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In 24 patients with irreversible lagophthalmos, gold weights ranging from 0.8 to 1.7 g were implanted in the upper lids, under local anaesthesia. With a follow-up period ranging from 2 1/2 to 4 years (average, 3 years), the results of the implantation were gratifying in 23 patients. While corneal irritation and epiphora was reduced and the loaded upper eyelid allowed patients to blink voluntarily.

Acute stroke multimodal CT imaging (MMCT: non-enhanced CT, CT angiography, and CT perfusion (CTP)) may show normal results despite persistent clinical stroke. We prospectively evaluated the sensitivity/specificity of MMCT infarct detection and the clinical outcome in patients with normal MMCT findings. From April 2007 to April 2008, all patients with acute hemispheric stroke within 6 h of symptom onset who underwent complete MMCT and MRI follow-up imaging were included. MMCT analysis included occlusion type, early infarct hypodensities (EIH), mean transit time (MTT), and cerebral blood volume (CBV) maps according to Alberta Stroke Program Early CT Score (ASPECTS). Clinical assessment included symptom onset to CT scan (≤3 h/>3 h), the National Institute of Health Stroke Scale score (admission/discharge), and the modified Rankin scale (mRS) 90 days after stroke onset. One hundred seven were included (mean age, 68.4 years; ≤3 h, n = 84; >3 h, n = 23; intravenous thrombolysis (IVT), n = 51; ≤3 h, n = 40; >3 h, n = 11). In patients with normal MMCT on admission (n = 54), follow-up MRT detected brain infarctions in 23 patients (lacunar strokes, n = 16; infratentorial strokes, n = 4; territorial infarction, n = 3). Sensitivity/specificity/positive predictive value/negative predictive value of any infarct detection was 69.5%/99.8%/99.9%/57.2% and of a any territorial infarct detection was 93.9%/99.9%/99.9%/93.6%, respectively. In univariate regression analysis (time to CT scan, ≤3 h/>3 h; IVT: yes/no; ASPECTS EIH/CBV/MTT, 10/

For prophylaxis or therapy of lagophthalmic keratopathy ointment application or hour glass dressing is indicated. The hour glass dressing has the disadvantage of a continuous visual impairment by steaming up of the moisture chamber. The main problem for the patients is the strong subjective feeling of physical handicap. A therapeutic alternative is the gravity dependent lidloading, which is known as gold implantation in cases of irreversible lagophthalmos.By lead weights of 0.8 to 2.0 g, which are fixed to the upper lid by a foil glueing on both sides (Tesafix) or in allergic patients by an adhesive layer, well tolerated by the skin (Combihesive), the lidclosure can become restored without impairment of lid-opening. In a controlled study the described method was first tested on 10 normal persons and than applied to 22 patients with lagophthalmos. Meanwhile the new method was applied to 32 further patients.The "liddynamic" procedure is effective and well tolerated; it is more accepted, especially during the day, because of better visual function and better cosmetics. The hour glass dressing, is still of importance in serious cases of keratopathy during the night."lidloading" with lead weights, which are glued by an adhesive layer to the upper lid, can be recommended as a new method in cases of reversible lagophthalmos or as a preparing step before a gold implantation.

In facial palsies with lagophthalmic keratopathy an hour-glass dressing is indicated, which has the disadvantage of a moisture chamber with visual impairment by steaming up. After weeks of placement dermal irritations are possible. An alternative is the gravity dependent lidloading, using gold implantation in cases of irreversible lagophthalmos.Lead weights of 0.8 to 2.0 g are glued to the upper lid with an adhesive layer, that is well tolerated by the skin (Combihesive*), or by a simple foil that is glued on both sides (Tesafix). This results in a lidclosure without impairment of lid opening. After tests on 10 normal persons the described method was placed on 22 patients with lagophthalmos and compared with hour-glass dressing. The new method was applied to 36 additional patients.The dynamic lead weight is effective and well-tolerated. It was more accepted cosmetically especially during the day because of better cosmetic appearance and better visual function than was the hour-glass dressing. These latter dressings are still important for use in serious cases during the night.lidloading with lead weights that are glued by an adhesive layer to the upper lid can be recommended as a new method in cases of reversible lagophthalmos or as a preparatory step before gold implantation.

