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This article presents neuroophthalmological conditions that may typically cause ocular pain. Particular attention is paid to the description of two cases of headache and facial pain where after a long diagnostic history the correct reason for the pain was only discovered via subtle diagnostic findings. This is exceptionally important as primary headaches are a main reason for ocular pain on the one hand but on the other hand they do not show unambiguous findings, therefore a definite exclusion of primary sources causing the pain is required before making this diagnosis.

We present an overview of the treatment and clinical outcome of 15 orbital lymphomas, carried out in our center from 1993 to 2002. The surgical approach was determined by the location, and type of the lesion. Lateral orbitotomy was performed in five laterally located lesions, a transconjunctival approach in five medial, basal, extra-, intraconal lesions. Pterional intradural and extradural and intradural approaches (three) were used in case of intracranial involvement or location in the apex and optic canal. Two lesions of the lid or extraconal space were operated via eyebrow incision. Ten patients were diagnosed as extranodal marginal zone lymphoma. Diffuse large B-cell lymphoma (DLCL) was encountered in two patients, follicular lymphoma (FCL) in two patients, and diffuse lymphoplasmacytic/lymphoplasmacytoid lymphoma in one patient. All patients except one failed to reveal systemic disease and had a localized orbital lymphoma. Twelve of the patients responded to radiation therapy with usually 40 Gy. Cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy was applied in two patients, once in combination with radiotherapy. Local recurrence was seen in one patient with FCL. Systemic manifestation occurred in one patient with DLCL, undergoing ifosfamide, carboplatin, etoposide chemotherapy with complete remission. The course of orbital non-Hodgkin's lymphoma is variable and requires a multidisciplinary treatment. Therapeutic options include surgical biopsy, radiation therapy, and chemotherapy.

Congenital and acquired nystagmus, particularly pendular and jerk nystagmus, see-saw nystagmus and spasmus nutans, may be the presenting sign of a suprasellar mass lesion.(1) The large variety of different suprasellar mass lesions requiring quite different therapeutic measures necessitates exact histological diagnosis for optimal therapeutic strategy planning.(2) Stereotactic tumor biopsy has become a well-established diagnostic approach, combining minimal surgical trauma with a high degree of diagnostic safety. Particularly in the two most frequent suprasellar mass lesions - craniopharyngiomas and pilocytic astrocytomas - accurately planned stereotactic drainage of tumor cysts combined with radiotherapy and/or stereotactic radiosurgery allows successful decompression and tumor control as well as maximum preservation of visual and endocrinological functions when compared with conventional surgical procedures.(2-11)

A nonrandomized, prospective, interdisciplinary pilot study of 102 patients with noncompressive optic disc swelling with visual loss (ODSWVL) was performed in order to investigate etiologic and pathogenetic mechanisms. Forty-six patients suffered from underlying inflammatory disease. Seventeen patients suffered from highly probable cardiogenic embolization, 16 patients from multiple vascular risk factors. The remaining patients of the noninflammatory disease group suffered from leukemia, previously unknown or severely decompensated diabetes mellitus, acute arterial hypertension, different kinds of coagulopathies and others. Ninety-six of the 102 patients required medical treatment according to general medical standards. Inhomogeneity of the underlying disease processes explains the ineffectiveness of different monotherapies in previous studies. Interdisciplinary search for the underlying causes allows causative treatment. ODSWVL and anterior ischemic optic neuropathy in particular seem to be a common final pathway of various pathogenetic mechanisms due to different etiologies rather than a disease entity by itself.

We describe a mutation in the FGFR2 gene in affected members of a large family with inherited autosomal dominant craniosynostosis. The mutation is a G1044A transition at codon 344 of exon B of the gene and results in abnormal splicing of the FGFR2 transcript. The phenotypic effect of the mutation varies greatly. It ranges from minor anomalies such as slight hypertelorism and maxillary hypoplasia to severe manifestations such as brachycephaly and dolichocephaly. The severe cases required surgery because of increased intracranial pressure. The patients cannot be assigned clinically to one of the known craniosynostotic syndromes with mutations in FGFR2, e.g., Crouzon, Pfeiffer, or Jackson-Weiss. This study demonstrates that FGFR2 mutations can result in a spectrum of craniofacial abnormalities even within one family. The known eponymic syndromes of Crouzon, Pfeiffer, or Jackson-Weiss only describe phenotypic extremes of this spectrum. Therefore, the clinical classification should be abandoned and replaced by a molecular one such as "FGFR-associated craniosynostosis syndromes."

