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December 1962

Familial Idiopathic Hypoglycemia: Neurological Complications

Author Affiliations

Section on Developmental Neurology, Surgical Neurology Branch, National Institute of Neurological Diseases and Blindness (Dr. Dekaban), and Clinical Endocrinology Branch, National Institute of Arthritis and Metabolic Diseases (Dr. Field), National Institutes of Health, United States Public Health Service, Department of Health, Education and Welfare, Bethesda, Md.; Children's Hospital of the District of Columbia and George Washington University School of Medicine (Dr. Stevens).

Arch Neurol. 1962;7(6):529-537. doi:10.1001/archneur.1962.04210060047003

In 1922 Mann and Magath1 demonstrated for the first time the 3 cardinal findings of experimental hypoglycemia in animals: muscular weakness, convulsions, and coma; the latter leading to death if the animals were left untreated. Association of recurrent convulsions with spontaneous hypoglycemia in man was first described by Ross and Josephs2 in 1924. Since that time, numerous reports appeared on the subject, raising the total number of cases to hundreds.3-9 It soon became apparent to many of these investigators that spontaneously occurring hypoglycemia in the human is a syndrome of multiple etiologies and pathogeneses. However, comprehensive etiological classification of all conditions with abnormally low blood sugar is still not possible. Utilizing refined biochemical methods and hormonal assays, the following major groups of hypoglycemia can be distinguished (modified from Conn and Seltzer10):

1. Hypoglycemia in patients with either adenoma, carcinoma, or hyperplasia of pancreatic islets (included are

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