Lesiones cutáneas específicas en pacientes con neurosarcoidosis / Specific Cutaneous Lesions in Patients With Neurosarcoidosis

La sarcoidosis del sistema nervioso (neurosarcoidosis [NS]) es poco frecuente, pero puede ser grave y producir secuelas importantes. Analizamos los tipos de lesión cutánea específica en pacientes con NS y su utilidad para el diagnóstico de la enfermedad. De un total de 58, 16 presentaron lesiones cutáneas específicas (27,6%). De ellos, 14 eran mujeres y dos varones (edad mediana 50 años [rango 20-84]), que presentaron 24 tipos de lesiones neurológicas (siete neuropatía craneal, cuatro parenquimatosa, tres meníngea, tres mielopatía, uno hipofisaria, dos hidrocefalia y cuatro neuropatía periférica) y 20 lesiones cutáneas específicas (seis máculo-pápulas, nueve placas, uno lupus pernio y cuatro sarcoidosis de las cicatrices [dos pacientes con máculo-pápulas y dos con placas presentaron también sarcoidosis de las cicatrices]. Las lesiones cutáneas estaban presentes al diagnóstico de la NS en 13 casos. Ante la sospecha de esta enfermedad hay que descartar la presencia de lesiones cutáneas específicas cuya biopsia puede acelerar el diagnóstico. (AU)

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis. (AU)

[Translated article] Specific Cutaneous Lesions in Patients With Neurosarcoidosis / Lesiones cutáneas específicas en pacientes con neurosarcoidosis

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis. (AU)

La sarcoidosis del sistema nervioso (neurosarcoidosis [NS]) es poco frecuente, pero puede ser grave y producir secuelas importantes. Analizamos los tipos de lesión cutánea específica en pacientes con NS y su utilidad para el diagnóstico de la enfermedad. De un total de 58, 16 presentaron lesiones cutáneas específicas (27,6%). De ellos, 14 eran mujeres y dos varones (edad mediana 50 años [rango 20-84]), que presentaron 24 tipos de lesiones neurológicas (siete neuropatía craneal, cuatro parenquimatosa, tres meníngea, tres mielopatía, uno hipofisaria, dos hidrocefalia y cuatro neuropatía periférica) y 20 lesiones cutáneas específicas (seis máculo-pápulas, nueve placas, uno lupus pernio y cuatro sarcoidosis de las cicatrices [dos pacientes con máculo-pápulas y dos con placas presentaron también sarcoidosis de las cicatrices]. Las lesiones cutáneas estaban presentes al diagnóstico de la NS en 13 casos. Ante la sospecha de esta enfermedad hay que descartar la presencia de lesiones cutáneas específicas cuya biopsia puede acelerar el diagnóstico. (AU)

Pituitary apoplexy as the first manifestation of non­functioning pituitary neuroendocrine tumour.

Not required for a Clinical Vignette.

A Case of Pituitary Apoplexy Following Leuprolide Injection for Prostate Cancer.

Pituitary apoplexy is a rare but potentially life-threatening complication of androgen deprivation therapy for prostate cancer. We present a case of a 70-year-old African American male with prostate cancer who developed symptoms of pituitary apoplexy, including hot flashes, nausea, vomiting, and cranial nerve III palsy, following the initiation of leuprolide therapy. Imaging revealed a pituitary adenoma with hemorrhage, and prompt multidisciplinary management was initiated. The patient was managed conservatively with improvement in symptoms. This case highlights the importance of recognizing the potential for pituitary apoplexy in patients receiving GnRH agonist therapy. We discuss the clinical presentation of GnRH agonist induced pituitary apoplexy, emphasizing that clinicians should maintain a high index of suspicion and promptly investigate any new neuro- ophthalmic symptoms in this group of patients. Ultimately, prompt diagnosis and treatment are crucial to mitigate the severity of this complication in patients with prostate cancer undergoing androgen deprivation therapy.

Subarachnoid hemorrhage due to pituitary adenoma apoplexy-case report and review of the literature.

