Myasthenia gravis in childhood

Anlar, Banu; Ozdirim, E; Renda, Yavuz; Yalaz, Kalbiye; Aysun, Sabiha; Topçu, Meral; Topaloğlu, Haluk

doi:10.1111/j.1651-2227.1996.tb14162.x

Cited by 38 publications

(14 citation statements)

References 16 publications

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“…Neben den angeborenen, autosomal rezessiv oder dominant vererbten, Formen der MG der Kindheit, wie der kongenitalen und der juvenilen Form, die durch präsynaptische, synapytische oder postsynaptische Defekte der motorischen Endplatte verursacht werden, ist für die transiente neonatale MG die plazentare Übertragung von AChR-AK auf den Fetus verantwortlich [2,15].…”

Section: Diskussionunclassified

“…Blutaustauschtransfusion und die Gabe von Immunglobulinen (IgG i.v.) eingesetzt [2,3,6,9,11,13,14,18,27,32]. Während Plasmapherese bzw.…”

Section: Therapieunclassified

“…Die Wirksamkeit beider Therapieformen wird in der Literatur kontrovers diskutiert: Eindeutigen Therapieerfolgen stehen eine ausbleibende oder verzögerte Symptomrückbildung gegenüber, die sich nicht vom spontanen Krankheitsverlauf unterscheiden lässt. Indiziert ist ein Therapieversuch in schweren Fällen mit respiratorischer Insuffizienz und verzögerter oder ausbleibender spontaner Symptomrückbildung [2,3,11,13,27,32] Sowohl AChR-AK als auch Cholinesterasehemmer können durch die Muttermilch auf das Kind übertragen werden und dadurch eine Verstärkung der myasthenen Symptomatik bzw. z.…”

Section: Therapieunclassified

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Transiente neonatale Myasthenia gravis

et al. 2002

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Ten to twenty percent of the offspring of mothers suffering from myasthenia gravis (MG) also develop transient neonatal MG, since maternal antibodies are able to cross the placenta. We report the course of two newborns of a mother with MG and a healthy father. The first pregnancy was complicated during the 3rd trimester by a hydramnion. The newborn presented with generalized muscle weakness, respiratory distress, weak sounding, anaemia, and poor sucking. Mechanical ventilation was necessary. Confirmation of the diagnosis was achieved by the result of repetitive muscle stimulation, showing a typical decrement in the EMG, and measurement of serum antiacetylcholin receptor antibodies. For 3 months, the infant was treated with neostigmin (cholinesterase inhibitor). After 26 days of hospitalization, the patient was released and followed up regularly. Myasthenic symptoms completely resolved. Side effects of the treatment were not observed. The course of the second pregnancy was normal. This second newborn was healthy. Our case report is remarkable for the very different presentation of two children of the same mother with MG during pregnancy and after delivery, with one child developing severe transient neonatal MG, initially requiring intensive care unit (ICU) treatment followed by quick recovery, and one child being healthy. We also present a score for monitoring the clinical course and adjusting anticholinesterase therapy accordingly.

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Section: Diskussionunclassified

“…Blutaustauschtransfusion und die Gabe von Immunglobulinen (IgG i.v.) eingesetzt [2,3,6,9,11,13,14,18,27,32]. Während Plasmapherese bzw.…”

Section: Therapieunclassified

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Transiente neonatale Myasthenia gravis

et al. 2002

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“…2,3 AChRs are also present in tissues other than muscle: lymphoid organs are innervated by the autonomic nervous system and lymphocytes have adrenergic and cholinergic receptors. 1,4,5,10 Lymphocyte AChR abnormalities can be expected in CM patients who have muscle AChR deficiency.…”

Section: Lymphocyte Acetylcholine Receptors In Childhood Myastheniamentioning

confidence: 99%

Lymphocyte acetylcholine receptors in childhood myasthenia

1998

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“…[1,2] In patients with autoimmune myasthenia gravis, the binding of specific antibodies to the acetylcholine receptor (AchR) results in degradation, endocytosis, or blockage of the receptor, and destruction of the motor endplate, which results in impaired neuromuscular transmission. Those patients presenting with such disorders during childhood and young adulthood may have juvenile (autoimmune) myasthenia (JM) or congenital myasthenic syndromes (CMS).…”

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confidence: 99%

Juvenile Myasthenia

Anlar

2000

Paediatric Drugs

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Myasthenia in children can be juvenile (autoimmune) or congenital. Juvenile myasthenia (JM) is an autoimmune disorder characterised by fluctuating weakness and fatigue in the ocular, facial, bulbar or limb muscles. Diagnosis is confirmed by electromyography (EMG), single fibre EMG and the patient's clinical response to anticholinesterase medication. Serology is less helpful in children because acetylcholine receptor antibodies, usually positive in adults, are frequently absent in patients with prepubertal onset of the disease. Treatment methods in JM include anticholinesterase drugs, thymectomy and immunomodulatory agents. Plasmapheresis and intravenous immunoglobulin are used in myasthenic crisis. The prognosis of patients with JM is usually good, clinical remission being achieved in the majority of patients with the current treatment methods.

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Myasthenia gravis in childhood

Cited by 38 publications

References 16 publications

Transiente neonatale Myasthenia gravis

Transiente neonatale Myasthenia gravis

Lymphocyte acetylcholine receptors in childhood myasthenia

Juvenile Myasthenia

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