Visual Troubles Research Articles

Pineal cysts in children: a paediatric series treated over the last twenty years in Lyon.

Pineal cysts are a rare lesion of the pineal gland. Pineal cysts are benign lesions, generally asymptomatic, and are usually an incidental discovery on MRI performed for other problems. The management of pineal cysts in children remains a matter for debate. Here, we report our own retrospective paediatric cases that have been surgically treated and review the paediatric literature on this topic. This is a retrospective monocentric study. All patients operated by the senior author (CM) for a benign pineal cyst from 2000 to 2021 were included. All other pineal region cystic lesions were excluded. Medical and surgical data were extracted from the hospital medical database. Twelve patients were included. The clinical symptomatology was characterized by headaches in seven patients, visual troubles in two patients, precocious puberty in one patient, signs of intracranial hypertension in two patients, seizures associated with headache in one patient, and headaches associated with behavioural troubles in another patient. No major post-operative complications were observed in this series. It is to noted that surgery was performed because a suspicion of a true pineal parenchymal tumour has been made. Histopathological study came back with the diagnosis of pineal cyst. Pineal cyst is rare. If the radiological diagnosis is clear, no surgery is advocated except in cases associated with hydrocephalus and rapid growth. In case of a suspicion of a true pineal parenchymal tumour, a surgery may be needed to confirm the diagnosis. Lastly, we stress that only cystic lesions of the pineal gland itself should be considered as pineal cyst.

The Effect of Changing Colours on Central Crowding Reading

Background: Crowding can be defined as the impaired recognition of closely spaced objects. Changing colour and lighting enhance visual comfort and perceptual troubles that influence impaired vision reading. Objective: The current study was aimed to investigate the impact of changing the flanker distance and unflanked targets with colours on central crowding reading for subjects with their distant best correction (BCVA) equal to or greater than 6/6. Methodology: Six native English speakers (age: 18–38) who participated in a cross-section intervention study were asked to identify the orientation of the letter E (flanked or unflanked) in different directions around the central target in different colours (red, green, blue and black) on a white background. Results: Different colours affect central crowding (p<0.05). However, the central crowding reading of red was not affected by changing flankers (P > 0.05). Conclusion: Central reading crowding is visual crowding. Different colours affect central crowding. However, the central crowding reading in red was not affected by changes in flankers.

Surgery versus conservative care for Rathke's cleft cyst

BackgroundRathke's cleft cysts are benign cystic lesions of the sellar region, which may cause headache, pituitary deficiencies and visual disturbances from mass effect. Their management is not standardized yet. This study is about establishing a consensus for medical care of RCC. Material and methodsWe performed a retrospective observational study of all patients that were diagnosed or followed for RCC between 2008 and 2018 (11 years), in the neurosurgical and the adult endocrine departments of our institution. The study's average time length of follow-up is 72.9 months (from 2 to 385 months). ResultsThe 57 included patients were divided into 2 groups: group A, which included 39 patients that were conservatively managed and group B, which included 18 surgically treated patients. Group A showed either an improvement or a spontaneous resolution of headaches in 56.1% of the cases (P<0.01); a resolution of hyperprolactinemia in 70% of the cases (P=0.21); and of hypogonadism, ACTH deficiency, growth hormone deficiency in 100% of the cases. There was no spontaneous improvement of visual disturbances (P<0.01) or diabetes insipidus (P=0.29) during follow-up. Regarding group B, surgery allowed improvement or complete resolution of headaches in 60% of the cases; visual troubles in 100% of the cases (P<0.01); and hyperprolactinemia in 100% of the cases. Pituitary deficiencies were not improved by surgery. ConclusionsThis study offers guidance in decision-making regarding the management of RCC patients. Surgery is particularly suitable for treating visual disturbances caused by RCC. Regular follow-up is more appropriate than surgery concerning headaches, hyperprolactinemia, endocrine disruptions and diabetes insipidus.

