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Leksell Gamma Knife : An Effective Non Invasive Treatment for Rare Case of Nelson's Syndrome.

Arshad F, Laway BA, Bhat MA, Irfan Showkat H, Kotwal S, Ahmad Mir S - Int J Endocrinol Metab (2013)

Bottom Line: Nelson's syndrome nowadays a rare entity results from an adrenocorticotropin (ACTH)-secreting pituitary adenoma in patients with refractory Cushing's disease after a therapeutic bilateral adrenal gland removal.We report a case of 25 year old female with cushing's disease who was initially managed with medical treatment, but in view of severe persistent hyper cortisol state was subjected to bilateral adrenalectomy following which she developed Nelson's syndrome after a gap of six years, which was difficult to diagnose because of limited investigations available.Patient was managed with stereotactic radiosurgery (gamma knife surgery).

View Article: PubMed Central - PubMed

Affiliation: Department of Endocrinology, Sher-i-Kashmir Institute of Medical Sciences Soura, Resident Scholar Endocrinology, Srinagar, India.

ABSTRACT
Nelson's syndrome nowadays a rare entity results from an adrenocorticotropin (ACTH)-secreting pituitary adenoma in patients with refractory Cushing's disease after a therapeutic bilateral adrenal gland removal. We report a case of 25 year old female with cushing's disease who was initially managed with medical treatment, but in view of severe persistent hyper cortisol state was subjected to bilateral adrenalectomy following which she developed Nelson's syndrome after a gap of six years, which was difficult to diagnose because of limited investigations available. Patient was managed with stereotactic radiosurgery (gamma knife surgery).

No MeSH data available.


Related in: MedlinePlus

T1 Weighted (Gadolinium enh.) MRI Revealing Large Irregular Sellar Mass With Parasellar Extension With Infiltration of Cavernous Sinus and Encasement of Internal Carotid Artery.
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fig3734: T1 Weighted (Gadolinium enh.) MRI Revealing Large Irregular Sellar Mass With Parasellar Extension With Infiltration of Cavernous Sinus and Encasement of Internal Carotid Artery.

Mentions: Respiratory system, Cardiovascular system and abdominal examinations were non-contributory. Central nervous system examination revealed the right side complete ptosis with reduced ocular movement nasally; left eye reduced ocular motility temporally suggesting homonymous hemianopia. Laboratory evaluation revealed a haemoglobin concentration was 11.3 g/dL and the white cell count 7.3×109/L, with 73% neutrophils, and 22% lymphocytes. Kidney function test revealed urea 38 mg/dL and creatinine 0.68 mg/dL. Liver function test was normal. Lipid profile was deranged revealing total cholesterol 262 mg/dL, triglycerides 193 mg/dL, low density lipoprotein levels (LDL) 190.4 mg/dL and high density lipoprotein levels (HDL) 33mg/dL. Her hormonal profile revealed prolactin < 1 ng/mL (normal range 1-27) , thyroxine 4.05 µg/dL (4-13), thyroid-stimulating hormone (TSH) < 0.15 µIU/mL (0.5-6.5) and adrenocorticotropin hormone (ACTH) levels 206 pg/ mL (10-46). Her 8 am cortisol levels were 6.2 µg/dL (5-25). Due to financial constraints MRI was delayed but later after arranging finances patient was subjected to magnetic resonance imaging (MRI) brain with sellar and parasellar focus which revealed large irregular sellar mass with parasellar extension (especially right side) with infiltration of cavernous sinus and encasement of internal carotid artery (Figure 2). The diagnosis of Nelson’s syndrome with external ophthalmoplegia with right sided ptosis was made on THE basis of her history, present complaints, high ACTH levels and MRI brain.


Leksell Gamma Knife : An Effective Non Invasive Treatment for Rare Case of Nelson's Syndrome.

Arshad F, Laway BA, Bhat MA, Irfan Showkat H, Kotwal S, Ahmad Mir S - Int J Endocrinol Metab (2013)

T1 Weighted (Gadolinium enh.) MRI Revealing Large Irregular Sellar Mass With Parasellar Extension With Infiltration of Cavernous Sinus and Encasement of Internal Carotid Artery.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3860107&req=5

fig3734: T1 Weighted (Gadolinium enh.) MRI Revealing Large Irregular Sellar Mass With Parasellar Extension With Infiltration of Cavernous Sinus and Encasement of Internal Carotid Artery.
Mentions: Respiratory system, Cardiovascular system and abdominal examinations were non-contributory. Central nervous system examination revealed the right side complete ptosis with reduced ocular movement nasally; left eye reduced ocular motility temporally suggesting homonymous hemianopia. Laboratory evaluation revealed a haemoglobin concentration was 11.3 g/dL and the white cell count 7.3×109/L, with 73% neutrophils, and 22% lymphocytes. Kidney function test revealed urea 38 mg/dL and creatinine 0.68 mg/dL. Liver function test was normal. Lipid profile was deranged revealing total cholesterol 262 mg/dL, triglycerides 193 mg/dL, low density lipoprotein levels (LDL) 190.4 mg/dL and high density lipoprotein levels (HDL) 33mg/dL. Her hormonal profile revealed prolactin < 1 ng/mL (normal range 1-27) , thyroxine 4.05 µg/dL (4-13), thyroid-stimulating hormone (TSH) < 0.15 µIU/mL (0.5-6.5) and adrenocorticotropin hormone (ACTH) levels 206 pg/ mL (10-46). Her 8 am cortisol levels were 6.2 µg/dL (5-25). Due to financial constraints MRI was delayed but later after arranging finances patient was subjected to magnetic resonance imaging (MRI) brain with sellar and parasellar focus which revealed large irregular sellar mass with parasellar extension (especially right side) with infiltration of cavernous sinus and encasement of internal carotid artery (Figure 2). The diagnosis of Nelson’s syndrome with external ophthalmoplegia with right sided ptosis was made on THE basis of her history, present complaints, high ACTH levels and MRI brain.

Bottom Line: Nelson's syndrome nowadays a rare entity results from an adrenocorticotropin (ACTH)-secreting pituitary adenoma in patients with refractory Cushing's disease after a therapeutic bilateral adrenal gland removal.We report a case of 25 year old female with cushing's disease who was initially managed with medical treatment, but in view of severe persistent hyper cortisol state was subjected to bilateral adrenalectomy following which she developed Nelson's syndrome after a gap of six years, which was difficult to diagnose because of limited investigations available.Patient was managed with stereotactic radiosurgery (gamma knife surgery).

View Article: PubMed Central - PubMed

Affiliation: Department of Endocrinology, Sher-i-Kashmir Institute of Medical Sciences Soura, Resident Scholar Endocrinology, Srinagar, India.

ABSTRACT
Nelson's syndrome nowadays a rare entity results from an adrenocorticotropin (ACTH)-secreting pituitary adenoma in patients with refractory Cushing's disease after a therapeutic bilateral adrenal gland removal. We report a case of 25 year old female with cushing's disease who was initially managed with medical treatment, but in view of severe persistent hyper cortisol state was subjected to bilateral adrenalectomy following which she developed Nelson's syndrome after a gap of six years, which was difficult to diagnose because of limited investigations available. Patient was managed with stereotactic radiosurgery (gamma knife surgery).

No MeSH data available.


Related in: MedlinePlus