Pituitary apoplexy (PA) may be complicated by development of subarachnoid hemorrhage (SAH). We conducted a literature review to evaluate the rate of PA-associated tumor rupture and SAH. We conducted a systematic literature search (PubMed, Web of Science, Medline) for patients with PA-associated SAH and report a case SAH following PA. Suitable articles, case series, and case reports were selected based on predefined criteria following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). We reviewed included publications for clinical, radiological, surgical, and histopathological parameters.We present the case of a patient with PA developing extensive SAH whilst on the MRI who underwent delayed transsphenoidal resection. According to our literature review, we found 55 patients with a median age of 46 years; 18 (32.7%) were female. Factors associated with PA-related SAH were hypertension, diabetes mellitus, prior trauma, anticoagulant, and/or antiplatelet therapy. The most common presenting symptoms included severe headache, nausea and/or vomiting, impaired consciousness, and meningeal irritation. Acute onset was described in almost all patients. Twenty-two of the included patients underwent resection. In patients with available outcome, 45.1% had a favorable outcome, 10 (19.6%) had persisting focal neurological deficits, 7 developed cerebral vasospasms (12.7%), and 18 (35.3%) died. Mortality greatly differed between surgically (9.1%) and non-surgically (44.8%) treated patients. PA-associated SAH is a rare condition developing predominantly in males with previously unknown macroadenomas. Timely surgery often prevents aggravation or development of severe neuro-ophthalmological defects and improves clinical outcome.

A Prospective, Multicenter, Observational Study of Surgical vs Nonsurgical Management for Pituitary Apoplexy.

CONTEXT: Pituitary apoplexy (PA) has been traditionally considered a neurosurgical emergency, yet retrospective single-institution studies suggest similar outcomes among patients managed medically. OBJECTIVE: We established a multicenter, international prospective registry to compare presentation and outcomes in PA patients treated with surgery or medical management alone. METHODS: A centralized database captured demographics, comorbidities, clinical presentation, visual findings, hormonal status, and imaging features at admission. Treatment was determined independently by each site. Key outcomes included visual, oculomotor, and hormonal recovery, complications, and hospital length of stay. Outcomes were also compared based on time from symptom onset to surgery, and from admission or transfer to the treating center. Statistical testing compared treatment groups based on 2-sided hypotheses and P less than .05. RESULTS: A total of 100 consecutive PA patients from 12 hospitals were enrolled, and 97 (67 surgical and 30 medical) were evaluable. Demographics, clinical features, presenting symptoms, hormonal deficits, and imaging findings were similar between groups. Severe temporal visual field deficit was more common in surgical patients. At 3 and 6 months, hormonal, visual, and oculomotor outcomes were similar. Stratifying based on severity of visual fields demonstrated no difference in any outcome at 3 months. Timing of surgery did not affect outcomes. CONCLUSION: We found that medical and surgical management of PA yield similar 3-month outcomes. Although patients undergoing surgery had more severe visual field deficits, we could not clearly demonstrate that surgery led to better outcomes. Even without surgery, apoplectic tumor volumes regress substantially within 2 to 3 months, indicating that surgery is not always needed to reduce mass effect.

Pituitary apoplexy score, toward standardized decision-making: a descriptive study.

Pituitary apoplexy (PA), a rare and life-threatening complication of pituitary adenomas, prompts urgent glucocorticoid administration. The optimal surgical approach is debated, and the Pituitary Apoplexy Score (PAS) aids decision-making. Our retrospective study (2003-2022) assesses variables in PA patient groups (surgical vs. non-surgical), applying PAS to establish a significant threshold for surgical decisions. Additionally, we aim to compare the rates of ophthalmological and endocrine deficit between both groups and identify any associated variables. PAS discrepancies were observed, with averages of 1.7 ± 1.7 (p < 0.0001) for conservative and 3.9 ± 1.7 (p < 0.0001) for surgical groups, confirmed by multivariate analysis (p = 0.009). A PAS threshold of 5, showing over 80% positive predictive value, was established. Patients with low prolactin levels (< 5 ng/ml) had higher corticotropic deficiency prevalence at 3-month and 1-year follow-ups (p = 0.017 and 0.027). Our study supports PAS as a valuable PA management tool, suggesting potential variable adjustments. Multicenter studies are crucial due to PA's low incidence.

Pituitary Apoplexy-associated Cerebral Salt Wasting Syndrome: A Case Report and Literature Review.

