Diagnostic pitfall: Optic neuritis mimicking optic nerve glioma

We report a case of optic neuritis with enlargement of the optic nerve on MRI. This neuroradiological finding is most commonly seen in tumours of the optic nerve and led together with an inconclusive intraoperative frozen section to an incorrect tentative diagnosis of optic nerve glioma which fatally resulted in the resection of the optic nerve.     

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy

We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis.     

Cerebrospinal fluid myelin basic protein-like material in acute monosymptomatic optic neuritis

Introduction
Cerebrospinal fluid (CSF) may provide markers of severity and outcome of optic neuritis.
Material and methods We examined the CSF from 29 patients with acute monosymptomatic optic neuritis (AMON) for content of myelin basic protein-like material (MBPLM) and correlated the levels with results of cranial magnetic resonance imaging. The length of the time from onset of AMON to the time of CSF collection did not exceed four weeks.
Results Only two patients (7%), one of whom developed an acute myelopathy one month after AMON, showed an elevated value of CSF MBPLM. No MBPLM was detected in 6 patients (21%), and other 21 (72%) had detectable levels of MBPLM but below the upper limit of normal of 0.1 ng/ml. The value of MBPLM was not significantly correlated with the interval to CSF sampling from onset of AMON or with severity of decreased visual acuity. The highest values of CSF MBPLM were observed among patients with severely decreased visual acuity and among patients with an abnormal MRI (13 of 27 i.e. 48%).
Conclusions CSF MBPLM was rarely abnormal in AMON. However, CSF MBPLM may have potential value in reflecting disease activity, as the highest values were obtained among patients with CSF sampled soon after the maximum visual dysfunction was reached, with severe visual impairment, and with an abnormal MRI.     

33例垂体卒中的临床分析

目的探讨和观察垂体卒中的临床特点及最佳治疗方案。方法回顾性分析2000—2006年我科收治的33例垂体卒中患者的临床资料,根据患者手术方式分为开颅组及经蝶组,比较术后患者视力改善情况及垂体功能低下的发生率。结果本组经蝶手术23例,其中视力完全恢复13例,明显恢复7例,略恢复3例,无视力障碍加重;10例术后发生垂体功能低下,其中6例需长期激素替代治疗。开颅手术10例,其中视力完全恢复3例,明显恢复3例,略恢复2例,视力障碍加重2例;8例术后发生垂体功能低下,其中5例需长期激素替代治疗。结论经蝶入路手术治疗垂体卒中创伤小、并发症少、疗效确实,术后垂体功能低下发生率低,可作为垂体卒中的首选手术入路。     

急性垂体卒中后视功能改变的临床分析

目的 探讨垂体卒中后视功能损害的原因、临床特点及最佳治疗方案.方法 回顾性分析37例垂体卒中患者的临床资料,包括临床表现、影像学检查、治疗方案和预后.结果 本组经蝶窦手术27例,其中视力完全恢复17例,明显恢复7例,略恢复3例.10例术后发生垂体功能低下,其中6例需要长期激素替代疗法.经颅于术治疗10例,视力完全恢复3例,明显恢复3例,略恢复2例,视力障碍加重2例.8例术后出现垂体功能低下,其中5例需要长期激素替代治疗.结论 经蝶窦手术较经颅手术对视力影响小、并发症少,尽早手术能有效逆转视力损害.     

亚临床型垂体腺瘤卒中的诊治

目的总结203例亚临床型垂体腺瘤卒中患者临床特点、诊断、处理与结果。方法回顾性分析亚临床型垂体腺瘤卒中病人203例,149例行经鼻蝶垂体腺瘤切除术,54例行开颅切除手术。患者术前术后均作内分泌学检查,影像学检查为cT或MRI。结果所有病人视觉损害、头痛或垂体功能得到不同程度改善。泌乳素腺瘤是本组病例中最常见病理类型。大腺瘤及巨腺瘤患者容易发生亚临床型垂体腺瘤卒中。术后随访有24例垂体腺瘤复发。19例接受放疗。9例术后长期服用甲状腺素替代药物,8例服用类固醇激素。结论亚临床型垂体腺瘤卒中的发病率比急性卒中高。亚临床型垂体腺瘤卒中的发病机制尚不清楚。磁共振优于cT诊断亚临床型垂体腺瘤卒中。经鼻蝶手术治疗亚临床型垂体腺瘤卒中安全有效。     

