вход по аккаунту


Chronic hydrocephalus associated with short stature and growth hormone deficiency.

код для вставкиСкачать
Chronic Hydrocephalus
Associated with Short Stature and
Growth Hormone Deficiency
D a n i e l B. H i e r , MD, and Albert C. Wiehl, MD
A 28-year-old m a n with chronic hydrocephalus d u e to aqueductal stenosis was f o u n d to have a subnormal response of
growth hormone to arginine infusion. It is postulated that compression of the pituitary by t h e enlarged t h i r d
ventricle produced a deficiency of g r o w t h h o r m o n e a n d short stature.
Hier DB, Wiehl AC: Chronic hydrocephalus associated with short stature and
growth hormone deficiency. Ann Neurol 2:246-248, 1977
C h r o n i c hydrocephalus may be complicated b y a varie t y of endocrine-related disturbances, including delayed or precocious sexual d e v e l o p m e n t , obesity,
a m e n o r r h e a , hypothyroidism, diabetes insipidus, and
short stature [2-4,7,9,11]. T h e s e abnormalities have
b e e n ascribed t o a disruption of hypothalamicpituitary function b y e n l a r g e m e n t of t h e third ventricle. We have recently evaluated a m a n with s h o r t
stature and longstanding hydrocephalus d u e t o
aqueductal stenosis. His endocrine function was normal except for a s u b n o r m a l r e s p o n s e of g r o w t h hormone to intravenous infusion of argjnine. We suggest
that a selective deficiency of this h o r m o n e , secondary
to compression of t h e pituitary bp an enlarged third
ventricle, may have b e e n responsible for his short
massively enlarged, with downward herniation of the third
ventricle into the sella turcica (Fig 1). The aqueduct, although patent, was ilarrowed in caliber, while the fourth
ventricle was of normal size (Fig 2). The association of a
normal-sized fourth ventricle with a greatly enlarged third
ventricle suggests strongly that the hydrocephalus was due
to aqueductal stenosis.
Endocrine studies included the following normal values:
testosterone 840 ng/ml, thyroxine 5.5 pgidl, free thyroxine
1.3 ng/dl, morning cortisol 11 pgidl, and afternoon cortisol
4 pg/dI. Responses of thyroid-stimulating hormone and
prolactin to infusion of thyrotropin-releasing factor were
also normal (for methods, see [lo]). The patient’s growth
hormone response to intravenous infusion of 30 gm of
arginine hydrochloride was subnormal (Table) and remained so even after pretreatment with dierhylstilbestrol,
2.5 mg twice daily for three days, prior to a repeat arginine
A 28-year-old man was admitted to the Massachusetts General Hospital for evaluation of optic atrophy and an enlarged
sella turcica. Except for short stature, his physical, sexual,
and intellectual development had been normal.
Physical examination revealed a well-developed man with
coarse facial features, a large head, and normal secondary
sexual characteristics. His weight was 74.6 kg, head circumference 60.5 cm (greater than ninety-eighth percentile), and
height 155 cm (less than third percentile). All immediare
family members were 168 cm in height or taller.
Neurological examination showed bilateral optic atrophy
and increased deep tendon reflexes in both lower extremities. Visual acuity was normal: and both plantar responses were flexor. The patient had a full-scale I Q of 105.
Polyromes of the sella turcica showed it ro be enlarged to
at least twice normal size (approximately 2,600 mm3). Opening pressure at the time of pneumoencephalography was
normal. The spinal fluid protein was 7 5 mgidl and the
glucose 70 mg/dl. The lateral and rhird ventricles were
M a n y children with hydrocephalus fail t o attain normal stature, a n d this has been attributed to
hypothalamic-pituitary dysfunction. In this patient,
marked deformation of t h e sella turcica indicates that
t h e third ventricle exerted considerable d o w n w a r d
pressure o n t h e pituitary. T h i s compression of t h e
pituitary may h a v e p r o d u c e d g r o w t h hormone deficiency a n d , ultimately, his s h o r t stature. T h e large
head circumference indicates that t h e hydrocephalus
was longstanding, and it is possible that the g r o w t h
h o r m o n e deficiency was present d u r i n g earlier life,
w h e n linear g r o w t h was occurring.
A deficiency of growth hormone was t h e only e n d o crine abnormality n o t e d in o u r patient. T h i s is compatible with t h e observation that g r o w t h h o r m o n e loss is
From the Neurology and Medical Services, Massachusetts General
Hospital, and the Departments of Neurology-Neuropachology and
Medicine, Harvard Medical School, Boston, MA.
Accepted for publication Mar 29, 1977.
