 |  a womanwith fatigue,dec libido,polyuria.
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| Author | 13 Posts |
zaki
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A 35-year-old woman is referred to you for evaluation of a 3-month history of fatigue, diminished libido, polyuria, and polydipsia. For the last month, she has experienced persistent nausea and vomiting. She has had no previous medical illnesses. Physical examination reveals dry skin and sparse pubic and axillary hair. Visual fields are intact. Chest radiograph is normal. Laboratory studies reveal an elevated serum sodium level of 148 meq/L and normal serum glucose and calcium levels, along with the passage of 4000 mL of urine per day with a specific gravity of 1.003. Evaluation of pituitary function reveals marked reduction in basal hormone levels and inadequate responses to stimulation for growth hormone, prolactin, thyrotropin, luteinizing hormone, follicle-stimulating hormone, adrenocorticotropic hormone (ACTH), and cortisol. Magnetic resonance imaging of the brain shows an isointense sellar mass involving the pituitary stalk, with loss of the hyperintense posterior pituitary signal. After replacement therapy with glucocorticoids, thyroxine, and DDAVP (desmopressin), to which the patient had a salutary response, transsphenoidal pituitary surgery was performed.
The most likely diagnosis is:
A) Pituitary adenoma
B) Metastatic carcinoma to the pituitary gland
C) Neurosarcoidosis
D) Lymphocytic hypophysitis
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| prep4usmle
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I'd say it's the pituitary adenoma although that MRI is confusing me.
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| Ouli Maty
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I think C is correct given the MRI results. Adenoma or LH have a similar MRI in that they show enhancing lesions with compression .
There is nothing in the story that may let us think about a carcinoma somewhere, that would metastasize to the brain.
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| dpk
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anyones has any views regarding this question...
i think it is pitu adenoma..
pt definately has SIADH ...
i dont think it is neurosarcoidosis as calcium level is normal..
not metastatic CA as there is no history pointing to it..
all opinions invited..
thanks..
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| dpk
Forum Junior
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oops!!!!!!!!!
not SIADH but DI...
PLZ HELP IF ANYONE CAN?
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| dimps
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plz can any one tell me ,MRI findings in allof these ds ,
it would be of great help
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| drk1980
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mostly bcoz pituitary adenomas are the most common cause of pit hypofunction, A seems good.
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| drdiv
Forum Junior
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lymphocytic hypophysitis
as per mri isointense mass involving the pituitary stalk not the gland itself so not adenoma
hyper intense area involving post pit,
correct me please
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| drk1980
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well whaddya know!! i just fell across the expln! drdiv, excellent job!
Answer: D
Educational Objective: Recognize the causes of diabetes insipidus, and know how to treat them.
This case illustrates the importance of considering alternative causes in the patient presenting with polyuria and polydipsia. In the absence of an elevated serum calcium or glucose level or the administration of an osmotically active agent such as mannitol, the presence of hypernatremic polyuria and polydipsia is consistent with diabetes insipidus. The concomitant presence of other features relating to loss of hypothala-mic/pituitary function suggests that the disease is related to loss of antidiuretic hormone (ADH) (central diabetes insipidus) rather than resistance to its action (nephrogenic diabetes insipidus). The response to administration of DDAVP (desmopressin) further supports this.
Lymphocytic hypophysitis presents most often in women during the peripartum period. However, there have been several reports of this disease in men as well as postmenopausal women. The autoimmune destruction associated with this disease is extremely variable, with some patients experiencing loss of both anterior and posterior function (such as this patient) and others exhibiting loss of only a selected anterior pituitary hormone (such as adrenocorticotropic hormone [ACTH] alone); many have hyperprolactinemia. Similarly, the course of recovery is variable. In some patients, the destruction is associated with an expanding mass that results in compression of adjacent structures and may therefore require surgical decompression. In others, if the disease is highly suspected and other causes unlikely, conservative management with hormone replacement (if necessary) is sufficient. Serial imaging has documented regression of the “tumor mass” with time. Glucocorticoids have not been shown to be of clear value unless a deficiency is noted. Because there is no specific test that confirms the diagnosis other than histologic examination, the diagnosis must be suspected to avoid unnecessary exploratory surgery. In addition to women in the peripartum period, lymphocytic hypophysitis should also be suspected in patients who present with a pituitary mass that is associated with loss of both anterior and posterior pituitary hormones, those in whom medical therapy is ineffective in lowering elevated prolactin levels, and those who present with a rapidly expanding pituitary mass.
