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Biomedical subjects

P Derome

Publications and source records attributed to P Derome.

At least 19 recordsLinked to original sources

Thalamocortical diaschisis: positron emission tomography in humans.

To investigate further the relations between cortical energy metabolism and neuropsychological impairment after unilateral thalamic lesion, 55 patients underwent positron emission tomography studies of either cortical oxygen consumption or glucose utilisation, including eight repeat studies, at times ranging from 4 days to 98 months after the onset of the lesion [stroke (n = 44) or stereotaxic VL-Vim thalamotomy performed for movement disorders (n = 11)]. Patients with thalamotomy were also studied preoperatively and the surgery induced a significant fall in cortical metabolism on both sides (more so ipsilaterally); post-operatively the magnitude of the ipsilateral cortex hypometabolism was positively correlated to the severity of global neuropsychological impairment; similar but less significant findings were obtained for the ipsilateral/contralateral cortical metabolic asymmetry. With respect to the whole patient sample, the cortical metabolic asymmetry was initially pronounced, with subsequent monoexponential recovery, in the cognitively impaired study group, but it was only mild and showed no meaningful trend for recovery in the cognitively unaffected study group; yet even soon (< 3 months) after thalamic lesion there was a noticeable overlap of individual asymmetry values among the two study groups. These results lend further support to the view that the neuropsychological impairment that frequently follows unilateral thalamic lesions is reflected in a depression of synaptic activity in both the overlying and the contralateral cerebral cortices. For individual patients, this study also illustrates the potentially misleading nature of the measured cortical metabolic asymmetry with respect to neuropsychological status, especially at late times after lesion, in part because side to side metabolic ratios do not reflect bilateral changes.

Adult

Apparently silent somatotroph adenomas.

We describe here 9 patients with somatotroph adenomas associated with mild features of acromegaly and basal plasma GH levels in the normal range. In 5 women and 4 men, 26 to 61 yrs old, the diagnosis of prolactinoma or non-secreting pituitary adenoma had been previously made on the basis of amenorrhea-galactorrhea or tumoral symptoms. However, they had discrete signs of coarsening of the facial features and moderate but evolutive changes of hand and foot sizes. Basal GH levels were in the normal range (0.4 to 4.5 micrograms/l, N less than 5 micrograms/l) but unaffected by oral glucose and insulin tolerance tests while IGF-I concentrations were elevated in all the cases (range 1.7 to 5.8 U/ml, N: 0.37-1.41 U/ml). Plasma PRL concentrations were elevated in 5 patients (range 16 to 80 micrograms/l, N less than 13 micrograms/l in men and N less than 19 micrograms/l in women). The 9 patients had a macroadenoma with an extrasellar extension in 8 of them and all were operated on by the transsphenoidal route. Immunocytochemical studies demonstrated IRGH-cells in all the adenomas and IRPRL-cells in 5 of them. Electron microscopic analysis of 3 tumors showed that the secretory granules were sparse and the Golgi apparatus poorly developed. Molecular biology of 7 tumors showed the presence of small amounts of GH mRNA. This result was in agreement with the morphological aspect, suggesting a low rate of GH synthesis. Thanks to these different approaches the diagnosis of silent somatotroph adenoma should sometimes be reconsidered.(ABSTRACT TRUNCATED AT 250 WORDS)

Acromegaly

Pituitary enlargement with suprasellar extension in functional hyperprolactinemia due to lactotroph hyperplasia: a pseudotumoral disease.