PURPOSE: This is a case report of a patient experiencing hyponatremia who was ultimately diagnosed with pituitary apoplexy-associated cerebral salt wasting syndrome (CSWS). METHODS: Laboratory tests, clinical evaluations, and magnetic resonance imaging were performed by specialists. FINDINGS: The patient presented with severe headache, thirst, and polyuria. Results of laboratory tests indicated hyponatremia, decreased plasma osmolality, and elevated urine osmolality. Fluid restriction worsened the situation, and normal saline treatment helped return serum sodium and chloride levels to normal. Pituitary apoplexy-associated CSWS was finally considered. IMPLICATIONS: Pituitary apoplexy is a rare but reasonable etiology for CSWS with hyponatremia. Saline therapy is usually effective.

Subarachnoid haemorrhage associated with pituitary apoplexy and radiographically occult supraclinoid internal carotid artery aneurysms.

In patients with pituitary adenomas, incidental intracranial aneurysms have been documented. Previous studies have highlighted the importance of preoperative imaging in these patients. However, imaging may be limited and fail to show the presence of vascular abnormalities. In this report, we discuss a case of a man in his 30s presenting with a newly diagnosed pituitary adenoma. CT and MRI, on admission, showed a pituitary mass with extension into the right cavernous sinus. After a sudden neurological deterioration, emergent CT/CT angiography revealed pituitary apoplexy with subarachnoid extension without vascular abnormalities. Successful emergency transsphenoidal hypophysectomy was followed by digital subtraction angiography which revealed the presence of two right supraclinoid internal carotid artery aneurysms. With this case, we aim to highlight the need for further vascular imaging in patients with pituitary apoplexy and subarachnoid haemorrhage, as preoperative imaging may be negative for vascular abnormalities especially in the setting of cavernous sinus invasion.

Preventing Postoperative Apoplexy in Giant Pituitary Adenomas using Combined Trans-Sphenoidal and Cranial Surgery.

Background: Combined surgery consisting of endoscopic trans-sphenoidal surgery (ETSS) and transcranial (TC) surgery for giant pituitary adenomas (GPAs) has been recommended to prevent lethal postoperative apoplexy. Based on our experience, we attempt to rationalize the indications for such surgery. Materials and Methods: We report the magnetic resonance (MR) characteristics of the tumor and the outcomes in patients with GPAs who underwent ETSS only and combined surgery. Total tumor volume (TTV), tumor extension volume (TEV), and suprasellar extension of tumor (SET) were calculated based on the lines drawn on MR images and compared between those who underwent ETSS only and those who underwent combined surgery. Results: Of 80 patients with GPAs, eight (10%) underwent combined surgery (seven in the same sitting and one had staged surgery). All eight patients (100%) who underwent combined surgery had tumors with multilobulations, extensions, and encasement of the vessels in the circle of Willis (COW). Of 72 patients who underwent ETSS alone, 21 (29.1%) had a multilobulated tumor, 26 (36.2%) tumors had anterior/lateral extensions, and 12 (16.6%) had encasement of the COW. The mean TTV, TEV, and SET for the combined surgery group were significantly higher than those in the ETSS group. None of the patients who underwent combined surgery suffered postoperative residual tumor apoplexy. Conclusion: Patients with GPAs in whom there are significant lateral intradural or subfrontal tumor extensions should be considered for combined surgery at the same sitting to avoid devastating postoperative apoplexy in the residual tumor, which can occur when ETSS alone is performed.

Natural history of pituitary apoplexy: a long-term follow-up study.