Address reprint requests to Dr Hier, Neurology Service, Massachusetts General Hospital, 32 Fruit St, Boston, MA 021 14.
Growth Hormone Response t o Intravenous
Infasion of Arginine Hydrochloride a
T i m e after
Start of Infusion
G r o w t h H o r m o n e Level"
"Normal response is for the growth hormone level to rise above 17
ngiml after arginine infusion.
hNurnbers in parentheses are thc results of a serond arginine
infusion after the patient was pretreated with diethylstilbestrol, 2.5
mg twice daily for three days.
F i g 1 . A i r j l f i n g anteriorportions of the lateraland third
crentricles during pneumoencephalograpb~i n the brow-up
position. The third 6entrirle is herniated downward into
the enlarged sella turcica.
F i g 2 , Diagvam basedon thepneunzoenc~phaloogram
showing how the enlarged tbird ventricle has expanded the
sella turciru by dou~nu~ard
compression. The d a h d line
Jhow the approximate normal configurution of the
tbird ventricle.
Case Report: Hier and Wiehl: Hydrocephalus and Short Stature
the most sensitive indicator of hypopituitarism, followed by loss of gonadotropin, adrenocorticotropic
hormone, and thyroid-stimulating hormone [S]. Two
other patients with hydrocephalus secondary to
aqueductal stenosis have been reported to have loss of
growth hormone, although both were of normal stature [21.
T h e frequency of endocrine disturbances in association with hydrocephalus due to aqueductal stenosis
should be contrasted to their rarity in cases (if either
empty sella syndrome or communicating hydrocephalus. Patients with empty sella syndrome, like
those with aqueductal stenosis, have an enlarged sella.
However, in cases of empty sella syndrome, sellar
enlargement is due to downward extension of the
subarachnoid space (presumably through a defective
sellar diaphragm) rather than downward herniation of
the third ventricle, as in cases of aqueductal stenosis.
Endocrine abnormalities associated with an empty
sella are either absent or very minor [ 5 , 101.
Patients with communicating hydrocephalus, like
persons with aqueductal stenosis, have enlarged ventricles. In cases of communicating hydrocephalus,
however, the third ventricle does not undergo disproportionate enlargement, and abnormalities of the
sellar region are uncommon [l]. Compression of the
pituitary would not be expected in cases of communicating hydrocephalus, and in fact, endocrine abnormalities have not been reported [6].
Thus, patients with aqueductal stenosis appear to be
at special risk to develop endocrine disturbances because disproportionate enlargement of the third ven-
Annals of Neurology
Vol 2
No 3
tricle produces compression of the pituitary. In the
patient described here, this pituitary compression
may have resulted in a longstanding deficiency of
growth hormone and short stature.
1. DuBoulay G, Trickey S: T h e sella in aqueduct stenosis and
communicating hydrocephalus. BrJ Radio1 43:319-326,1970
2. Fiedler R, Krieger DT: Endocrine disturbances in patients with
congenital aqueductal stenosis. Acta Endocrinol (Kbh) 80:lL
13, I975
3 . Guillaume J, Roge R: Troubles neuroendocrinaires et hydrocephalique chronique. Rev Neurol (Paris) 82:424-427,
4. Kahana L, Lebovirz H , Lusk W', er al: Endocrine manifestations
of intracranial extrasellar lesions. J Clin Endocrinol Metab
22:304-122, 1962
3. Neelon FA, GoreeJA, LebovitL HE: T h e primary empty sella:
clinical and radiographic characteristics and endocrine function. Medicine 52:73-92, 1973
6. Ojemann R G , Fisher CM, Adams R D , er al: Further experiences with the syndrome of normal'^ pressure hydrocephalus.
J Neurosurg 31:279-294, 1969
7. Pollock LJ: Hypopituitarism i n chronic hydrocephalus. JAMA
64:395-398, 1915
8. Rabkin MT, Frantz AG: Hypopituitarism: a study of growth
hormone and other endocrine functions. Ann Intern Med
64:1197-1207, I966
9. Rheim J H W : Hypophysial pressure symptoms due to hydrocephalus causing cystlike distension of the third ventricle.
Arch Neurol Psychiatry 13:?1-79, 1925
10. Ridgway EC, Kourides IA, Kliman B, et al: Thyrotropin and
prolactin pituirary reserve in the "empty sella syndrome." J
Clin Endocrinol Metab 41:968-973, 1975
11. Strauch A: Hypophysial dystrophy in hydrocephalus. JAMA
72:1?31-1?34, 1919
September 1977
Без категории
Размер файла
239 Кб
short, associates, growth, deficiency, stature, hydrocephalus, hormone, chronic
Пожаловаться на содержимое документа