Pituitary apoplexy and associated cranial nerve palsies secondary to bleeding caused by immune thrombocytopaenia in a patient with known pituitary macroadenoma

An 84-year-old man presented with a frontal headache and easy bruising. He had a background history of a pituitary macroadenoma, diagnosed incidentally a year earlier. Investigations showed haemorrhage into the pituitary macroadenoma leading to a diagnosis of pituitary apoplexy in the context of low platelet count secondary to immune thrombocytopaenia. He was treated with intravenous hydrocortisone, platelet transfusion, intravenous immunoglobulin and high-dose steroid. Neurosurgical intervention was not indicated initially. Five days into his admission, he developed bilateral ptosis and ophthalmoplegia. MRI confirmed further haemorrhage associated with compression of the optic chiasm. He was transferred to a tertiary neurosurgical centre where he underwent urgent surgical decompression. To date, there has been minor improvement in his neurological symptoms. Management of this patient required considerable multidisciplinary teamwork between the clinics of endocrinology, haematology, neurosurgery, ophthalmology and geriatrics.

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Pituitary Apoplexy

Neurosurgery ◽

10.1227/01.neu.0000144840.55247.38 ◽

2005 ◽

Vol 56 (1) ◽

pp. 65-73 ◽

Cited By ~ 149

Author(s):

Patrick L. Semple ◽

Michael K. Webb ◽

Jacques C. de Villiers ◽

Edward R. Laws

Keyword(s):

Pituitary Apoplexy ◽

Average Length ◽

Imaging Modality ◽

Previous History ◽

High Dose ◽

Corticosteroid Administration ◽

Cranial Nerve Palsies ◽

History Of ◽

Dose Corticosteroid ◽

University Of Cape Town

Abstract OBJECTIVE: Pituitary apoplexy is a rare yet potentially fatal disease. We reviewed the combined experience of the University of Virginia in Charlottesville, VA, and Groote Schuur Hospital, University of Cape Town, South Africa, with 62 cases of pituitary apoplexy. METHODS: An Internet web site with a database was constructed, and the records of 62 patients were entered into the database from both institutions and then systematically analyzed. RESULTS: The average age of patients was 51.1 years; 60% were male, and the average length of follow-up was 55 months. The average time of presentation was 14.2 days after the ictus, and 81% had no previous history of pituitary tumor. Headache was the most common presenting symptom (87%). Diminished visual acuity was found in 56% of patients, bitemporal hemianopia in 34%, ocular palsies in 45%, and diminished level of consciousness in 13%. Seventy-three percent of the patients had laboratory evidence of hypopituitarism, and 8% had diabetes insipidus. Fifty-eight patients underwent surgery, 3 were treated conservatively, and 1 died before intervention. Histological examination revealed hemorrhagic infarction in 47%, simple infarction in 40%, and frank hemorrhage in 8%. Seventy-nine percent had a good outcome, although 83% required subsequent hormonal replacement therapy. CONCLUSION: Pituitary apoplexy is often misdiagnosed because the majority of patients have undetected pituitary adenomas, and the presentation is often mistaken for subarachnoid hemorrhage. Most cases of pituitary apoplexy occur spontaneously, although precipitating factors have been suggested. Magnetic resonance imaging is the imaging modality of choice. Treatment includes high-dose corticosteroid administration and surgery. Transsphenoidal surgery is indicated in patients with diminished levels of consciousness, hypothalamic dysfunction, and visual deterioration. Conservative management for patients with isolated cranial nerve palsies has been advocated but remains controversial.

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Metastasis of a Dorsal Melanoma to a Pituitary Adenoma Mimicking Pituitary Apoplexy

Arquivos Brasileiros de Neurocirurgia Brazilian Neurosurgery ◽

10.1055/s-0037-1608907 ◽

2017 ◽

Vol 36 (04) ◽

pp. 238-242

Author(s):

Rui Ramos ◽

Maria Machado ◽

Cristiano Antunes ◽

Vera Fernandes ◽

Olinda Marques ◽

...