The merosin M-chain (or laminin-alpha 2) is one of three subunits of laminin-2 which is highly expressed in striated muscle and peripheral nerve. Complete lack of laminin-alpha 2 expression in skeletal muscle is the hallmark of one form of congenital muscular dystrophy which is characterized by dysmyelination of the central nervous system (CNS), links to chromosome 6q2 and is common among Caucasians. Laminin-alpha 2 expression was also found to be significantly reduced in Fukuyama congenital muscular dystrophy which links to chromosome 9q3. We report consistently preserved laminin-2 expression, including laminin-alpha 2, as detected by immunofluorescence in skeletal muscle from five patients with Walker-Warburg syndrome which is characterized by congenital muscular dystrophy and, in addition, type II lissencephaly or pachygyria, defective CNS myelination, and ocular dysgenesis. These findings show that in spite of partial phenotypic overlap between Fukuyama CMD and Walker-Warburg syndrome the two disorders are nosologically separate disease entities. They also exclude that Walker-Warburg syndrome is allelic to the common form of congenital muscular dystrophy with laminin-alpha 2 deficiency.

Optic neuropathy, also described as "autoimmune optic neuropathy", cna be treated successfully. It can be isolated, an early sign of or associated with systemic inflammatory disease and vasculitis. Recognition of this entity appears important since the natural course of the disease tends to end up with severe visual loss and optic atrophy. Early systemic high-dose steroid treatment and immunosuppression usually restore and maintain visual function. The clinical spectrum of this disease entity is described on the basis of the literature and our own cases, some of which have been followed up for several years. The differential diagnostic and therapeutic aspects are discussed. Association of this ill-known optic neuropathy with various inflammatory diseases is described; a rich reference list indicates its existence.

Of a total of 80 operated intraorbital lesions, 2 were located in the posterior intraconal space, medial and inferior to the optic nerve. Because they were unfavorably located for standard surgical approaches, we operated via a contralateral pterional transsphenoidal-transethmoidal route. This technique provided excellent exposure and results in these two cases of intraorbital cavernous malformations. A brief description of the approach is presented.

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a self-limited ocular disease with a favorable visual outcome. Regarding its pathogenesis, there is increasing evidence of a vascular disorder of the precapillary choroidal arterioles that causes ischemic edema of the overlying retinal pigment epithelium. Systemic and neurological abnormalities associated with APMPPE have been documented. The development of severe cerebral vasculitis simultaneously or after a period of months or even years has been described in only a few cases. We present two new well-documented cases that developed several months after manifestation of the characteristic findings of APMPPE. A thorough medical and neurological workup is recommended, as is careful information of the patients and their families about the signs and symptoms of potential associated cerebral complications so as to facilitate early and adequate treatment.

The etiology of the endocrine orbitopathy is still unknown and thereby their treatment remains symptomatic. The surgical decompression is achieved in resecting lateral wall, roof and partly bottom of the orbit. We treated 23 eyes in 14 patients and in only one patient the decompression obtained with this method was insufficient. This patient underwent additionally transantral transethmoidal decompression. One patient had a complete loss of vision after surgery probably due to direct lesion of the optic nerve. The decompression of the orbit is indicated in fast progressive endocrine orbitopathies with impairment of the visual function.

The use of endoscopical methods augments the macroscopical diagnosis in autopsies. Cavities and sinuses which are hardly accessible may be inspected and photographically documented as well without damage of the anatomical context. In terms of forensic autopsies the endoscopical technique is mostly recommended for the inspection of the fundus of the eye, the external auditory meatus up to the tympanic membrane, the naso- and laryngopharynx. Pathological and traumatical changes can be valued at once in situ. Forensic evidence of postmortal endoscopical findings is exemplified.

High resolution multiplanar images of the optic canal can be obtained using computer transformations of 1.5 mm axial transverse sections through the orbit. Parasagittal sections through the optic canal along the course of the optic nerve or sections perpendicular to the canal are easily and reproducibly obtained. This approach is a much more efficacious means of examining the optic canal both in terms of information yield and reduction in radiation dose.

