[An association of Werdnig-Hoffman disease and pregnancy: apropos of an unusual case]. 1990

F Laffargue, and P Boulot, and L Lafont, and O Jonquet, and B Hedon, and J L Viala
Services de Gynécologie-Obstétrique, Montpellier.

The authors describe the first case in the literature of pregnancy in a twenty year old patient suffering from Werdnig Hoffman's disease. This is pure progressive spinal muscular atrophy which involves respiratory and locomotor functions. It brings into consideration several problems of respiration and in particular of genetics to be considered in this case, and we report that pregnancy was well tolerated in the patient who had a severe physical handicap.

UI MeSH Term Description Entries
D009134 Muscular Atrophy, Spinal A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089) Bulbospinal Neuronopathy,Oculopharyngeal Spinal Muscular Atrophy,Progressive Muscular Atrophy,Scapuloperoneal Form of Spinal Muscular Atrophy,Spinal Muscular Atrophy,Adult Spinal Muscular Atrophy,Adult-Onset Spinal Muscular Atrophy,Amyotrophy, Neurogenic Scapuloperoneal, New England Type,Distal Spinal Muscular Atrophy,Hereditary Motor Neuronopathy,Muscular Atrophy, Adult Spinal,Myelopathic Muscular Atrophy,Myelopathic Muscular Atrophy, Progressive,Progressive Myelopathic Muscular Atrophy,Progressive Proximal Myelopathic Muscular Atrophy,Proximal Myelopathic Muscular Atrophy, Progressive,Scapuloperoneal Spinal Muscular Atrophy,Spinal Amyotrophy,Spinal Muscular Atrophy, Distal,Spinal Muscular Atrophy, Oculopharyngeal,Spinal Muscular Atrophy, Scapuloperoneal,Spinal Muscular Atrophy, Scapuloperoneal Form,Adult Onset Spinal Muscular Atrophy,Amyotrophies, Spinal,Amyotrophy, Spinal,Atrophies, Progressive Muscular,Atrophy, Myelopathic Muscular,Atrophy, Progressive Muscular,Atrophy, Spinal Muscular,Bulbospinal Neuronopathies,Hereditary Motor Neuronopathies,Motor Neuronopathies, Hereditary,Motor Neuronopathy, Hereditary,Muscular Atrophies, Progressive,Muscular Atrophy, Myelopathic,Muscular Atrophy, Progressive,Neuronopathies, Bulbospinal,Neuronopathies, Hereditary Motor,Neuronopathy, Bulbospinal,Neuronopathy, Hereditary Motor,Progressive Muscular Atrophies,Spinal Amyotrophies
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011248 Pregnancy Complications Conditions or pathological processes associated with pregnancy. They can occur during or after pregnancy, and range from minor discomforts to serious diseases that require medical interventions. They include diseases in pregnant females, and pregnancies in females with diseases. Adverse Birth Outcomes,Complications, Pregnancy,Adverse Birth Outcome,Birth Outcome, Adverse,Complication, Pregnancy,Outcome, Adverse Birth,Pregnancy Complication
D012131 Respiratory Insufficiency Failure to adequately provide oxygen to cells of the body and to remove excess carbon dioxide from them. (Stedman, 25th ed) Acute Hypercapnic Respiratory Failure,Acute Hypoxemic Respiratory Failure,Hypercapnic Acute Respiratory Failure,Hypercapnic Respiratory Failure,Hypoxemic Acute Respiratory Failure,Hypoxemic Respiratory Failure,Respiratory Depression,Respiratory Failure,Ventilatory Depression,Depressions, Ventilatory,Failure, Hypercapnic Respiratory,Failure, Hypoxemic Respiratory,Failure, Respiratory,Hypercapnic Respiratory Failures,Hypoxemic Respiratory Failures,Respiratory Failure, Hypercapnic,Respiratory Failure, Hypoxemic,Respiratory Failures
D004417 Dyspnea Difficult or labored breathing. Orthopnea,Platypnea,Recumbent Dyspnea,Rest Dyspnea,Trepopnea,Breathlessness,Shortness of Breath,Breath Shortness,Dyspnea, Recumbent,Dyspnea, Rest,Dyspneas, Rest
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014897 Spinal Muscular Atrophies of Childhood A group of recessive inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3) Infantile Spinal Muscular Atrophy,Juvenile Spinal Muscular Atrophy,Kugelberg-Welander Disease,Muscular Atrophy, Spinal, Infantile,Spinal Muscular Atrophy, Infantile,Spinal Muscular Atrophy, Juvenile,Werdnig-Hoffmann Disease,HMN (Hereditary Motor Neuropathy) Proximal Type I,Kugelberg-Welander Syndrome,Muscular Atrophy, Infantile,Muscular Atrophy, Juvenile,Muscular Atrophy, Spinal, Infantile Chronic Form,Muscular Atrophy, Spinal, Intermediate Type,Muscular Atrophy, Spinal, Type I,Muscular Atrophy, Spinal, Type II,Muscular Atrophy, Spinal, Type III,Proximal Hereditary Motor Neuropathy Type I,SMA, Infantile Acute Form,Spinal Muscular Atrophy 1,Spinal Muscular Atrophy Type 2,Spinal Muscular Atrophy Type I,Spinal Muscular Atrophy Type II,Spinal Muscular Atrophy Type III,Spinal Muscular Atrophy, Mild Childhood and Adolescent Form,Spinal Muscular Atrophy, Type 3,Spinal Muscular Atrophy, Type I,Spinal Muscular Atrophy, Type II,Spinal Muscular Atrophy, Type III,Type I Spinal Muscular Atrophy,Type II Spinal Muscular Atrophy,Type III Spinal Muscular Atrophy,Werdnig Hoffman Disease,Infantile Muscular Atrophy,Juvenile Muscular Atrophy,Kugelberg Welander Disease,Kugelberg Welander Syndrome,Werdnig Hoffmann Disease

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