Beside the well characterized PRL-secreting adenomas, a wide spectrum of functional hyperprolactinemic states exists. We describe here five women, 21-38 yr old, all suspected of having a PRL-secreting adenoma because of a pseudotumoral appearance of the pituitary on computerized tomographic (CT) scan or magnetic resonance imaging (MRI). Four had oligomenorrhea with or without galactorrhea, one had amenorrhea with galactorrhea, and two complained of infertility. In the same patient, basal plasma PRL levels were variable on different days, sometimes normal (mean +/- SEM, 11.3 +/- 1.5 micrograms/L), sometimes elevated (49 +/- 7 micrograms/L), but in all cases, a PRL response of large amplitude to TRH (6- to 8-fold increase in the basal value) was observed. Basal plasma levels of estradiol were within luteal phase normal values (0.41 +/- 0.13 pmol/L), while progesterone levels were low (1.92 +/- 0.47 nmol/L). CT scan or MRI showed an intrasellar mass with suprasellar extension, suggesting a tumoral process. However, the signal intensity was homogeneous, and on coronal views, the suprasellar extension was pyramidal and symmetrical, and the pituitary stalk was always in the midline. The five patients were operated on by the transsphenoidal route, but no adenoma was found. Surgical biopsies were taken in four cases, and lactotroph hyperplasia, i.e. enlarged cell cords consisting mainly of PRL cells, was found in three of them. One case displayed a continuum between areas of lactotroph hyperplasia and adenomatous PRL cells. We conclude that functional hyperprolactinemia may mimic on CT scan or MRI a PRL-secreting adenoma.

Adult

The effect of somatostatin analogue on chiasmal dysfunction from pituitary macroadenomas.

The long-acting somatostatin analogue SMS 201-995 has been shown to be efficient in the treatment of somatotropic and thyrotropic adenomas. In some cases, it can suppress adenoma secretion and lead to tumor shrinkage. Pituitary macroadenomas are often associated with a vision-threatening chiasmal syndrome. In this series, SMS 201-995 was administered subcutaneously to eight patients with pituitary macroadenomas of various types responsible for severe long-lasting visual defects. An obvious improvement of both visual fields and acuity occurred in six patients, in two of these during the first 4 to 6 hours of treatment; in two patients, gonadotropic adenomas were unresponsive. Maximal improvement (normalization of visual fields in three cases) occurred within 6 to 45 days and was sustained during the 1- to 12-month follow-up period. This effect seems independent of the type of adenoma since the adenomas secreting growth hormone (GH) and thyroid-stimulating hormone and silent corticotropic-secreting adenomas responded as well as did two of the non-functioning adenomas. In one acromegalic patient visual improvement was obtained while the abnormal GH secretion remained unaltered. In all cases but one, no tumor shrinkage could be demonstrated. These data demonstrate that SMS 201-995 can rapidly improve the chiasmal syndrome due to pituitary macroadenoma, and suggest that this effect might be independent of a reduction in tumor volume.

Adenoma

[Treatment of post-neurosurgical diabetes insipidus with desmopressin by intramuscular route].

Desmopressin (DDAVP) is used intramuscularly in the treatment of post operative diabetes insipidus as soon as the condition is diagnosed to ensure continuous replacement of antidiuretic hormone secretion during the first 5 days of therapy. Two successive studies, each involving 15 patients, were conducted. The first study was designed to test the effectiveness and detect the possible side effects of intramuscular DDAVP, while the purpose of the second study was to evaluate the clinical application of the drug. With seven 2 mcg doses of DDAVP, administered 12-hourly by intramuscular injection to patients weighing more than 30 kg, continuous antidiuresis during 96 hours was achieved. This method is simple and effective, but it should not be prolonged beyond that period of time. Moreover, to prevent plasma hypo-osmolality, fluid intake must be strictly controlled and kept at the same level as or below diuresis.

Adolescent

Absence of receptors for thyrotropin (TSH)-releasing hormone in human TSH-secreting pituitary adenomas associated with hyperthyroidism.

In patients with TSH-secreting pituitary adenomas associated with hyperthyroidism, TSH secretion usually does not respond to exogenous TRH stimulation. To determine the basis for this unresponsiveness, we studied TRH binding to the membranes of two such TSH-secreting pituitary adenomas. The patients, a 28-yr-old man and a 60-yr-old woman, had clinical and biochemical hyperthyroidism with increased serum TSH levels (15.7 and 14 mU/L, respectively; normal range, 1.1-7.2) and alpha-subunit to TSH molar ratios greater than 1 (2.4 and 1.7, respectively). Neither patients had an increase in serum TSH in response to TRH (200 micrograms, iv). Immunocytochemistry of the two adenomas, removed by transsphenoidal surgery showed a pure population of thyrotropic cells. Binding studies were performed by incubation of tumor cell membrane suspensions with increasing amounts of [3H]TRH. Two PRL-secreting adenomas and one normal human pituitary were used as controls. The characteristics of the TRH-binding sites from the control tissues were similar to those previously reported (Kd, 56, 30, and 49 nM; maximum binding, 187, 46, and 94 fmol/mg protein, respectively). In contrast, no specific TRH binding was found in the two TSH-secreting adenomas. We conclude that the unresponsiveness of TSH after TRH administration is related to the absence of specific TRH-binding sites in these thyrotropic tumors.