In facial palsies with lagophthalmic keratopathy, an hour-glass dressing is indicated. This dressing has the disadvantage of a moisture chamber, visual impairment occurs by steaming up. After weeks of placement dermal irritations are possible. An alternative is gravity-dependent lid-loading, using gold implantation in cases of irreversible lagophthalmos.Lead weights of 0.8 to 2.0 g are glued to the upper lid with an adhesive layer that is well-tolerated by the skin (Combihesive*) or by a simple foil that is glued on both sides (Tesafix). This results in a lid closure without impairment of lid opening. After tests on 10 normal persons lid-loading implants were placed on 22 patients with lagophthalmos and compared with hour-glass dressing. The new method was also applied to 26 additional patients.The dynamic lead weight was effective and well-tolerated. It was more accepted eosine tically, especially during the day because of its better cosmetic appearance and better visual function than was the hour-glass dressing. These latter dressings are still important for use in serious cases during the night.Lid-loading with lead weights that are glued by an adhesive layer to the upper lid can be recommended as a useful method in cases of reversible lagophthalmos or as a preparatory step before gold implantation.

Thirty patients with irreversible facial palsy were treated with gold implants from 0.5 to 1.8 g. For the immediate treatment of postoperative facial palsies and reversible kinds of lagophthalmos, lead weights of 1.2 to 1.8 g ("lid dynamic eye dressing") are preferred to hourglass dressings. Insufficient orbicularis muscle function is compensated by the weight. The observation time for gold implants is more than 2 years; the fixation time of the adhesive lead weights is 1 to 10 days. Tolerance is excellent to both treatments. The principle is gravity dependent and is limited to the vertical and oblique head position. For sleeping at night, ointment application and pressure on the upper lid by a pillow are advisable; an additional hourglass dressing is possible. No serious keratopathies were seen in any cases. All patients found the return of lid motility from the restored levator muscle function to be a functional and cosmetic advantage. After weighing the advantages and disadvantages, especially the operative/technical expense in relation to the effective results, it would appear that simple lid-loading is so far the best method of treating lagophthalmos.

Therapeutic decisions in cases of arteriosclerotic stenosis of the internal carotid artery usually depend mainly on the degree of stenosis. However, the recommendations with regard to suitable ultrasonographic criteria are so controversial that even authors of repute describe "confusion" and "chaos in methodology".The aim of this study is to assess which of the most frequently recommended sonographic criteria for stenosis best fulfill the requirements of an exact quantification of stenoses of the internal carotid artery.In 42 consecutive cases the preoperative ultrasound findings were compared with the degree of stenosis in surgically removed specimens. The sonographic technique employed consisted of the analysis of 2 direct and 3 indirect hemodynamic criteria of stenosis. In 34 of these cases planimetry was performed, too. The specimens were obtained by eversion thrombendarteriectomy or arteriotomy; for determination of the degree of stenosis a Paladur molded cylinder was prepared and measured.Quantification of the haemodynamic criteria carries provided well with the endarterectomy specimens. Only in two cases there was a deviation of more than 10 %; however, in these two cases a change with time in the degree of stenosis was probably responsible for the discrepancy. In contrast, planimetry usually underestimated the degree of stenosis: In 61 % of the findings the ultrasonographic results differed by more than 10 % from the reference standard.Quantification of stenosis of the internal carotid artery should be made using a combination of direct and indirect haemodynamic ultrasonographic criteria.

In early stages choroidal peripapillary choroidal melanoma may be confused with a choroidal nevus or melanocytoma.A 41-year old female was diagnosed having a choroidal nevus in close proximity to the optic nerve head. Unfortunately the patient did not show up for annual review of this lesion. Ten years after the patient was examined for the second time. A massive increase in tumor size and prominence was noted including serous retinal detachment. Medical work up excluded any metastatic growth, and the globe was enucleated. Three years later, the patient is still healthy and there are no signs of metastatic spreading.Heavily pigmented peripapillary choroidal melanoma with fascicularly vasocentric proliferative structure, but no infiltration of the sclera or the lamina cribrosa of the optic nerve. The tumor cells were of midgrade size and some of the nucleoli were slightly enlarged. Mitosis and a syncytial structure were present. Immunohistochemically S-100 proteins, HMB 45 and NSE were found. The TNM classification was: ICD-O C 69.3; pT2, G1, S0, V0, pNx, pMx, microscopically RO.Pigmented juxtapapillary tumors resembling choroidal nevi require annual surveillance.