Keyword(s):

Differential Diagnosis ◽

Pituitary Adenoma ◽

Adjuvant Radiotherapy ◽

Transsphenoidal Surgery ◽

Pituitary Apoplexy ◽

Clinical Presentation ◽

Pituitary Macroadenoma ◽

Histology Result ◽

History Of ◽

Transcranial Approach

AbstractMetastases to pituitary adenomas are very rare. From the 20 cases found in the literature, none originated from a cutaneous melanoma. We present the case of a 67-year-old man with a history of transcranial approach to treat a pituitary macroadenoma followed by adjuvant radiotherapy. Fifteen years later, he presented a dorsal nodular melanoma, and three years after that, he developed symptoms of pituitary apoplexy. He was submitted to transsphenoidal surgery, and the histology result revealed metastasis of the melanoma into a pituitary adenoma.The similarity in the clinical presentation of the two entities—pituitary apoplexy and metastasis of the melanoma into a pituitary adenoma—and the rarity of this type of metastization alert to challenges in the differential diagnosis that may confound the neurosurgeon's decision.

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Stubborn hiccups as a sign of massive apoplexy in a naive acromegaly patient with pituitary macroadenoma

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-17-0044 ◽

2017 ◽

Vol 2017 ◽

Author(s):

Gulay Simsek Bagir ◽

Soner Civi ◽

Ozgur Kardes ◽

Fazilet Kayaselcuk ◽

Melek Eda Ertorer

Keyword(s):

Pituitary Apoplexy ◽

Pituitary Surgery ◽

Optic Chiasm ◽

Oral Glucose ◽

List Type ◽

Pituitary Macroadenoma ◽

Large Tumor ◽

Gh Secretion ◽

Transsphenoidal Pituitary Surgery ◽

Magnetic Resonance Imaging Mri

Summary Pituitary apoplexy (PA) may very rarely present with hiccups. A 32-year-old man with classical acromegaloid features was admitted with headache, nausea, vomiting and stubborn hiccups. Pituitary magnetic resonance imaging (MRI) demonstrated apoplexy of a macroadenoma with suprasellar extension abutting the optic chiasm. Plasma growth hormone (GH) levels exhibited suppression (below <1 ng/mL) at all time points during GH suppression test with 75 g oral glucose. After treatment with corticosteroid agents, he underwent transsphenoidal pituitary surgery and hiccups disappeared postoperatively. The GH secretion potential of the tumor was clearly demonstrated immunohistochemically. We conclude that stubborn hiccups in a patient with a pituitary macroadenoma may be a sign of massive apoplexy that may result in hormonal remission. Learning points: Patients with pituitary apoplexy may rarely present with hiccups. Stubborn hiccupping may be a sign of generalized infarction of a large tumor irritating the midbrain. Infarction can be so massive that it may cause cessation of hormonal overproduction and result in remission.

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SAT-273 Case of Mistaken Identity?

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1229 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Carlos Pla

Keyword(s):

Muscle Weakness ◽

Pituitary Adenomas ◽

Complete Resolution ◽

Pituitary Apoplexy ◽

Optic Chiasm ◽

Visual Disturbance ◽

Pituitary Macroadenoma ◽

Patient Reported ◽

Work Up

Abstract Introduction Pituitary adenomas occur in 10-15% of patients and the majority are benign. Prolactinomas are the most common form of secretory pituitary adenoma. Pituitary apoplexy, a medical emergency with resulting visual loss and hormonal hyposecretion, requires rapid surgical intervention. We present a case of pituitary macroadenoma that underwent pituitary resection for acute visual disturbance which was later discovered to be caused by undiagnosed demyelinating disease. Clinical Case Patient is a 32-year-old male who presented initially with complain of fatigue and decreased libido. Work up revealed elevated prolactin level and low testosterone. MRI showed a 2x3cm pituitary macroadenoma. At moment of diagnosis, patient was otherwise asymptomatic. He was started on bromocriptine. During follow up visits, patient reported visual disturbance. First MRI in our clinic showed no suprasellar extension, no impingement of optic chiasm and nonspecific white matter disease. At that time, visual field testing showed left temporal defect in superior quadrant. Follow up MRI 1 year later continued to show a stable macroadenoma without impingement of the optic chiasm, but patient reported progressive left vision disturbance and new right vision loss. He was evaluated in the emergency room where he was treated for pituitary apoplexy with steroids and surgery. Vision improved the next day. Despite uncomplicated post-operative course, patient developed proximal muscle weakness and exam notable for diffuse motor deficit in bilateral lower extremities with hyperreflexia. Endocrinology workup was negative for hypercortisolism and ophthalmology diagnosed him with optic neuropathy. Neurology evaluation led to a diagnosis of multiple sclerosis (MS). Patient was started on natalizumab with complete resolution of all visual and muscle symptoms. Clinical lesson Our patient presented with complaints of fatigue, decreased libido and work up that showed a macroprolactinoma without MRI evidence of optic chiasm impingement. During treatment, he developed acute visual deficits that were attributed to pituitary apoplexy. This visual disturbance improved after surgery and use of high dose IV steroids, with the latter likely treating what had been an MS flare. In hindsight, ophthalmologic evaluation before surgery had shown new color blindness, a sign of optic neuropathy. Despite temporary relief, patient progressed to develop new muscle weakness and recurrent visual disturbance which led to the diagnosis of MS. Since being diagnosed and treated for MS, he has had complete resolution of his symptoms. This case stresses the importance of considering other etiologies for visual defects in patients with pituitary adenomas.