垂体腺瘤卒中危险因素分析

目的 分析垂体腺瘤卒中的危险因素。方法 选取2020年6月—2021年12月中南大学湘雅医院诊治的50例垂体腺瘤患者,根据影像及手术资料分为垂体瘤合并卒中组（23例）和垂体瘤不合并卒中组（27例）,分析卒中因素。结果 多因素Logistic分析显示垂体瘤手术切除史、垂体瘤最大直径大是其影响因素。其中有1例伽玛刀治疗后出现肿瘤亚临床卒中的特殊病例。结论 有垂体瘤手术切除史、垂体瘤最大直径大的患者是发生卒中的高危因素,伽玛刀治疗后出现影像学上的肿胀,可能是亚临床卒中。     

Manifestation, management and outcome of subclinical pituitary adenoma apoplexy

The objective of this study was to investigate the clinical features, diagnosis, treatment and outcomes of subclinical pituitary adenoma apoplexy (SPAA) in 185 consecutive patients between January 1990 and May 2007. Of the 185 patients, 133 (71.89%) underwent transsphenoidal tumor decompression and the remaining 52 patients (28.11%) underwent transcranial surgery. Preoperative and postoperative endocrinological hormone concentrations were measured in all patients. Pituitary imaging was obtained by CT scans or MRI. Follow-up outcomes were obtained from the records of outpatient visits and by telephone interviews. Visual disturbance, headache and pituitary function impairment improved significantly in all patients. Prolactinoma was the most frequent type of pituitary adenoma in our series (51.89%). SPAA usually occurred in patients with large or giant adenomas (85.95%). Postoperative follow-up ranged from 1 year to 17 years with a mean of 7.4 ± 1.6 years. The tumor recurred in 23 patients, 18 of whom were treated with postoperative radiotherapy for either residual tumors (n = 8) or recurrence (n = 10). Long-term thyroid hormone replacement was necessary in eight patients and steroid hormone replacement in six patients. The incidence of SPAA is relatively high compared with that of acute pituitary apoplexy. The exact pathogenic mechanism of SPAA remains unknown. MRI is significantly better than CT scans for detection of SPAA. Transsphenoidal decompression is safe and effective. Surgical decompression should be performed as early as possible.     

亚急性垂体卒中49例临床分析

目的探讨亚急性垂体卒中的诊断及评价手术治疗效果。方法亚急性垂体卒中49例,其中男27例,女22例,分别采用额下入路、翼点入路、经鼻—蝶窦入路行垂体腺瘤切除术。结果 术后对视力损害、视野缺损及眼肌麻痹疗效满意,有效率分别为84.04％、79.22％、100.00％。结论 亚急性垂体卒中临床表现以视力、视野损害及头痛为主,症状多不典型,头颅CT/MRI扫描有助于明确诊断,应在术前行完整的内分泌功能评估,于常规应用肾上腺糖皮质激素的基础上择期手术治疗,其手术术式不能一概而论。     

Changes in the retinal veins in acute optic neuritis

OBJECTIVE: To investigate patients with acute optic neuritis (ON) for changes of the retinal veins. MATERIAL AND METHODS: Seventy-six patients with acute ON were extensively neuro-ophthalmologically examined. RESULTS: Multiple sclerosis (MS) was found in 41 patients of whom 1 had periphlebitis retinae (PR) and 2 had venous sheathing (VS). Probable MS was found in 15 patients without prior symptoms of MS. One had PR and VS, and 2 had VS. Twenty patients had mono-symptomatic ON, none had retinal changes. CONCLUSION: Changes of the retinal veins should alert the clinician to a probable diagnosis of MS. ON appears frequently as the initial symptom of MS. Our observation of VS in these patients suggest that clinically silent retinal disease activity had occurred prior to the ON.     