ANd to rule OUT the others:
Expanding pituitary adenomas almost never grow to affect ADH synthesis and therefore do not present with diabetes insipidus. Although metastatic disease to the pituitary gland can present with central diabetes insipidus, it is a rare occurrence, especially in a patient of this age. Moreover, the patient has no clinical or biochemical features to suggest systemic sarcoidosis or a neurologic component to this typically infiltrative disease.
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| drhyd
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Well friends , i rule out neurosarcoidosis, as there are no other h/o autoimmune disease and question says they done surgery which is not done in sarcoidosis, only steroids can be done, what we learnt in general sarcoidosis( lung or any other organ)
Its not metastatic carcinoma, because metatstatic cancers lie only at grey white matter junction. they are treated with radiation.not surgery.
WEll how to diffrentiate between adenoma and hyphysistis please let me know.
thank u
drhyd
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| drdiv
Forum Junior
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thanq drk 1980 ,that is a verygood explanation which cleared my doubts
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| AAAAA
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drhyd wrote:
Well friends , i rule out neurosarcoidosis, as there are no other h/o autoimmune disease and question says they done surgery which is not done in sarcoidosis, only steroids can be done, what we learnt in general sarcoidosis( lung or any other organ)
Its not metastatic carcinoma, because metatstatic cancers lie only at grey white matter junction. they are treated with radiation.not surgery.
WEll how to diffrentiate between adenoma and hyphysistis please let me know.
thank u
drhyd
Neurosarcodisosis normally requires USMLE to state the patient is a young black female (most of the times)
Metaststic carcinoma is unlikely without visual disturbance !
Pituitary adenoma (adenoma) is normally not possible to cause panhypopituitary dysfunction without visual disturbance !
Excellent question !
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| AAAAA
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This will not be in the USMLE but possible Internal Medicine or endocrine board !!
Sleep tight !! Sweat dreams !
Introduction: Lymphocytic hypophysitis is a rare autoimmune disease predominantly affecting females in the antepartum and postpartum period. It is characterized by destruction and lymphocytic infiltration of predominantly adenohypophysis with various degrees of hypopituitarism. Posteriour pituitary gland involvement is rare but has been reported. We report a case of a 32 year old lady who presented with diabetes insipidus and hypoadrenalism.
Case: A 32 year old lady complained of polydipsia and polyuria at 7 months gestation but had an otherwise uncomplicated pregnancy. She subsequently presented at 9 weeks post partum with worsening polydipsia and polyuria, generalized arthralgia and weakness. Random cortisol was <30nmol per litre and cranial diabetes insipidus was confirmed on water deprivation testing. MRI pituitary showed a 2cm enhancing pituitary mass causing compression of the optic chiasm on the left side. Visual field testing showed left sided nasal field defect. The patient underwent trans-sphenoidal pituitary exploration and histology confirmed severe lymphocytic hypophysitis with fibrosis. Subsequent dynamic pituitary function testing showed growth hormone and gonadotrophin deficiencies. 5 year follow up reveals the patient to be medically stable. Medications include nasal DDAVP spray, deltacortril, growth hormone and oestrogen replacement in the form of the oral contraceptive pill. Follow up MRI pituitary showed no evidence of disease recurrence.
Conclusion: Lymphocytic hypophysitis should be considered in the differential diagnosis of females with pituitary enlargement presenting in the peripartum period. Close monitoring for multiple hormone deficiencies is indicated in this condition.
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