Neuro-ophthalmologic Symptoms Research Articles

Sudden bilateral blindness in Wernicke's encephalopathy: Case report and review of the literature

We report on a patient suffering from bilateral sudden blindness as initial symptom of Wernicke's encephalopathy (WE). A 37-year-old male alcoholic was admitted to a psychiatric clinic because of excessive alcohol consumption (3.4‰). 24 h later he developed acute bilateral blindness with no light perception, downbeat nystagmus, bilateral ocular abduction deficits, cerebellar ataxia as well as a slight psychomotor slowing and mild disorientation. MRI including diffusion-weighted imaging and MR-angiography 3 h after symptom onset did not reveal findings suggestive for ischemic stroke. Immediate iv-application of thiamine led to a nearly complete remission of the neuroophthalmologic symptoms within 12 h. Although we critically discuss other potential etiologies, we conclude that the complex clinical picture with initial sudden blindness is an unusual presentation of WE.

Diagnosis and treatment of dural carotid–cavernous fistulas: a consecutive series of 27 patients

Objectives: To report clinical characteristics, angiographical findings and results of endovascular treatment of patients presenting with dural carotid–cavernous fistulas (DCCFs). Method: Retrospective analysis of 27 consecutive patients with DCCF referred...

Neuro-Ophthalmologic Presentations of Hemicrania Continua

We describe a series of patients with hemicrania continua, a rare indomethacin-responsive primary headache syndrome, who presented for evaluation with neuro-ophthalmologic signs and symptoms. Observational case series. Nine patients between the ages of 29 and 58 years were seen with various neuro-ophthalmologic findings and a unilateral continuous headache. A detailed history was taken from each patient, followed by a focused ophthalmologic and neurologic examination. The risks and benefits of treatment with indomethacin were discussed. Patients were instructed to call after several days of treatment to report any change in their headache and neuro-ophthalmologic symptoms, in addition to any adverse side effects. All patients responded favorably to indomethacin, with rapid near-complete or complete resolution of headache and autonomic symptoms, and treatment was initiated as early as possible. Because of its absolute response to indomethacin, recognizing the neuro-ophthalmologic symptoms of hemicrania continua as a component of the headache syndrome is critical for prompt initiation of treatment.

Ophthalmodynamometry for diagnosis of internal carotid artery dissection

Dear Editor, The central retinal artery and vein are the only structures in the body that are visible and non-invasively accessible and that may offer information about the status of the cerebro-afferent arterial vessels, the arterial and venous blood pressure in the eye, the tissue pressure in the orbit, the cerebrospinal fluid pressure and the systemic blood pressure, as suggested by recent studies using a modified technique of ophthalmodynamometry [1–5]. The purpose of the present report is to describe a patient who presented with neuro-ophthalmological symptoms and for whom modified ophthalmodynamometry was helpful in elucidating the diagnosis of an obstruction of the internal carotid artery. A 56-year-old man in good general health came for a routine ophthalmological examination. Visual acuity measured 25/20 in both eyes, and intraocular pressure was 15 mmHg OU. Slit lamp biomicroscopy of the anterior segment was unremarkable. Testing the pupillary reactions revealed a Horner syndrome on the right side. There was no relative afferent pupillary defect. Ophthalmoscopy showed a normal ocular fundus including an unremarkable visibility of the retinal nerve fibre layer. The performance of Goldmann contact lens-associated ophthalmodynamometry for assessment of the central retinal artery collapse pressure [1–4] revealed significantly lower readings of that pressure in the right eye than in the left eye [mean ± SD of 10 repeated measurements for each eye: 51.9±17.5 arbitrary units (right eye) vs 92.6±6.7 arbitrary units (left eye); P=0.046]. This pressure difference between the right and left eyes was significantly (P=0.036) higher than that in a healthy population group with a mean value of 15.0±13.4 arbitrary units. The central retinal vein showed spontaneous pulsations in both eyes. A more detailed history with regard to these findings disclosed that the patient had suffered a dissection of the internal carotid artery 2 years ago with typicalipsilateral headache and miosis in the absence of neurological deficits. The dissection of the internal carotid artery was located at the entry of the artery into the carotid canal at the base of the skull. While direct ultrasound studies failed to demonstrate the lesion far distal from the extracranial carotid bifurcation at the time the patient presented for the ophthalmological examination, magnetic resonance imaging still showed an J. B. Jonas (*) Department of Ophthalmology, Faculty of Clinical Medicine Mannheim, University of Heidelberg, 68167 Mannheim, Germany e-mail: jost.jonas@augen.ma.uniheidelberg.de Tel.: +49-621-3832242 Fax: +49-621-3833803