急性垂体腺瘤卒中病人行经蝶手术前后的垂体功能

目的探讨急性垂体卒中病人在经蝶手术前后的垂体功能变化情况.方法回顾性分析25例急性垂体卒中病人的临床及内分泌资料,其中伴垂体-甲状腺轴受损9例,肾上腺功能低下14例,性腺功能低下11例.13例在人院后3 d内手术,12例在1周内手术.结果伴垂体-甲状腺轴受损者术后恢复5例,肾上腺功能低下者恢复8例,性腺功能低下者恢复7例.结论急性垂体卒中多合并垂体功能低下表现,早期手术有助于恢复正常垂体功能.对于术后仍存在垂体功能低下表现者应给予相应激素替代治疗.     

Pituitary apoplexy: a transient benign presentation mimicking mild subarachnoid hemorrhage with negative angiography

We report on a patient who presented with isolated transient headache as the only manifestation of pituitary apoplexy. A high index of suspicion and MRI led to the diagnosis.     

Preclinical stress originates in the rat optic nerve head during development of autoimmune optic neuritis

Optic neuritis is a common manifestation of multiple sclerosis, an inflammatory demyelinating disease of the CNS. Although it is the presenting symptom in many cases, the initial events are currently unknown. However, in the earliest stages of autoimmune optic neuritis in rats, pathological changes are already apparent such as microglial activation and disturbances in myelin ultrastructure of the optic nerves. αB-crystallin is a heat-shock protein induced in cells undergoing cellular stress and has been reported to be up-regulated in both multiple sclerosis and its animal model, experimental autoimmune encephalomyelitis. Therefore, we wished to investigate the timing and localization of its expression in autoimmune optic neuritis. Although loss of oligodendrocytes was not observed until the later disease stages accompanying immune cell infiltration and demyelination, an increase in oligodendrocyte αB-crystallin was observed during the preclinical stages. This was most pronounced within the optic nerve head and was associated with areas of IgG deposition. Since treatment of isolated oligodendrocytes with sera from myelin oligodendrocyte glycoprotein (MOG)-immunized animals induced an increase in αB-crystallin expression, as did passive transfer of sera from MOG-immunized animals to unimmunized recipients, we propose that the partially permeable blood–brain barrier of the optic nerve head may present an opportunity for blood-borne components such as anti-MOG antibodies to come into contact with oligodendrocytes as one of the earliest events in disease development.     

Bilateral and recurrent optic neuritis in multiple sclerosis

Burman J, Raininko R, Fagius J. Bilateral and recurrent optic neuritis in multiple sclerosis.
Acta Neurol Scand: 2011: 123: 207–210.
© 2010 John Wiley & Sons A/S. Objective – To assess the frequency of bilateral and recurrent optic neuritis (ON) in multiple sclerosis (MS) and to compare these results with epidemiological data of ON in neuromyelitis optica (NMO) and recurrent ON without other signs of disease. Methods – We identified 472 patients with diagnosis of MS from the Swedish Multiple Sclerosis Register. These patients were evaluated for the presence of ON and whether the ON was the presenting symptom of MS; unilateral or bilateral; monophasic or recurrent. Results – Twenty‐one percent presented with ON as their first manifestation of MS. The proportion of patients developing a second attack of ON before demonstration of other manifestations of MS was 5.5% and the frequency of recurrent bilateral ON as the presenting symptom was 3.8%. Only two patients presented with simultaneously appearing bilateral ON corresponding to 0.42%. Conclusion – Recurrent ON, whether unilateral or bilateral, is a common presentation of MS. As MS is a much more common disease than NMO, care must be taken when evaluating the work‐up of patients with recurrent ON. In some cases repeated MRI and lumbar punctures are warranted to improve diagnostic accuracy, even in the presence of the serological marker NMO‐IgG.     