Painful dysesthesia in association with ocular motor palsies characterized intraneural carcinomatous invasion in a 48-year-old man. Transcranial biopsy of the cavernous sinus stopped the pain; the specimen confirmed the diagnosis and provided the rationale for radiation therapy. One year after treatment, the patient was healthy and pain-free, with improved ocular motor function. The source of the carcinoma remains unknown.

Computed tomography (CT) is a simple and non-invasive method of demonstrating infarctions of the visual cortex. Seventy-eight CT-proven infarctions were correlated with the visual field defects. This correlation between cortical infarctions and visual field defects is difficult to achieve by angiography because of the variations in the vascular supply. CT, on the other hand, provides a far better correlation, showing the functional-anatomical structure of the visual cortex by projection in three planes (transverse, sagittal, coronar). CT, however, can not replace angiography in cases where arteriovenous malformations are suspected.

Thirteen patients with varying degrees of Graves' ophthalmopathy were examined using high-resolution infrared oculography to determine peak velocities for horizontal eye movements between 3 degrees and 30 degrees. As severity of the orbital disease increased, peak velocities became substantially lower. Vertical-muscle surgery failed to have any effect on peak velocity of horizontal eye movements. In contrast, orbital decompression caused notable improvement in peak velocity of eye movements. Eye-movement recordings, which provide a measure of extraocular muscle function rather than structure, may provide a safe, sensitive, and accurate method for classifying and following up patients with Graves' ophthalmopathy.

Tumors of the lids, the lacrimal gland and the orbit can be early manifestations of sarcoidosis. A timely diagnosis based on biopsy of periocular tissues and cinico-radiographic evaluation at an early stage of the disease may prevent irreversible lesions of the parenchymatous organs. The clinical and histological signs in two patients, one with palpebral and one with parabulbar sarcoidosis, are described and the differential diagnosis and therapeutic aspects are discussed.

CT scans of 25 clinically typical cases of Graves' disease and 15 cases of orbital pseudotumor (idiopathic orbital inflammation) are analyzed and differential diagnostic criteria evaluated. The CT-findings overlap and are best viewed as a continuous spectrum at both ends of which characteristic patterns can be identified. Massive swelling of ocular muscles, involving of several muscles, usually without density changes of the orbital fat, are diagnostic for Graves' disease. More or less circumscribed masses of soft tissue density surrounding the globe, eye muscles or the optic nerve, severe diffuse increase of density in the retrobulbar space masking orbital structures and calcifications indicate orbital pseudotumor. Localization of the swelling within a muscle, "scleral thickening", enhanced contrast, moderate density changes of the orbital fat, and involvement of the lacrimal gland are of minor differential diagnostic value. CT pictures of clinically typical and histologically proven cases of Graves' disease indicate heterogeneity of this group, probably reflecting different immunologic features. CT shows the extent and degree of the pathologic changes and indicates the optimal approach for tissue biopsy. Evaluation of both diseases by CT requires inclusion of coronal sections.

CT-scans of 29 histologically proven cavernous hemangiomas were evaluated with respect to their location, shape, delineation from surrounding tissue, contrast-enhancement, and secondary changes of the bony orbit. Whenever a round or oval tumor, located in the outer upper muscle cone, sharply delineated from surrounding tissue, unattached to optic nerve and ocular muscles, spares a small triangular space in the orbital apex, it is in all probability a cavernous hemangioma. Evaluation of the tumors shape and its separation from surrounding tissues requires imaging in multiple sections in two planes oriented, if possible, at right angles. -- Changes in position of the optic nerve and eye muscles in different directions of gaze demonstrated by CT rule out significant tumor-attachments. The portion of the intraconal space least affected by optic nerve shifts and muscle contractions during eye movements, as demonstrated by CT, is the upper outer quadrant, the site preferred by the mobile tumor. -- Tumors which cannot be differentiated from cavernous hemangiomas by CT-criteria are rare usually benign. Reports of rare examples of well delineated or encapsulated malignant intraconal lesions indicate the possibility -- however remote -- of mistaking a malignant tumor for a cavernous hemangioma by CT.

The computed tomographic (CT) appearance of the optic nerve was studied in various positions of gaze. In addition, the image quality of CT scans in different axial planes was analyzed. Based on these studies, an optimal examination technique for the clinical CT evaluation of the optic nerve is suggested. A negative angulation of -20 degrees to the orbitomeatal base line with the eye in upgaze position appears to be optimal. With this technique, the optic nerves are stretched and course parallel to the plane of section. They are therefore included in a minimum number of sections.