The historical background of Jules and Augusta Dejerines’ work

The historical background of Jules and Augusta Dejerines’ work

Hydroxychloroquine retinopathy in a cohort of patients from upper Egypt

Purpose To identify risk factors and state demographics of a cohort of patients from upper Egypt experiencing hydroxychloroquine (HCQ) retinopathy. This was an observational study. This study was conducted in Sohag University Hospital, Egypt. Patients and methods The medical records of the rheumatology department were retrospectively reviewed to identify patients who stopped taking HCQ owing to its induced visual troubles. Ten patients were identified and were subjected to complete ophthalmological examination in addition to automated perimetry and optical coherence tomography of the macula. Results All patients (nine females and one male) had received HCQ 200 mg twice daily for years. The mean age was 59.1 years, mean real weight was 83.3 kg, mean height was 1.63 m, mean ideal body weight was 56.72 kg, and the mean daily dose per kg of ideal weight was 7.14 mg/kg/day, whereas the mean daily dose per kg of real weight was 4.85 mg/kg/day. Conclusion Calculation of HCQ dose according to the ideal weight is a better predector of retinal toxicity than real body weight.

Primary pituitary lymphoma: an update of the literature.

Primary intracranial lymphomas (Weller et al. in Neuro Oncol 14(12):1481-1484, 2012) are an emerging disease and an isolated localization in the pituitary gland i.e. primary pituitary lymphoma (PPL) represents a rare condition. We present an update of the most recent evidence for PPL through a systematic review of the literature. A systematic literature review was conducted using PubMed database up to October 2015. The population was defined as immunocompetent patients with a pathologically confirmed diagnosis of PPL. Patients' characteristics, clinical presentation, radiological features, pathology reports, adjuvant treatment and follow-up data were analyzed. We reported one case of PPL and included our data in this analysis. A total of 33 cases of PPL were identified, including ours. A slight not significant female prevalence was evident, with a mean age of 59 years at diagnosis. Visual troubles and headaches were the most common presenting symptoms. About 80 % of patients presented a cranial nerve (CN) deficit. The most frequently involved were the II and III CN. Anterior hypopituitarism was present in 70 % of cases and a diabetes insipidus in 36 % of cases. PPL was rarely limited to the sella and most often extended to the suprasellar and parasellar space. 70 % of cases underwent resection, 21 % a biopsy. A B-cell lymphoma was isolated in 82 % of cases, a T-cell lymphoma in 15 % and a NK/T cell lymphoma in one case. Overall mean survival rate was 14.4 months (95 % confidence interval 9.0-19.8 months) and there was no difference in terms of survival rates when patients were stratified according to the treatment they received. PPL is an emerging clinical entity. Literature data are too scarce to allow the definition of specific protocols of treatment and the management is based on the guidelines present for PCNSL. The role of surgery aiming at a complete resection of PPL should be reevaluated in wider studies including only this category of patients, to establish the real role of each therapeutic strategy.

FSH-Producing Pituitary Macroadenoma: Report of 2 Cases with Clinical Manifestations of Hormone Excess

ABSTRACT Objective: Gonadotropinomas with signs related to hypergonadotropinemia have been rarely published. We report 2 clinical cases of follicle-stimulating hormone (FSH)-producing macroadenomas showing manifestations of hormone hypersecretion. Methods: The clinical, laboratory, and imaging findings are presented with a review of the literature. Results: A 51-year-old man was admitted to the hospital because of cranioencephalic trauma following seizures. He reported previous altered behavior, depression, sphincter incontinence, and visual troubles. Physical examination revealed signs of hypogonadism and increased bilateral testicular volume. Magnetic resonance imaging (MRI) showed a large lesion in the sellar-suprasellar area, with mass effect. Hormone testing showed high levels of FSH (517 mIU/mL) and low levels of both luteinizing hormone (LH) (2.2 mIU/mL) and testosterone (1.3 ng/mL). A partial resection of an immunopositive FSH adenoma was performed; FSH declined after surgery, but the patient died...