New therapeutic perspectives for demyelinating retrobulbar optic neuritis

In patients with demyelinating retrobulbar optic neuritis (RON), a spontaneous or corticosteroid-induced improvement is generally observed within the first month, but this is clinically insignificant in 5%-7% of patients. We report the case histories of four patients who were considered to be "non-responders" to corticosteroids because their visus remained unchanged or had improved by only 1/10 after one month from intravenous corticoid therapy begun 2-7 days after disease onset, and who were therefore subsequently administered high intravenous doses of immunoglobulin. Three of these patients completely recovered in a period of 3-9 months; the fourth showed only a partial improvement, but this was consolidated after long-term continuation of the same therapy. These cases suggest the possible efficacy of early administration of intravenous immunoglobulin in RON patients who fail to respond to cortisone therapy. As recently demonstrated in animal models, it can be hypothesised that the result is due to immuno-mediated mechanisms of action that reduce autoimmune responses in the short- and medium-term, and in the long-term favour remyelination.     

Optic radiation changes after optic neuritis detected by tractography-based group mapping

Postmortem data suggest that trans-synaptic degeneration occurs in the lateral geniculate nucleus after optic nerve injury. This study investigated in vivo the optic radiations in patients affected by optic neuritis using fast marching tractography (FMT), a diffusion magnetic resonance imaging (MRI) fiber tracking method, and group mapping techniques, which allow statistical comparisons between subjects. Seven patients, 1 year after isolated unilateral optic neuritis, and ten age and gender-matched controls underwent whole-brain diffusion tensor MR imaging. The FMT algorithm was used to generate voxel-scale connectivity (VSC) maps in the optic radiations in each subject in native space. Group maps of the left and right optic radiations were created in the patient and control group in a standardized reference frame using statistical parametric mapping (SPM99). The reconstructed optic radiations in the patient group were localized more laterally in the posterior part of the tracts and more inferiorly than in the control group. Patients showed reduced VSC values in both tracts compared with controls. These findings suggest that the group mapping techniques might be used to assess changes in the optic radiations in patients after an episode of optic neuritis. The changes we have observed may be secondary to the optic nerve damage.     

Magnetic resonance imaging of the brain in patients with acute monosymptomatic optic neuritis

Ophthalmologists and neurologists from a catchment area of 1.5 million people were asked to refer all patients with symptoms and signs of optic neuritis (ON) to our neurological department immediately. A total of 68 consecutive patients were extensively screened for known etiologies of ON. Eleven had definite multiple sclerosis (MS); 7 patients could not participate for various reasons. The cerebrum, cerebellum and brainstem of the remaining 50 untreated patients with monosymptomatic ON were MRI scanned at 1.5 T with 2 sequences (slice thickness 4 mm, voxel size 1.2 x 1.2 x 4 mm3): double spin-echo (TR = 1.8 s, TE = 30 and 90 ms, 12 slices axially) and inversion recovery (TR = 2.45 s, TI = 400 ms, TE = 30 ms, 5 slices sagittaly). Magnetic resonance imaging (MRI) was performed within 3-49 (median 16) days from onset of symptoms. MRI demonstrated multiple asymptomatic central nervous system (CNS) lesions in 62% of patients. The appearance, locations and extents of lesions were consistent with demyelination. A scoring system based on number and size of lesions is proposed. Seven of 50 patients developed definite MS during a median follow-up of 11 months; they all had CNS lesions on the first scan. MRI has thus improved the pathophysiological knowledge of idiopathic ON, which from the very onset is a manifestation of MS in at least two thirds of cases. Patients who have early CNS lesions run a much higher risk of later developing clinically definite MS.     

Cell-mediated immunity in acute idiopathic optic neuritis and multiple sclerosis

Lymphocyte transformation responses to phytohaemagglutinin, measles antigen and tuberculin and the absolute numbers of circulating T and SIg+ cells were determined in 16 patients with acute idiopathic optic neuritis (ON), 42 patients with multiple sclerosis (MS) and 78 healthy controls.
Patients with acute ON showed impaired lymphocyte transformation responses in both autologous plasma and AB serum similar in extent to those seen in MS patients in relapse. They were not associated with a reduced total number of circulating T cells.