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Chiasmal Syndromes

Neuro-Ophthalmology ◽

10.1093/med/9780190603953.003.0011 ◽

2019 ◽

pp. 61-66

Author(s):

Matthew J. Thurtell ◽

Robert L. Tomsak

Keyword(s):

Visual Field ◽

Clinical Features ◽

Pituitary Apoplexy ◽

Optic Chiasm ◽

Surgical Decompression ◽

Visual Field Defects ◽

Pituitary Macroadenoma ◽

Visual Recovery ◽

Field Defects ◽

Pituitary Macroadenomas

Dysfunction of the optic chiasm typically produces bitemporal hemianopic visual field defects. Optic chiasmal dysfunction most often results from compression by extrinsic lesions, such as pituitary macroadenomas and meningiomas. In this chapter, we begin by describing the various bitemporal hemianopic visual field defects that can occur with optic chiasmal dysfunction. We next list potential causes of optic chiasmal dysfunction. We then review the clinical features and evaluation of pituitary apoplexy, which results from infarction of (or hemorrhage into) a pituitary macroadenoma. Lastly, we discuss the management of pituitary apoplexy, including the indications for and timing of surgical decompression, and review factors that affect the prognosis for visual recovery.

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Pituitary Macroprolactinoma Apoplexy in a Prepubertal Girl

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1435 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A704-A705

Author(s):

Sabliny Carreiro Ribeiro ◽

Angélica Cristina Dall agnese ◽

Laís Marques Mota ◽

Cesar Geremia ◽

Marina Bressiani ◽

...

Keyword(s):

Visual Field ◽

Pituitary Apoplexy ◽

Serum Cortisol ◽

Optic Chiasm ◽

Breast Development ◽

Free T4 ◽

Prepubertal Girl ◽

History Of ◽

The Central Nervous System ◽

Hormonal Evaluation

Abstract Background: Prolactinomas are rare in children, with an incidence of 1: 10.000.000 cases, representing less than 2% of all intracranial tumors in this age group (1). Indeed, only a few cases are reported in prepubertal children. Clinical Case: Eight-year old female prepubertal child, previously healthy, presented a progressive loss of the visual acuity in the last 5 months and was admitted to the emergency department with an intensive headache associated with nauseas, started 48 hours before the admission. At the physical examination, the patient was eutrophic, with an infantile genitalia and no breast development or galactorrhea. No focal neurologic deficits were detected and presented normal pupils reflex and preserved extrinsic eye movements. The Campimetry evidenced bitemporal heteronymous hemianopsia and the nuclear magnetic resonance of the central nervous system identified a suprasellar and intrasellar expansive lesion measuring 3.2 x 2.6 x 2.3 cm, with bleeding signs and compression of the optic chiasm. The hormonal evaluation showed: free T4 0.55 ng/dL (normal value: 0.93-1.70ng/dL), total T4 4.8ng/dL (5.1-14.1ng/dL), TSH 4.06µUI/mL (0.6-5.4µUI/mL), morning serum cortisol 1.80mcg/dL (6.2-2mcg/dL), ACTH 7.0pg/mL (<46pg/mL), prolactin 3.376ng/mL (4.8-23.3ng/mL). The patient initially started glucocorticoid replacement, subsequently levothyroxine and cabergoline. A diagnosis of macroprolactinoma with pituitary apoplexy and optic nerve compression was performed and confirmed by the Immunohistochemical after the transsphenoidal resection. No history of any neoplasms were reported by family members and calcium abnormalities were excluded in the child. A significant improvement of the visual field was suddenly observed and the prolactin substantially decreased to 35ng/mL within 30 days after the intervention. CONCLUSION: We report a rare case of macroprolactinoma in a prepubertal girl associated with pituitary apoplexy. Complete assessment and prompt intervention were essential to recover the visual field and to prevent major sequelae. Reference: (1) Yang, A., Cho, S. Y., Park, H., Kim, M. S., Kong, D.S., Shin, H.J., & Jin, D.K. (2020). Clinical, Hormonal, and Neuroradiological Characteristics and Therapeutic Outcomes of Prolactinomas in Children and Adolescents at a Single Center. Frontiers in Endocrinology. 2020; 11:527.