Pituitary tumors and pyramidal syndrome

Giant pituitary tumors (≥ 4 cm in diameter) are more frequent in men. Their main manifestations are headaches with sexual and visual troubles. But, sometimes they can have severe neurological complications, such as psychiatric troubles, hydrocephalus, memory troubles, meningitis, and epilepsy. To our best knowledge, pyramidal and cerebellar syndromes are exceptional. Our aim was to describe two men with giant PT associated to pyramidal syndrome with or without the cerebellar one. Case 1 A 38 years old man was sent for a large prolactinoma infiltrating the cerebellum [Prolactin = 376 ng/ml, tumor size: 61 mm (height), 50 (transverse diameter) and 81 (antero posterior diameter)] with gonadal deficit and uncommon neurological signs, such as right central facial paralysis, pyramidal syndrome in lower and upper limbs, dysarthria and static and kinetic cerebellar syndrome. Neurological symptoms and signs, which began one year ago, disappeared miraculously two weeks after starting bromocriptine and reduction in the tumor size. He is now speaking, walking and working normally. Case 2 A man aged 63, suffering from right hemi paresis for two years and recent vomiting was sent to our department for a non functioning PT (42 × 35 × 60) with hydrocephaly. On clinical examination apart from pituitary insufficiency, he had left facial paralysis and pyramidal syndrome. Cerebral MRI showed a multidirectional tumor with hydrocephalus and invasion of cerebral trunk. Conclusion Both PT has an important invasion of the brainstem, which explains pyramidal, and cerebellar syndromes. Those tumors need to be recognized as they are life threatening.

Giant pituitary tumors secreting growth hormone in children and adolescents

Introduction Pituitary tumors secreting growth hormone (GH) are relatively rare in children and adolescents. Giant ones (maximal diameter ≥ 4 cm) are even rarer. We aimed to analyze their frequency and their complications in Algerian population. Methods It is a retrospective and multicenter study over a long period of time (1980–2013) which aimed to collect giant pituitary tumors secreting GH in order to analyze their characteristics. Results Among 31 well proved somatotroph adenomas observed in children and adolescents (≤ 20 years old) or in older subjects with lack of pubertal development, we found 12 giant tumors = 38.7%. Median age at diagnosis was 22.4 years old (13–30). Eight on 12 suffered from gigantism (66.6%) other had acromegaly. Median tumor diameter = 60.5 mm (40–100). Median GH = 570 ng/mL (30–1430). All had moderate to severe visual troubles. Two or more pituitary deficits were observed in 4 cases = 33.3%, hydrocephalus = 8 cases (66.6%), frontal syndrome = 3 = 25% and epilepsy n = 1 = 8.3%. Conclusion In our population giant tumors secreting GH are frequent and diagnosed late even in those with gigantism which explains the high frequency of severe and life threatening neurological complications.

Nasal Bleeding Due to Huge Prolactinomas

Prolactinomas are the largest pituitary tumours (PT) in males, but huge ones (defined arbitrarily as ≥ 8 cm in height) are exceptional. They are usually diagnosed after impotency, headaches and visual troubles. Other manifestations such as memory troubles, unconsciousness, meningitis, epilepsy, psychiatric disorders, and exophthalmos are unusual. We aimed to report two cases diagnosed after epistaxis. Case 1. A man, aged 38, suffering from chronic nasal bleeding and impotency was diagnosed with a huge prolactinoma [90x90x80 mm, PRL=3737 ng/mL (n<15)] invading the nasal cavity. Nasal biopsy confirmed PT with positivity to PRL antibodies. After dopamine agonists intake, PRL and tumour size were significantly reduced and nasal bleeding disappeared. Case 2. A male aged 29 was referred for a huge PT [PRL= 2586 ng/mL, tumour= 130x80x60 mm] diagnosed after epistaxis. Nasal endoscopy showed a bleeding greyish tumour whose immunostaining confirmed PT. Dopamine agonists stopped epistaxis after PT tumour reduction. Conclusion. Both cases suffered from epistaxis secondary to huge prolactinomas invading the nasal cavity. So, chronic nasal bleeding should be kept in mind as a possible sign of PT. Nasal biopsy should be kept in mind for diagnosis confirmation. Nasal invasion by PT can induce suppurative meningitis which is a life threatening condition, fortunately not observed in our cases.