Apoplexie hypophysaire

Pituitary apoplexy is a life threatening condition caused by sudden hemorrhage or infarction of the pituitary gland. Most patients present with headaches and neuro-ophthalmologic symptoms and signs, often associated with altered mental status.

Neuro‐ophthalmological findings in sarcoidosis

This review emphasizes the importance of neuro-ophthalmological signs and symptoms in sarcoidosis. The presence of ophthalmological and neuro-ophthalmological findings may lead to diagnosis of the disease and the initiation of adequate treatment. Patients who had been diagnosed with neurosarcoidosis during the period 1990 - 2001 were identified from the departmental diagnostic index. The history, clinical, laboratory and imaging data of patients were analysed. Fifteen patients were identified, four men and 11 women, with a mean age of 44.1 years (range 26-65 years). In six of the 15 (40%), neurological deficits were the initial symptoms. Nine (60%) had known sarcoidosis at the time of presentation. Ten patients (66%) had ophthalmological/neuro-ophthalmological symptoms and signs. Neuro-ophthalmological symptoms may develop early in neurosarcoidosis. If neuro-ophthalmological symptoms arise in patients with established biopsy-proven sarcoidosis, the diagnosis is usually easy to make. However, a number of patients with neurosarcoidosis may present with neuro-ophthalmic symptoms before systemic involvement becomes obvious. In this situation the diagnosis is challenging, and the major goal is to establish the presence of systemic sarcoidosis.

Endocrine and metabolic deficiency

Endocrine and metabolic disorders that cause neuro-ophthalmologic manifestations are frequently encountered by ophthalmologists in clinical practice. This review focuses on three of the most common entities for which neuro-ophthalmologic signs and symptoms are a prominent feature. These entities include pituitary disorders (adenoma, apoplexy, and hypophysitis), thyroid-associated ophthalmopathy, and neuro-ophthalmologic complications of diabetes mellitus, such as ischemic ocular motor mononeuropathies and diabetic papillopathy.

Imagerie des urgences en neuro-ophtalmologie

MRI often is mandatory in the diagnostic work-up of visual loss, visual field alterations and oculomotor problems. It is performed emergently in patients with painful diplopia associated to mydriasis, to exclude aneurysm, or in patients with painful Horner syndrome to exclude dissection of the internal carotid artery. CT scan in emergency remains useful in case of acute lateral hemianopsia or acute post traumatic visual loss. Progressive neuro-ophthalmological symptoms may require imaging examination in a short delay to define the therapeutic strategy: monocular transient blindness (dissection or carotid stenosis), progressive visual loss (optic nerve compression), bitemporal hemianopsia (optic chiasm lesion), painful visual loss (optic neuritis). A very precise clinical indication is helpful for the choice of imaging protocol and to improve its diagnosis value.

Neuro-ophthalmology and paraneoplastic syndromes.

