenerative process affecting nerve or muscle but, rather, an apparent lack of maturation of fetal muscle fibers, indicating a defective normal trophic interaction between nerve and muscle.
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ranking = 6242.3254849096
keywords = neuromuscular junction, junction
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10/16. A new congenital neuromuscular disease with trilaminar muscle fibers.

    An infant was born with marked rigidity, a paucity of spontaneous movements, and increased serum creatine phosphokinase activity. Electromyogram was normal. A muscle biopsy, taken at the age of 7 weeks, contained numerous fibers composed of three concentric zones, warranting the designation "trilaminar fibers." Electronmicroscopy showed the innermost zone contained a densely packed collection of mitochondria, glycogen, electron opaque material, and single filaments. The middle zone consisted of myofibrils with Z-band smearing. The outer zone resembled a sarcoplasmic mass. Extrajunctional acetylcholine receptor (AChR) was present in the trilaminar fibers between the middle and outer zones. The increased muscle tone and extrajunctional AChR suggest and altered neural influence, but the markedly increased creatine phosphokinase activity is more characteristic of muscle damage. Precise definition of the nature of the defect awaits further study.
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ranking = 0.4
keywords = junction
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