Tentorial dural fistula with giant venous ampulae treated with embolisation and surgery. A case report

Tentorial dural arteriovenous fistulas are rare and complex lesions in deep locations with unusual vascular anatomy and critical surrounding neuroanatomy. A rare case presenting a complex fistula with a giant venous draining ampulae, causing headaches and visual troubles is presented. We describe the case of a 52-year-old woman admitted in our department for headaches and visual troubles. Magnetic resonance imaging and cerebral angiography showed a tentorial dural arteriovenous fistula draining in a giant tentorial venous ampulae and leptomeningeal veins. The patient was embolised via an arterial route with a good clinical and radiological result. However, 4days later she presented a sudden change of her clinical status with coma, left hemiparesis and a right midriasis. The cerebral computed tomography scan showed a huge occipital haemorrhagic mass and a severe cerebral oedema. An emergent surgical procedure was decided realising evacuation of the occipital haematoma and a complete resection of the giant venous ampoule. The neck of the ampulae was sutured and clipped at its dural entrance. Postoperatively a new embolisation was realised because of persistent of a small dural fistulae with occipital leptomeningeal drainage. The patient recovered rapidly with only a residual hemianopsy. Treatment of dural AV malformation represent a serious challenge. Our report describes an unusual case of a tentorial dural complex fistula treated by an endovascular procedure with secondary clinical aggravation that needed emergent surgical therapy. Even in a case for good immediate radiological result after endovascular procedure, dural arteriovenous fistulas with giant venous ampulae and important venous engorgement, need closed follow-up, because of the possibility of aggravation secondary to venous thrombosis and haemorrhage. Treatment and patophysiology of the aggravation mechanism are discussed.

Prise en charge de l’acromégalie évolutive au cours de la grossesse

Pregnancy in acromegalic patients is an infrequent event, due to perturbed gonadotroph function. On the other hand, pregnancy may cause an enlargement of the adenoma or an increase of growth hormone (GH) secretion. We report the case of a 26-year-old woman with a GH-secreting pituitary macroadenoma who was operated by transphenoidal approach. After surgery, she had a persistant acromegaly due to an intrasellar tumour. She was treated by lanreotide. After one year, the patient had married and became pregnant. Lanreotide was stopped when the diagnosis of pregnancy was established. The follow-up of this patient showed a progressive increase of IGF1 level during pregnancy without tumoral syndrome or visual troubles. No metabolic complication was detected. The patient went to term and gave birth to a healthy baby. In postpartum, the IGF1 level was normal, but the brain MRI revealed a 10mm intrasellar tumour. The pituitary adenoma was not significantly enlarged during pregnancy. Therefore, pregnancy doesn’t influence acromegaly in young women well controlled by medical treatment.

Migrainous infarction: association with vascular risk factors in a male subject

Migraine with aura (MA) is associated with an increased risk of ischemic stroke, especially in young women with vascular risk factors (smoke, contraceptive pill). Patent foramen ovale (PFO) has also been associated with MA. We describe a 41-year-old man, in good health, with MA since 16, familiar history of diabetes, heavy smoker (30 cigarettes/day). Frequency (1-2 attacks/year) and clinical features of migraine have been unchanged since the onset. A few days before our examination he suffered a typical migraine attack. In the following hours, however, the headache became more and more throbbing and the aura symptoms (regressed as usual in 30 min) reappeared and persisted, so he went to an Emergency Department. The CT-scan (without contrast) was normal. The following days he had visual disturbances and spatial disorientation. We found a normal neurological examination and fundus oculi. He referred persisting visual troubles. We prescribed MR + angioMR which confirmed a migrainous infarction and ruled out others pathological conditions. Further tests found out dyslipidemia, hyperhomocysteinemia, impaired glucose tolerance. Transcranial Doppler showed right to left shunting. We also prescribed the screening tests for vasculitis (normal). In our opinion this case highlights the relevance of vascular risk factors in MA complications also in male subjects.