Adenoma

Transsphenoidal pituitary surgery for the treatment of Cushing's disease: results in 64 patients and long term follow-up studies.

Transsphenoidal pituitary surgery was performed in 64 patients with Cushing's disease in search of a corticotroph adenoma. In 4 patients, profuse local bleeding prevented completion of the exploration. Of the 60 patients who had an adequate exploration, 4 could not be followed after surgery. Short term assessment of the surgical outcome (3-6 months postoperatively) was performed on 60 patients, including the 4 who had incomplete pituitary exploration. Forty-two patients (70%) were judged as immediate successes [urinary cortisol excretion, less than 90 micrograms (less than 248 nmol)/day]; the mean urinary cortisol excretion and plasma ACTH level fell from 463 +/- 70 (+/- SE) to 26.7 +/- 3.6 micrograms/day (1277 +/- 193 to 74 +/- 10 nmol/day; n = 33) and from 111 +/- 33 to 36 +/- 14 pg/mL (24 +/- 7 to 8 +/- 3 pmol/L; n = 23) in patients who had both measurements pre- and postoperatively. Eighteen patients (30%) were judged as immediate failures; neither urinary cortisol excretion nor plasma ACTH levels changed significantly in patients who had both measurements pre- and postoperatively. The preoperative epidemiological, clinical, hormonal, and radiological characteristics of the 2 groups were similar. Histological examination of pituitary fragments removed in 58 of the 60 patients evaluated postoperatively revealed the presence of tumoral tissue in a higher percentage of patients in the immediate success group (72%) than in the immediate failure group (24%; P less than 0.01). The 42 patients in the immediate success group were followed from 6 months to 7 yr (median, 2 yr); 6 patients had recurrences from 2-3 yr after operation. Actuarial analysis indicates that the probability of a patient remaining well 6 yr after surgery is 72 +/- 20% (95% confidence limit). Most of the patients in the immediate success group had transient ACTH deficiency preceding a progressive return to normal pituitary-adrenal function.

Adenoma

[Stereotaxic guidance in open surgery of the brain].

The authors describe the technique used to precisely locate, before surgical ablation, small superficial or deep seated intracerebral lesions. When they are superficial, a burrhole is placed above the lesional site, that is reconstructed by superimposing the CT image onto a plain film radiogram taken during stereotaxy. Deep seated lesions are localized by inserting a guide under stereotaxy, prior to open surgery, which will lead the neurosurgeon without error from the cortex to the lesion. 37 observations are reported where this technique lead to a greatly simplified intervention with minimal cortical damage.

Brain

[Middle-term course of craniopharyngiomas in children as a function of initial therapeutic choice].

Despite numerous studies and publications, the treatment of craniopharyngiomas in children remains controversial. The present series of 33 cases, followed for the last 10 years, is analysed according to therapeutic protocols jointly defined, case by case, from each patient's features and in restricting the extent of surgical excisions. In agreement with other recently published series, two options give superior results: complete excision, when the risk is low; in the other cases, partial excision or rather a simple biopsy or decompression, followed by irradiation. Risks of relapse are thus quite reduced and mortality greatly reduced. The unavoidable consequence of hypopituitarism is easily treated. However, the frequency of psychic and/or neurologic sequellae as well as the risk of post-radiation complications should not be disregarded when selecting treatment.

Adolescent

Surgical treatment of prolactinomas. Short- and long-term results, prognostic factors.