BACKGROUND AND AIMS: Pituitary apoplexy (PA) is a rare neurosurgical emergency, associated with deficiency of one or more pituitary hormones. Few studies have explored the relative outcomes associated with conservative and neurosurgical intervention. METHODS: A retrospective evaluation of all patients with PA reviewed at Morriston Hospital was undertaken and diagnosis was obtained from Morriston database (Leicester Clinical Workstation database) between 1998 and 2019 from clinic letters and discharge summaries. RESULTS: Thirty-nine patients with PA were identified with a mean age of 74.5 years and 20 (51.3%) patients were women. Patients were followed up for a mean±SD 68.1±61.7 months. Twenty-three (59.0%) patients had a known pituitary adenoma. Commoner clinical presentations of PA were ophthalmoplegia or visual field loss. Following PA, 34 (87.2%) patients were noted to have a non-functioning pituitary adenoma (either pre-existing or new), while 5 (12.8%) patients had a pre-existing functional macroadenoma. Neurosurgical intervention was taken in 15 (38.5%) patients of which 3 (20.0%) patients also received radiotherapy, 2 (13.3%) patients had radiotherapy alone and the remainder managed conservatively. External ophthalmoplegia recovered in all cases. Visual loss remained in all cases. One (2.6%) patient with chromophobe adenoma had a significant second episode of PA requiring repeat surgery. CONCLUSION: PA often occurs in patients with undiagnosed adenoma. Hypopituitarism commonly occurred following conservative or surgical treatment. External ophthalmoplegia resolved in all cases, however, visual loss did not recover. Pituitary tumour recurrence and further PA episodes are rare.

Pituitary Apoplexy in Pregnancy: What do We Know?

Pituitary apoplexy refers to a rare clinical syndrome consisting of signs and symptoms that occur due to rapid expansion of the contents of the sella turcica. It can occur spontaneously or associated with pituitary tumors. It can have a broad clinical spectrum, but usually presents with severe headache, visual impairment and hypopituitarism. Sudden onset of symptoms associated to imagiologic confirmation makes the diagnosis. Surgical treatment is advised when there is important compression of the optic tract. We present a case report and a review of the literature on pituitary apoplexy in pregnancy. The cases were reviewed to obtain information on maternal characteristics, clinical presentation, diagnostic studies, therapeutic modalities and maternal and fetal outcomes. Our review found 36 cases of pituitary apoplexy in pregnancy. Most of the cases occurred in the second trimester of pregnancy and headache was the most frequent symptom at presentation. Surgical therapy was required in more than half of the patients. In what respect maternal and fetal outcomes, there were 3 cases of preterm delivery and one case of maternal death. Our clinical case and literature review reinforces the importance of an early diagnosis to avoid potential adverse consequences.

Acromegaly complicated with fulminant pituitary apoplexy: clinical characteristic analysis and review of literature.

PURPOSE: To retrospectively summarize the clinical features of acromegaly complicated with fulminant pituitary apoplexy and analyze the prognostic factors to guide early identification and timely treatment of such patients. METHODS: A retrospective analysis was carried out to summarize the clinical manifestations, hormone changes, imaging, treatment and follow-up of ten patients with acromegaly complicated with fulminant pituitary apoplexy admitted to our hospital from February 2013 to September 2021. RESULTS: The mean age of the ten patients (five males and five females) at the time of pituitary apoplexy was 37.1 ± 13.4 years old. There were nine cases with sudden severe headaches and five cases with visual impairment. All patients had pituitary macroadenomas, of which six cases with Knosp grade ≥3. The level of GH/IGF-1 hormone after pituitary apoplexy was lower compared with pre-apoplexy, and 1 patient reached biochemical remission spontaneously. Seven patients underwent transsphenoidal pituitary surgery after apoplexy and one patient was treated with long-acting somatostatin analog. The biochemical remission rate was 37.5% in eight patients immediately after treatment and 50% at the last follow-up. Patients with Knosp grade ≥3 were less likely to achieve biochemical remission than those with Knosp grade <3 (16.7% vs. 100%, p = 0.048), and patients who achieved biochemical remission had a smaller maximum tumor diameter [20.1 (20.1,28.0) mm vs. 44.0 (44.0,60) mm, p = 0.016]. CONCLUSION: Acromegaly complicated with fulminant pituitary apoplexy remains a diagnostic and therapeutic challenge.

Pituitary apoplexy and cerebral infarction.