Limbal relaxing incision (LRI) is an easy and safe procedure to reduce astigmatism. It should be clarified, whether the effect is discussed controversely, because the varying corneal diameter and consequently the varying LRI position was not considered.56 patients aged 76 +/- 9 years with preoperative astigmatism of 1.6 D (0.6 to 7.0 D) underwent an almost astigmatically neutral cataract procedure (3.2 mm temporal clear corneal phacoemulsification with foldable lens implantation) and received independently from the given corneal diameter limbus related relaxing incisions of 80 degrees length and 0.6 mm depth. We performed the paired LRI in 4.5 mm, 5 mm and 5.5 mm distance from the corneal center on the steeper meridian.4.3 months postoperatively we observed an astigmatic reduction of -1.0 D (-0.1 to -3.2 D) following LRI with a 9 mm optical zone, LRI with 10 mm diameter led to an astigmatic reduction of -0.4 D (-0.1 to -2.9 D) and LRI with 11 mm diameter were followed by an astigmatic reduction of -0.3 D (+0.5 to -1.1 D). Undercorrections were more frequently observed in younger patients, overcorrections more in elderly people.The application of three different kinds of limbus related relaxing incisions, with 4.5, 5 and 5.5 mm distance from the corneal center or 9, 10 and 11 mm optical zone respectively, corresponds to the variable anatomic situation of the limbus, therefore leading to nearly predictable data and explaining the divergent results of previous reports. The nearer the LRI is applied to the corneal center, the stronger is the relaxing effect.

We report the clinical course of a 48 year old woman, who underwent a cranial MRI-examination in 1995, which confirmed the diagnosis of encephalomyelitis disseminata, but also showed a left temporal venous malformation without evidence of prior hemorrhage. Three month after immunosuppressive treatment with Methotrexate in 1997 begun, first hemorrhage in the left temporal lobe occurred with de novo formation of a cavernoma in association to the known venous malformation. The lesion was totally removed after stereotactic guided craniotomy without any complication. The pathogenetic relationship of de novo cavernomas and associated venous malformations and the remarkable association with immunosuppressive treatment will be discussed.

No reviews of orbital gunshot injuries have been published in German ophthalmological journals. In this article biomechanical and prognostic factors of this rare type of injury are analysed.We report on 4 patients, aged 20-63 years, who tried to commit suicide by shooting themselves in the right temple. Clinical and radiological diagnosis as well as treatment by an interdisciplinary team are reported.All patients became blind on the right side despite immediate surgery including reconstruction of the injured bones and soft tissues. Three of four patients suffered severe functional defects in the left eye; one of them is now blind.Orbital gunshot wounds are severe injuries. The prognosis depends on the course of the bullet and the interdisciplinary care.

Unpigmented tumorous changes of the conjunctiva can frequently be classified as chalazion, basalioma or carcinoma.In a 70 years old diabetic female a 2.5 x 2.5 cm tumor of the lower conjunctival fornix was observed. After complete excision the tumor was examined by histologic routine procedures and immunohistochemically. The patient was irradiated by 40 Gy with the linear accelerator.The monotypical secretion of the heavy chain gamma and the light chain kappa demonstrated a well differentiated extramedullar plasmocytoma. A MALT lymphoma could be excluded by the absence of centrocytoid cells. The bone cytology and histology did not give any evidence for a medullar plasmocytoma. The patient is free from local or diffuse tumor growth since 2 1/2 years.Since the worldwide most important ophthalmopathologic statistics of the AFIP from 1984-1989 describes only 1 plasmocytoma out of 2104 tumors of the lids and conjunctiva, the demonstrated case is an important rarity.

The procedures for prophylaxis and treatment of keratopathy following facial palsy with lagophthalmos are unsatisfying from the functional point of view.Three years ago we created a "lid-dynamic" procedure and applied it to 46 patients with Bell's palsy or before implantation of a gold lid weight. Fixation of lead weights of 0.8-2.0 g to the upper lid by a foil adhesive on both sides (Tesafix), can lead to restoration of lid closure. Within 6 years we implanted 24-carat gold weights into the upper lid in 72 patients.In all cases lid function was markedly improved; all patients appreciated the procedure. The lead weights were well tolerated. In 27% of the operative cases we observed a slight underdosage, in 10% a slight overdosage.Lid loading is a simple and effective method for functional and cosmetical rehabilitation of patients with lagophthalmos. Despite the dependence upon gravity, the procedure can be recommended for all cases of facial palsy.