To describe the neuro-ophthalmological manifestations of paraneoplastic syndromes and their immunological associations. Neuro-ophthalmological signs and symptoms are usually present in paraneoplastic syndromes of the central nervous system. Unlike opsoclonus, less characteristic eye movement abnormalities are difficult to recognize as presenting symptoms of paraneoplastic syndromes. In this setting, the detection of several antibodies, including anti-Hu, Yo, Ma2, Ri, Tr, CV2/CRMP5 or voltage-gated calcium channel antibodies may help to establish that the neuro-ophthalmological disorder is paraneoplastic. Among the recently characterized antibodies, those against the Ma proteins often associate with brainstem encephalitis and vertical gaze paralysis. A small subset of patients with opsoclonus and ataxia harbor anti-Ri antibodies. In other patients, there is preliminary evidence that the autoantigens of opsoclonus reside in the postsynaptic density, but no dominant antibody marker has been identified. Uveitis and optic neuritis are rare accompaniments of paraneoplastic encephalomyelitis; some of these patients harbor anti-CV2/CRMP5 in association with other antibodies. Studies on paraneoplastic retinopathy indicate that immunity to retinal proteins other than recoverin can result in a similar syndrome to that associated with recoverin antibodies, and that melanoma-associated retinopathy may associate with several retinal antibodies. There is increasing recognition of an extensive variety of neuro-ophthalmological abnormalities as manifestations of paraneoplastic syndromes and of several antineuronal antibodies as clinical markers of these disorders. Basic immunological studies support the pathogenic role of some of these antibodies, and are elucidating the pathogenic mechanisms that underlie these and other antibody-associated paraneoplastic syndromes.

Medical management of hypopituitarism in patients with pituitary adenomas.

Hypopituitarism is a disease complex with variable clinical manifestations. Recent studies have improved our understanding of its pathophysiology, particularly in patients with pituitary adenomas. In that setting, hypopituitarism was previously considered a permanent and irreversible process, requiring life-long hormone replacement therapy. While this could be true in some instances, recent data demonstrated recovery of pituitary function in a large number of patients with hypopituitarism following surgical decompression. Mechanical compression of portal vessels and the pituitary stalk, by the expanding adenoma was postulated to be the predominant mechanism causing hypopituitarism in this setting. Since portal vessels also provide blood supply to the anterior lobe, ischemic necrosis of portions of the pituitary can occur as a result of increased and prolonged compression by the expanding adenoma. Recent data demonstrated increases in intrasellar pressure (ISP) in patients with pituitary macroadenomas, particularly those with hypopituitarism. The data showed that ISP measurements correlated positively with the serum prolactin levels but not with tumor sizes. It is postulated that increased ISP has predominant role in the pathogenesis of hypopituitarism in patients with pituitary adenomas while. The increase in ISP results in decreased blood flow through the portal vessels and the pituitary stalk. The latter will result in diminished delivery of hypothalamic hormones to the anterior pituitary and may also cause ischemia and/or necrosis in some portions of the normal gland. Recovery of pituitary function can thus be anticipated after surgical decompression, in patients who have viable pituitary cells. Understanding the pathophysiology of hypopituitarism and recognizing the probability for recovery of function should be emphasized in the management of patients with this disease. An important aspect of the management is patients' education about their disease, including the use of medic alert identification. The managing physician should appreciate the variable clinical manifestations of the disease and the possible occurrence of other associated neuroendocrine, neurological and neuro-ophthalmologic signs and symptoms. Treatment of hypopituitarism should not be rigid but instead, always individualized. Management should take into consideration the patients' age, sex, education, original disease process and clinical history.

Neurovisual manifestations of herpesviruses.

Herpes simplex virus and varicella-zoster virus cause dermatitis, blepharoconjunctivitis, keratitis, uveitis, glaucoma, and retinitis. These conditions are encountered frequently by the ophthalmologist in clinical practice. It should not be overlooked that herpesviruses can also produce an extraordinary variety of neuroophthalmological signs and symptoms. What follows is an overview of the most common and important neuroophthalmological manifestations of herpesvirus infection.

Giant pituitary adenomas: clinical characteristics and surgical results

Although most pituitary neoplasms are benign, some grow rapidly, spreading to extrasellar tissues. Definition of these 'giant' pituitary adenomas (PAs) is not clear. In order to clarify this question, we studied all pituitary macroadenomas operated in our hospital during the last 20 years, differentiating those with diameter S 3 cm ( n = 43) and attempting to identify their typical features and prognosis. The reason for consultation was local in 58.3% of giant PAs, hormonal in 22.9% and tumour recurrence in 16.7%. Surgery was performed via a trans-sphenoidal approach in 92.5% of cases and succeeded in completely removing the tumour in only 27% of cases. In conclusion, we found that giant PAs are not a special type but the extreme case in the gradient of invasiveness of tumours of adenohypophyseal origin. They are characterized by a higher frequency of neuro-ophthalmological symptoms and hormonal deficits, and poorer response to surgical treatment.