Pituitary apoplexy (PA) is a possible complication of pituitary adenoma but is rarely followed by cerebral infarction. The mechanism by which this occurs is not totally understood but is believed to have multiple aetiologies such as arterial compression due to mass effect, vasospasm induced by the presence of blood or by vasoactive agents. In this report, we present a man in his 80s with known pituitary adenoma with a sudden onset of left central facial palsy, left hemiparesis, paresis of the VI left pair and previously unrecognised atrial fibrillation in the ECG. At first, the signs of haemorrhage on imaging were unnoticed, which led to a diagnosis of ischaemic stroke that was submitted to thrombolysis. Due to complications during hospitalisation, the team suspected of PA with panhypopituitarism, confirmed by brain MRI and blood tests. The patient underwent conservative management with glucocorticoids with resolution of the acute adrenal insufficiency related symptoms.

Overcoming anaesthetic challenges in a pregnant woman with pituitary apoplexy undergoing endoscopic trans-sphenoidal resection of the pituitary tumour.

Pituitary apoplexy is a rare condition that occurs due to acute haemorrhage in the pituitary gland and usually presents with features of acute hypopituitarism, visual defects and ophthalmoplegia. Pregnancy is one of the risk factors for pituitary apoplexy. Medical management is the first line of management in pregnancy irrespective of the gestational age. However, acute neuro-ophthalmological deterioration might warrant an urgent surgical decompression. Preoperative corticosteroids have dual benefits of providing hormonal replacement in the mother and also promoting fetal lung maturity in the offspring. Uterine contractions should be monitored and tocolysis provided as per requirements in a post-viable fetus.

Pituitary Apoplexy in Pregnancy: What do We Know? / Apoplexia hipofisária na gravidez: o que sabemos?

Abstract Pituitary apoplexy refers to a rare clinical syndrome consisting of signs and symptoms that occur due to rapid expansion of the contents of the sella turcica. It can occur spontaneously or associated with pituitary tumors. It can have a broad clinical spectrum, but usually presents with severe headache, visual impairment and hypopituitarism. Sudden onset of symptoms associated to imagiologic confirmation makes the diagnosis. Surgical treatment is advised when there is important compression of the optic tract. We present a case report and a review of the literature on pituitary apoplexy in pregnancy. The cases were reviewed to obtain information on maternal characteristics, clinical presentation, diagnostic studies, therapeutic modalities and maternal and fetal outcomes. Our review found 36 cases of pituitary apoplexy in pregnancy. Most of the cases occurred in the second trimester of pregnancy and headache was the most frequent symptom at presentation. Surgical therapy was required in more than half of the patients. In what respect maternal and fetal outcomes, there were 3 cases of preterm delivery and one case of maternal death. Our clinical case and literature review reinforces the importance of an early diagnosis to avoid potential adverse consequences.

Antiplatelet and anticoagulation therapy and the risk of pituitary apoplexy in pituitary adenoma patients.

PURPOSE: Pituitary apoplexy can be a life threatening and vision compromising event. Antiplatelet and anticoagulation use has been reported as a contributing factor in pituitary apoplexy (PA). Utilizing one of the largest cohorts in the literature, this study aims to determine the risk of PA in patients on antiplatelet/anticoagulation (AP/AC) therapy. METHODS: A single center, retrospective study was conducted on 342 pituitary adenoma patients, of which 77 patients presented with PA (23%). Several potential risk factors for PA were assessed, including: patient demographics, tumor characteristics, pre-operative hormone replacement, neurologic deficits, coagulation studies, platelet count, and AP/AC therapy. RESULTS: Comparing patients with and without apoplexy, there was no significant difference in the proportion of patients taking aspirin (45 no apoplexy vs. 10 apoplexy; p = 0.5), clopidogrel (10 no apoplexy vs. 4 apoplexy; p = 0.5), and anticoagulation (7 no apoplexy vs. 3 apoplexy; p = 0.7). However, male sex (p-value < 0.001) was a predictor for apoplexy while pre-operative hormone treatment was a protective factor from apoplexy (p-value < 0.001). A non-clinical difference in INR was also noted as a predictor for apoplexy (no apoplexy: 1.01 ± 0.09, apoplexy: 1.07 ± 0.15; p < 0.001). CONCLUSIONS: Although pituitary tumors have a high risk for spontaneous hemorrhage, the use of aspirin is not a risk for hemorrhage. Our study did not find an increased risk of apoplexy with clopidogrel or anticoagulation, but further investigation is needed with a larger cohort. Confirming other reports, male sex is associated with an increased risk for PA.