Purpose: To compare the effect on astigmatism of phacoemulsification using a 4.0 mm, no-stitch, clear corneal incision with that of extracapsular cataract extraction (ECCE) using a 12.0 mm, sutured, clear corneal incision. Setting: Augenklinik, Stadtisches Klinikum Karlsruhe, Germany. Methods: The study comprised 211 patients who had cataract extraction and intraocular lens implantation through a superior clear corneal incision; 108 patients had phacoemulsification with a 4.0 mm no-stitch incision, and 103 had ECCE using a 12.0 mm sutured corneal incision. The main outcome measure was amount of astigmatism preoperatively and at 1 week and 3 and 6 months postoperatively. Corresponding medians (lower and upper quartiles) were evaluated. Results: Median surgically induced cylinder was 1.00 diopter (D) (range 0.56 to 1.50 D) in the 4.0 mm no-stitch incision group and 1.75 D (range 1.00 to 2.62 D) in the 12.0 mm sutured incision group. In eyes with preoperative with-the-rule astigmatism, astigmatism decreased from a median of 0.75 D (range 0.50 to 1.00 D) to 0.50 D (range 0 to 1.50 D) in the 4.0 mm incision group. The difference between preoperative and postoperative astigmatism in the 12.0 mm incision group was not statistically significant. Conclusion: Clear corneal cataract surgery leads to a predictable reduction in astigmatism when performed on the steeper axis with a small, no-stitch incision. Larger sutured incisions are not suitable for planned refractive changes but are still recommended in certain cases such as hard cataract and glaucoma.

Postoperative fibrinous reactions following the small-incision technique seem to be very rare. Problem cases can be treated with tissue Plasmin Activator.To investigate the new prophylactic and therapeutic possibilities the last 2,056 cataract procedures with PMMA lens implantation were statistically analyzed.In 152 (7.4%) fibrin cases we observed after ECCE (n = 586) 12.8%, after phacoemulsification (PE) with sutured 6.5-mm corneoscleral incision (n = 546) 7.0% and after PE with clear corneal selfsealing 4.1-mm incision (n = 924) only 3.9% fibrinous reactions (p0.01). In case of ECCE the rate of fibrinous reactions amounted to 22.4% following prolonged procedures (50 min), while it was low as 9.5% with a short period of time (30 min) (p0.01). In 8 steroid-resistant cases we applied 25 micrograms Plasmin Activator intraocularly. The injections led to complete dissolution of the fibrinous membranes without complications.Postoperative fibrinous reactions can be reduced statistically significantly by application of the corneal small-incision technique. The most efficient therapy is intraocular Plasmin Activator fibrinolysis.

Self-sealing corneal cataract procedures have been known since 1992 and are recommended for foldable silicon lenses. Our goal was a technical modification without refractive disadvantages, which would also allow for the implantation of more rigid, but less expensive PMMA lenses of high optical quality.The "scleral stretch incision" by Freeman (1991) was applied to the corneal situation by widening the inner wound lip up to 6.5 mm; this makes it possible to implant a 5 mm optic through an outer wound opening of 4-4.2 mm. This method has been used since October 1992 on 980 patients, whereby the lamellar corneal incision was placed 1.5-1.8 mm deep to compensate for the astigmatism, if possible in the steeper meridian.The first 107 cases were followed up for longer than 6 months; they showed a surgically induced astigmatism of 1.18 +/- 0.79 D and a postoperative astigmatism of 0.86 +/- 0.70 D. Neither postoperative infection nor hypotony was observed in any of the cases.The modified corneal tunnel incision is recommended for 5 mm PMMA lenses; this is especially suited for cases of low to midgrade preoperative astigmatism that can be optimally improved by this procedure. In cases of pure spheric refraction, a 3 to 3.5 mm incision with foldable lens implantation is preferred.

Can the capsulotomy with small radial incisions compared to the continuous curvilinear capsulorhexis with perfect IOL centration still be recommended? This problem should be clarified under standard conditions.On 100 patients both procedures were applied alternating with corneoscleral incision, phacoemulsification and endocapsular implantation of a 6 mm one-piece PMMA lens.The perfect IOL-centration following capsulorhexis (0.25 +/- 0.31 mm) is confirmed. But the circular capsular fibrosis can make it difficult to examine the fundus periphery or to perform a secondary cataract aspiration. The IOL centration after capsulotomy is not always ideal (0.55 +/- 0.51 mm) but sufficient. Advantages are the simplicity of the procedure and an easy approach to the posterior capsule in case of secondary cataract.Both methods are still appropriate.