神経内科医によるめまいの診断と治療

includes dizziness, vertigo, ataxia and psychogenic unsteadiness and is the second most common symptom next to pain. The diagnosis and treatment of dizziness is discussed, together with a discussion to differentiate life-threatening dizziness from non-life-threatening dizziness. A complaint of dizziness may be more serious than a complaint of vertigo, and danger signs include markedly dilated pupils. Dizziness with abnormal vital signs and with neurological and/or neuro-ophthalmological symptoms may indicate that the patient is in a very serious condition. Neurological therapy for dizziness includes tPA, Heparin, anticoagulants for CVA related dizziness, anti-Parkinsonism therapy for Parkinsonism related dizziness. In this report therapy for migraine without Ergotamine alkaloid for dizziness of brainstem migraine is discussed, together with therapy for cervical dizziness.

Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis. French Vasculitis Study Group.

To describe the clinical, biologic, and histologic features of temporal artery biopsy (TAB)-localized systemic necrotizing vasculitides (SNV), and to assess their frequency among elderly patients undergoing TAB for suspected giant cell (temporal) arteritis (GCA). The frequency of a TAB localization of SNV was prospectively assessed in a multicenter study of elderly patients undergoing TAB for suspected GCA. All patients with SNV fulfilling the American College of Rheumatology criteria for a specific vasculitic syndrome and with evidence of vasculitis on TAB were included in a retrospective, descriptive study. SNV was diagnosed based on the TAB in 1.4% of the patients with suspected GCA and in 4.5% of the positive (inflamed) TAB specimens. We retrospectively selected 27 patients (18 female, 9 male; mean +/- SD age 62+/-15 years, range 22-79 years) with SNV and TAB-localized vasculitis. Only 2 of these patients were known to have SNV before TAB localization. Twenty-two patients (81%) had cephalic symptoms, including jaw claudication in 33%, clinically abnormal temporal arteries in 33%, and neuro-ophthalmologic symptoms in 11%. All patients had systemic symptoms suggestive of SNV and histologically proven NV in the TAB specimens (70%) or elsewhere in other biopsy sites (74%). Abnormal biologic results suggestive of SNV were present in 17 patients (63%). For 4 patients, the TAB-documented involvement led to initial misdiagnoses of GCA, and systemic manifestations that developed under steroid therapy revealed the correct diagnosis. The final diagnoses of the patients were polyarteritis nodosa (PAN) (n = 11), Churg-Strauss syndrome (n = 6), micropoly-angiitis (n = 3), Wegener's granulomatosis (n = 3), hepatitis B virus-related PAN (n = 2), hepatitis C virus-related cryoglobulinemic vasculitis (n = 1), and rheumatoid vasculitis (n = 1). TAB-localized SNV presents a major diagnostic dilemma because it can mimic GCA. Careful analysis of clinical, biologic, and histologic data should lead to the correct diagnosis and help guide the clinician's choice of appropriate therapy.

Recent advances in the diagnosis and treatment of central nervous system germ-cell tumours.

Primary germ-cell tumors of the central nervous system are rare neoplasms that are seen primarily in the pediatric age group. Because of their frequent location in the pituitary and suprasellar regions, they present with typical neuro-ophthalmologic and neuroendocrine symptoms. Sophisticated imaging and surgical biopsy allow precise anatomic definition, but only allow an approximate guess of the tumor histopathology. Tumor markers in the serum and cerebrospinal fluid are extremely helpful in the diagnosis and monitoring of response to treatment when they are detectable. Because of the deleterious effects of irradiation on neurocognitive and neuroendocrine functioning, we have looked at strategies that either reduce or eliminate radiation exposure. Large, randomized, prospective, cooperative trials in the future will be the only way to identify subgroups of patients that may benefit from particular treatment strategies.