All 347 patients surgically treated for a prolactinoma from January 1, 1976 to December 31, 1982, in the neurosurgical ward of Foch Hospital, were retrospectively studied. The frequency of postoperative normalisation of plasma prolactin (PRL) depends on prolactinoma size, preoperative PRL level, duration of first clinical symptom, previous oestroprogestative contraception, and adenoma necrosis. Postoperative PRL values were normalized in 75% of small prolactinomas (grade 0, 1 or 2) with preoperative PRL values less than 200 ng/ml, and clinical duration less than 5 years (n = 102). There was no operative death and minor morbidity (2.7%). Among the 96 patients with postoperative PRL normalisation, operated between 1976 and 1979, 70 were followed up for an average time of 4.4 +/- 0.2 years. 17% of patients had hyperprolactinemia recurrence with a delay of 1.5 +/- 0.4 years. Postoperative PRL levels near the upper normal limit, and weak PRL response to TRH tests were found to be unfavourable prognostic factors for hyperprolactinemia recurrence. Pregnancy did not increase the risk of recurrence, but could reflect genuine long-lasting remission. Selective adenomectomy remains an interesting treatment for prolactinoma, particularly if the adenoma is small, recent and with PRL moderately increased. The frequency of postoperative PRL normalisation after surgery is less than with bromocriptine, but surgery is the only treatment able to achieve a definitive cure with a low iatrogenic risk.

Adenoma

[Loss of consciousness during benign intracranial hypertension. Correlations between cerebrospinal fluid pressure and the electroencephalogram].

A patient with benign intracranial hypertension developed paroxysmal attacks of falling due to loss of consciousness. Prolonged simultaneous recordings of CSF pressure, through a lumbar needle, and of EEG showed that these episodes resulted from bouts of elevation of CSF pressure (120 mm Hg systolic pressure). The pathophysiology of the increased pressure waves, described as Lundberg 's A waves or plateau waves, is discussed. The analogy between EEG records and those observed in normal subjects during Valsalva's manoeuvre is a further argument in favor of cerebral anoxia during the increased pressure plateau, this being apparently the explanation for the clinical disorders.

Electroencephalography

Fronto-orbito-nasal dislocations. Secondary treatment of sequelae.

Fronto-orbito-nasal dislocation untreated or maltreated can have many important sequelae for both function and aesthetics. Two types of cases may be observed, those with: -extensive bone loss -malunited fracture. The treatment of sequelae caused by bone loss in order to be rational, logical and complete necessitates: 1. protection of the underlying elements = eye and meningo-cerebral tissues, 2. reconstitution of an harmonious cranio-facial profile, 3. definitive isolation of cranial contents from the facial cavities. 4. repair of nasal and orbital walls and the necessary ligamentous re-insertions on them. The best material for such a repair remains the autogenous bone graft. The treatment of sequelae caused by malunited fractures necessitates repositioning osteotomies. In addition to rotation, elevation and translation osteotomies the following may be required: -either a monoblock advancement osteotomy, -or an "expansion" osteotomy. They require solid fixation. This may be obtained by: -either superior fixation to a previously conserved intermediate frontal strut -or a rebuilding of the shape of the vault, laterally fixed to sound bone parts, and an interposed bone graft to act as a "keystone" of the vault. Such repairs seem to be better than simple onlay bone grafts which are useful from an aesthetic point of view, but which neglect the underlying injuries with their concomitant functional sequelae, and which expose the patient to secondary sinus or cerebro-meningeal complications. They permit a single, total, simultaneous, and definitive repair of all the sequelae both functional and aesthetic; an approach which is more rational than successive surgical sessions. But they require: 1. Dura mater repair by suture and pericranial grafting. 2. Total isolation of cranial contents from the face. 3. Obliteration of the frontal sinus. It is therefore necessary to work with a combined neuro- and maxillo-facial team. Four cases are described to illustrate these proposed procedures, one case with very extensive bone loss, three with malunited fractures: the first orbito-frontal, the second with a fronto-zygomatic lateral component, the third with a fronto-nasal medical component.

Bone Transplantation

Acromegaly: analysis of 132 cases treated surgically.

The surgical treatment of 132 acromegalics is analyzed. There was an equal sex distribution among patients between the ages of 30 and 59. In the younger patients there was a preponderance of males, and the opposite situation occurred in patients older than 59 years. The overall cure rates were 58% (growth hormone (GH) less than 5 ng/ml) and 78% (GH less than 10 ng/ml). Endocrinological evidence of cure was observed most commonly in patients who exhibited preoperative GH values below 70 ng/ml.

Acromegaly