47-jähriger Patient mit Sehstörungen, Polydipsie und Polyurie

Bronchuscarcinoma ist the most frequent death cause with tumor patients. At time of diagnosis the stadium is often already advanced, the patient is inoperable. We present a patient (non-smoker) with polydipsia, visual troubles and polyuria. The lab results confirmed diabetes insipidus, but the following x-rays proved multiple intracerebral spots. And also multiple spots in the lungs, the mediastinum, in the liver, the coloumn and the adrenals. Histological diagnosis was non small cell lung cancer (NSCLC).

Hypertension artérielle pulmonaire primitive familiale révélée par l’association bilatérale chémosis, myopisation et exophtalmie

To report a rare case of primary pulmonary hypertension (PPH) revealed by a relapsing chemosis, exophthalmos, and subacute myopia. A 46-year-old woman presented with recurrent bouts of chemosis, exophthalmos, and subacute myopia. Her visual troubles were triggered by physical effort. She had a history of a longstanding high blood pressure that was noncompliant to all conventional hypotensive therapy. Automated refraction under cycloplegia showed recent myopia. At observation she had a bilateral, moderate chemosis. Hertel exophthalmometer measured a symmetric exophthalmos of 23 mm. Intraocular pressure was 20 mmHg OD and 24 mmHg OS. Eye fundus was strictly normal. Laboratory analysis showed no thyroid or inflammatory disease. HIV serology was negative as were antinuclear antibodies. Orbital CT confirmed the axial, symmetric exophthalmos. Echocardiogram brought out the diagnosis of PPH. Genetic studies by PCR localized the BMPR2 mutation that is characteristic of the familial type of PPH. Symptoms decreased under treatment. PPH is a rare disease caused by thickening of the arteriolar walls in the pulmonary bed, which results in a right-heart hypertension with venous stasis. Many authors have reported different posterior segment diseases secondary to PPH. However, anterior segment manifestations are extremely rare. These may be caused by a ciliary body congestion and retrobulbar vasodilatation. The association of chemosis, exophthalmos, and subacute myopia is unusual in PPH. We recommend keeping in mind the PPH as a differential diagnosis in those cases, especially in patients who suffer from high blood pressure.

Asomatognosie et troubles de l'oralité. Une lecture psychanalytique

Objective: The psychoanalytic concept of specular image refers to the complex construction that associates the body image with the language coordinates of the individual, thus making him/her a human subject. The acquisition of this specular image implies the loss of corporeal exchanges between mother and child, i.e., the “neutralization” of those body parts or extensions where these exchanges take place. These conceptions of body image and subjectivity lead to the hypothesis that neurological disturbances of body schema may alter the patients' subjectivity and their relation to the lost “object” insofar as they alter body image. Material: In the present paper, we present two patients aged under 50, with a unique first ever stroke due to ischemia in the right middle cerebral artery territory and asomatognosia. Methods: On one hand, Bisiach's protocol was used to assess hemiplegia, sensory troubles, visual troubles, hemineglect and anosognosia, and adapted to assess asomatognosia. On the other hand, subjective data were gathered during a semistructured interview and a self-portrait test. Results: This showed that asomatognosia was accompanied by a destructuration of body image and aberrant oral manifestations involving the paralyzed hand. Discussion – Conclusion: The psychological positive phenomena accompanying asomatognosia might correspond to the intrusion of the lost object in the patients’ psychic reality, due to the alteration of body schema and body image.