Neuro-Ophthalmology of Arteriovenous Malformations

Intracranial arteriovenous malformations may result in a number of neuro-ophthalmologic signs and symptoms. In some patients, the neuro-ophthalmic findings are the presenting or only manifestations. A multi-disciplinary team approach including neurologists, ophthalmologists, neurosurgeons, interventional radiologists, and radiation therapists is ideal.

Elevation of Serum Tumor Marker CA 15.3 Levels as the First Manifestation of Metastatic Breast Cancer to the Cavernous Sinus

We report rising tumor marker levels of CA 15.3 as the presenting manifestation of metastatic breast cancer to the cavernous sinus and orbit. A 39-year-old woman with a history of breast cancer developed increasing levels of tumor marker CA15.3. Ten months later, she developed vision loss in the right eye, diplopia, and right-sided ptosis. A magnetic resonance scan of the head showed a mass involving the right cavernous sinus and superior orbital fissure. Biopsy of the lesion showed metastatic breast cancer. She was treated with surgery and radiotherapy and did well. Ophthalmologists should be aware of the significance of increasing levels of tumor markers, such as CA 15.3, in patients with a history of breast cancer and new neuroophthalmologic signs or symptoms.

Clinical and morphological features of “inactive” pituitary adenomas: a retrospective analysis of 95 cases

Ninety-five case histories of patients with “inactive ” pituitary adenoma are analyzed. These patients were operated on from 1977 to 1983, and the diagnosis was verified in them by histological, electron-microscopic, and immunohistochemical studies of the operation material. Clinical manifestations of the disease included a) neuroophthalmological symptoms; b) sexual disorders in men and women; c) pituitary insufficiency (hypogonadism, hypothyrosis, or hypocorticoidism); d) neurological and psychopathological symptoms. By the moment of the first manifestation of the symptoms the majority of patients were middle-aged. In older patients the disease starts with ocular and/or neurological disorders, in women under 50 and men under 55 years of age as a rule with sex disorders. Moderate hyperprolactinemia was detected in half of the patients, which in 30% of cases was associated with oligo-amenorrhea-galactorrhea often leading to hyperdiagnosis of prolactotrophic adenoma. In general, all types of inactive pituitary adenoma are chromophobic by their tinctorial properties, except oncocytoma, which is poorly eosinophilic in half the cases; electron microscopy showed zero-cell adenoma to be the most incident (40%) in the examined patient population.

Pentoxifylline suppression of TNF-α mediated axonal degeneration in the rabbit optic nerve

In AIDS patientsl axonal degeneration in the optic nerve occurs as a histopathological manifestation of the optic neuropathy. Direct infection of neurons by HIV is unlikely, and the axonal injury may be an indirect effect mediated by cytotoxic factors such as tumor necrosis factor-alpha (TNF-α) which we have previously demonstrated to cause axonal degeneration in the rabbit optic nerve. To test the suppressive effects of pentoxifylline in preventing TNF-a-mediated axonal degenerationl we applied pentoxifylline to an established rabbit model that demonstrates an AIDS-like optic neuropathy using intravitreal TNF-α injections. Degenerated axonal profiles were numerous in control rabbit optic nerve (mean 1879) and reduced in rabbits receiving the medium dose of pentoxifylline (300 mg PO BIOI mean 4391 p < 0.001) and the highest dose of pentoxifylline (600 mg PO BIOI mean 120, P< 0.007). High dose pentoxifylline reduced TNF-α-induced axonal losses to less than 10% that seen without pentoxifylline pretreatment. Lower doses of pentoxifylline had a lesser but significant protective effect. Our results suggest that TNF-amediated axonal degeneration can be suppressed by high doses of pentoxifylline. Pentoxifylline may therefore be useful in AIDS patients demonstrating neurological or neuro-ophthalmological symptoms. [Neural Res 1997; 19: 551-554]

Neuro-ophthalmology of multiple sclerosis.

Multiple sclerosis remains a pathophysiologic enigma. The epidemiology has recently been reexamined and appears to lend credence to a greater genetic basis than previously believed. Neuro-ophthalmologic signs and symptoms in multiple sclerosis are extremely common and often present to the eye care practitioner. Although diagnostic methodology is progressing, treatment of multiple sclerosis is currently undergoing a revolution.