Babinski-Fröhlich Syndrome

Partial destruction of hypothalamic nuclei resulting in hormonal dysfunction with obesity, growth retardation, and hypogonadism.

Adiposogenital Syndrome; Dystrophia Adiposo-Genitalis; Neuropituitary Dystrophy; Cerebral Adiposity; Pituitary Infantilism of Adults; Fröhlich Syndrome; Fröhlich Obesity; Morbus Fröhlich Syndrome; Launois-Cléret Syndrome.

Unknown, but male prevalence.

Several organic lesions of the hypothalamus may cause this disorder, including tumors (craniopharyngioma), encephalitis, Friedreich ataxia, demyelinating diseases, and microcephaly. Originally described as delayed puberty, hypogonadism, and obesity associated with a tumor impinging on the hypothalamus. Presence of obesity indicates damage to the appetite-regulating regions of the hypothalamus. Hypothalamic dysfunction and hypopituitarism mainly affect the somatotropic hormones and the gonadotropins.

Obesity with prepubertal onset affecting breasts, abdomen, femoral regions, and hips. Delayed development of secondary sexual characteristics. Headache, visual impairment, mental retardation. Polyuria and polydipsia as a result of diabetes insipidus.

Based on the clinical and biochemical features (low serum gonadotropin levels; positive vasopressin test for polyuria, if diabetes insipidus is present). Radiologic examinations may show delayed ossification of skeletal structures, suprasellar calcification or destruction, depending on the etiology.

Dehydration as a consequence of diabetes insipidus requires preoperative fluid replacement and check of serum electrolytes. The patient may be anxious and uncooperative because of mental retardation and visual disturbances. Sedative premedication and/or the presence of the primary care giver for induction of anesthesia may be helpful.

Venous access can be difficult. Depending on the grade of obesity, a rapid sequence induction should be considered. Obesity and upper airway obstruction may render face-mask ventilation difficult.

Prader-Labhart-Willi Syndrome: Infantile hypotonia, early childhood-onset obesity, hypogonadism, mental retardation.