Phase I Study of Retelliptine Dihydrochloride (SR 95325 B) Using a Single Two-Hour Intravenous Infusion Schedule

Retelliptine dihydrochloride (SR 95325 B, NSC D-626717-W) is an ellipticine derivative having a very high level of antitumor activity in resistant murine solid tumor models. We studied in a Phase I trial escalating doses of retelliptine using a single 2-hour IV infusion schedule. Data from other Phase I studies allowed a starting dose of 80 mg/m2 and a rapid dose escalation. Included were 15 patients (M/F = 13/2) with a median age of 55 (range: 17-72). There were 22 courses delivered at the following dose levels: 80, 180, 700, 900, 1,200, and 1,500 mg/m2. Primary tumor types were kidney (6 patients), colon (3 patients), pancreas (2 patients), and others (4 patients). Mild dose-related visual troubles (blurring, accommodation troubles, oculomotor paresis) occurred in 9/11 patients starting from 700 mg/m2. Asymptomatic EKG anomalies, including significant prolongation of PR and QRS intervals occurred at 1500 mg/m2 (in 3/3 patients) marking the maximum tolerated dose. Both visual and EKG anomalies were spontaneously reversible few minutes to few hours after the end of infusion. Other possible drug-related toxicity occurred sporadically such as somnolence, bronchospasm, dry mouth, and vomiting (2 patients each). There were no significant laboratory anomalies. Neither drug-related deaths nor objective complete or partial responses were observed. The recommended dose for Phase II trial using the 2-hour intravenous infusion schedule is 1,200 mg/m2.

A randomised trial of MACC chemotherapy with or without lonidamine in advanced non-small cell lung cancer

Combination chemotherapy with anti-proliferative agents is the usual treatment for patients with advanced non-small cell lung cancer (NSCLC), good performance status and no major clinical contraindications. Lonidamine (LND), a new drug with an innovative mechanism of action, might potentiate anti-cancer activity of conventional cytotoxic drugs, with no increase of specific toxicity. Following a pilot study of feasibility, we now report the results of a randomised trial evaluating MACC chemotherapy, as originally described, versus the same regimen + LND. 151 patients with advanced NSCLC were assigned at random to the two treatment arms. LND 150 mg was given orally three times daily. Treatment was continued until progression of disease, unacceptable toxicity or refusal by the patient (median number of cycles of MACC, three for both arms; median duration of LND administration, 8 weeks in the arm concerned). Actual dose intensities (DI) of MACC and LND were, respectively, 100 and 83% of those intended (median values). There was a negative correlation between duration of chemotherapy and the DI of MACC reached in each patient, but no correlation between the duration of treatment with LND and its DI. DIs of LND and MACC were not correlated with each other. In all, 15 objective responses (one complete and four partial responses in the MACC group, 10 partial responses in patients on MACC + LND) were observed. Median progression-free survivals were 20 weeks (confidence interval, CI 14–22) for the group on LND and 17 weeks (CI 12–17) for the control group (non-significant difference). Median overall survivals were, respectively, 30 weeks (CI 23–40) and 27 weeks (CI 22–34), P = non-rmsignificant. Toxicity was as expected by the use of MACC, and similar in both arms, except for more severe anaemia and gastric toxicity in the group on MACC + LND. Other uncommon side-effects, seen only in this latter group, were mild to moderate and reversible and included myalgia, asthenia, testicle pain, headache, visual troubles, incubi and dizziness. Subjective tolerance to the treatment, and perception of physical and psychological well-being were rated similarly by patients of both groups. MACC plus LND is a moderately active regimen in advanced NSCLC, with a foreseeable and reversible toxicity of low-medium grade. Potential enhancements of anti-tumour efficacy of chemotherapy, and possible host survival benefits derived from the use of LND are not substantiated by the results of this trial.

Le Doppler encéphalique en ophtalmologie quotidienne

Encephalic circulation has been studied by Doppler ultrasonography in a number of persons who complained of discreet visual troubles, transient alterations of the uni- or bilateral visual acuity, apparently isolated alterations of the visual fields, or organic or functional alterations of the retina. In a significant number of cases, the examination has shown cerebral vascular alterations--sometimes very severe--which warrant very careful medical supervision and the institution of a sustained vascular treatment.

Serious Visual Troubles Caused by Unusual Circulatory Disturbances in the Region of the Optic Nerve

Serious Visual Troubles Caused by Unusual Circulatory Disturbances in the Region of the Optic Nerve