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Immunoglobulin G4 hypophysitis in a 63-year-old woman with no autoimmune history: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-03018-7 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Zachary C. Gersey ◽

Kenan R. Rajjoub ◽

Thomas M. Pearce ◽

Scott A. Segel ◽

Paul A. Gardner ◽

...

Keyword(s):

Autoimmune Disease ◽

Response To Treatment ◽

White Female ◽

High Dose ◽

Demographic Differences ◽

Immunoglobulin G4 ◽

Case Presentation ◽

Cranial Nerve Palsies ◽

History Of ◽

Pituitary Lesion

Abstract Background Immunoglobulin-G4-related hypophysitis is a rare inflammatory disease that can present as a tumefactive pituitary lesion mimicking hypophyseal neoplasms such as pituitary adenoma or craniopharyngioma. The literature on this entity is sparse, with fewer than 100 cases reported across 19 publications; a recent review found only 24 cases published from 2007 to 2018. Previous reports have described demographic differences, with immunoglobulin-G4-related hypophysitis in females tending to present in the second and third decades in association with other autoimmune disease, while males tend to present in the fifth and sixth decades of life without an autoimmune history. Case presentation In contrast to the reported demographic trends, here we describe a unique case of immunoglobulin-G4-related hypophysitis in a 63-year-old white female with no history of autoimmune disease who presented with a rapidly enlarging sellar and hypothalamic mass causing headaches and cranial nerve palsies, prompting biopsy for diagnosis. The patient experienced rapid response to treatment with high-dose steroids and rituximab. Conclusion The case contributes to the growing clinicopathologic description of immunoglobulin-G4-related hypophysitis and illustrates that this diagnosis should be a consideration even outside the conventional demographic setting.

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Abstract #507 Panhypopituitarism Secondary to Pituitary Macroadenoma with Optic Chiasm Compression and Possible Apoplexy

Endocrine Practice ◽

10.1016/s1530-891x(20)46851-x ◽

2019 ◽

Vol 25 ◽

pp. 252

Author(s):

Olesya Petrenko ◽

Wilhelmine Wiese-Rometsch

Keyword(s):

Optic Chiasm ◽

Pituitary Macroadenoma

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ITI with high-dose FIX and combined immunosuppressive therapy in a patient with severe haemophilia B and inhibitor

Hämostaseologie ◽

10.1055/s-0037-1617018 ◽

2009 ◽

Vol 29 (02) ◽

pp. 155-157 ◽

Cited By ~ 22

Author(s):

H. Hauch ◽

J. Rischewski ◽

U. Kordes ◽

J. Schneppenheim ◽

R. Schneppenheim ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Immunosuppressive Agents ◽

Tolerance Induction ◽

Intravenous Immunoglobulins ◽

Factor Ix ◽

High Dose ◽

Allergic Reactions ◽

Success Rates ◽

Serious Infections ◽

History Of

SummaryInhibitor development is a rare but serious event in hemophilia B patients. Management is hampered by the frequent occurrence of allergic reactions to factor IX, low success rates of current inhibitor elimination protocols and the risk of development of nephrotic syndrome. Single cases of immune tolerance induction (ITI) including immunosuppressive agents like mycophenolat mofetil (MMF) or rituximab have been reported. We present a case of successful inhibitor elimination with a combined immune-modulating therapy and high-dose factor IX (FIX). This boy had developed a FIX inhibitor at the age of 5 years and had a history of allergic reactions to FIX and to FEIBA→. Under on-demand treatment with recombinant activated FVII the inhibitor became undetectable but the boy suffered from multiple joint and muscle bleeds. At the age of 11.5 years ITI was attempted with a combination of rituximab, MMF, dexamethasone, intravenous immunoglobulins and high-dose FIX. The inhibitor did not reappear and FIX half-life normalized. No allergic reaction, no signs of nephrotic syndrome and no serious infections were observed.

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