Very rare neurologic disorder characterized by failure
to thrive, emaciation, amnesia, intense sleepiness, unusual eye position,
and sometimes blindness. It is normally seen in infancy or early childhood
but some cases in older children and even adults have been reported.
Diencephalic syndrome usually is caused by a brain tumor such as a low-grade
glioma or astrocytoma.
Russell Diencephalic Cachexia Syndrome; Russell Syndrome
Very rare disorder that affects
males and females equally. Onset age of presentation usually is the young
infant or child between the ages of 18 months and 3 years. However, it also
can develop during adolescence and even adulthood. The syndrome occurs
worldwide. Childhood brain tumors arise in 2.5-3.5:100,000 children per
Results from the development of a brain tumor in
the hypothalamic region, often a low-grade glioma or astrocytoma. The tumor
may invade the anterior portion of the third ventricle and affect the optic
nerve or optic chiasm. The presence of this expanding intracerebral mass
leads to raised intracranial pressure. The cause of the weight loss is
unknown, but excessive growth hormone secretion, activation of proteins
produced by the pituitary gland that break down fat, or secretion of a fat
mobilizing compound have been implicated. There is no known genetic
predisposition for diencephalic syndrome.
Very rare disorder that usually affects infants
and young children. It is characterized by a failure to thrive, emaciation,
and normal linear growth. A history of normal development and weight gain is
normally followed by either a prolonged period of failure to gain weight or
weight loss. The individual may have anorexia or bulimia. Over time, loss of
body fat occurs and leads to an emaciated appearance. The child may have a
relatively large head compared with body weight. Overall development is
often slowed, but results of neurologic testing are normal. The eyes are
often affected, and the infant or child may present a strabismus, nystagmus,
papilledema, or blindness. The child may be unusually sleepy. The patient
usually behaves in a normal, alert, happy manner that is not in keeping with
the physical appearance. Growth hormone plasma level is usually higher than
Presence of emaciation must be assessed
preoperatively for electrolytes and glucose imbalances. Intraoperative
thermoregulatory heat loss must be carefully managed. Presence of elevated
intracranial pressure must be ruled out. If present, proper anesthesia
management for raised intracranial pressure must be ensured. The anesthetic
considerations for brain tumour are not specific to this syndrome and are
reviewed in the textbook entitled Pediatric Anesthesia: Principles and Practice by
the same author.
Tolosa-Hunt Syndrome: Rare disorder that includes headaches,
fever, and vision impairment. There may be eye muscle paralysis, swelling
and protrusion of the eye, drooping eyelid, and diminished vision.
Menzes AH, Bell WE, Perret GC: Hypothalmic tumors in children: Their
diagnosis and management. Child Brain 3:265, 1977.