In 20 patients with irreversible facial palsy, dynamic voluntary lid closure was achieved by implantation of 0.8-1.6 g gold weights in the upper lid. Because of the high rate of efficiency, low complication rate and technical simplicity, this method is recommended more often than comparable procedures (magnet or wire-spring implantation).

Seven weeks after a generalized cerebral seizure a 27-year-old woman from Ghana developed nausea, vomiting and weight loss, gradually increasing over two weeks. Cranial computed tomography revealed several hyperdense formations with extensive associated oedema and a midline shift. Among extensive biochemical tests only a raised erythrocyte sedimentation rate of 24/50 mm and leukopenia of 2,600/microliters (with normal differential count) were notable. Diagnostic laparotomy was performed because of sonographic and computed tomographic evidence of enlarged abdominal lymph nodes. Histological examination of representative lymph nodes and of tiny nodules deposited on the peritoneum revealed caseous granulomatous inflammation. Mycobacterium tuberculosis was cultured from these specimens. Antituberculosis treatment was started with 0.3 g/d isoniazid, 0.6 g/d rifampicin, 2 g/d pyrazinamide and 1 g/d streptomycin, plus dexamethasone, 4 mg four times daily. After eight weeks treatment an intracerebral focus, removed to exclude neoplasm, proved histologically to be a tuberculoma. Only after four months was it possible to reduce the glucocorticoid dosage to prednisone, 20 mg/d. The antituberculosis treatment was continued for 18 months, with only isoniazid and rifampicin taken during the last 14 months. Final clinical and biochemical examinations were unremarkable. Computed tomography demonstrated regression of the abdominal lymph nodes and the cerebral foci. The patient was without any symptoms.

Infrared pupillography was used to determine physiological time parameters of the direct phasic light reflex in 101 normal subjects. These parameters include latency, contraction and redilatation times as well as total reaction time, measured in response to 4 different light stimulus intensities. A total of more than 4000 pupillary stimulations in these subjects was analyzed statistically. The study of these time parameters included determination of the mean, the standard deviation and three dimensional analysis of variance, with the following results: 1. The physiological time parameters are bilaterally symmetrical. 2. The most stable parameters with least variability in an individual subject were latency and contraction time. The redilatation time, however, showed marked intra-individual variability. 3. Variance analysis revealed a high degree of correlation among the different parameters to light stimuli of varying intensity. 4. There was a highly significant prolongation of latency and of contraction time with increasing age. 5. No sex dependent effect on physiological time parameters was found. In addition, no interaction among light stimulus intensity, age and sex could be detected. Possible applications of the method for clinical neurological examination are discussed.

A report on 22 patients with idiopathic facial paralysis is given. A systematic interdisplinary examination in short intervals was carried out. The efficiency of the more time consuming and technically more difficult methods was checked. At the same time it was investigated whether as a result of these findings new aspects of early therapy and prognosis could be expected.

A report on the neurological results of an investigation carried out on 38 patients with pigmentary degeneration of the retina. The ophthalmological investigation did not indicate a systemic disease. The neurological findings, however, revealed a systemic disorder in many cases.-The constitutions of 20 patients (52.5%) were marked by pathological stigmata. The EEG was abnormal in more than 50% of the cases. The EMG showed a prolonged nerve conduction time for 2 of a total of 4 deaf patients. The EMG also revealed a patient with both pigmentary degeneration of the retina and a mold form of myasthenia gravis. The biochemical results indicated hyperlipoproteinemia in 6 cases (15.8%), diabetes mellitus in 2 cases and a pathological increase of uric acid in the serum in 8 cases. These results suggest that pigmentary degeneration of the retina is not a homogenous disease. It must be seen as a "phenomenologically identical, polygenetic type of reaction" (Janzen). A comprehensive analysis of the individual case can therefore lead to implications which are of direct therapeutic significance.

A report given of a 32-year-old female patient who exhibited a unilateral paradoxical pupillary reaction to light, but was obviously healthy in all other respects. All of the neuroophthalmological findings, including the infrared reflex-pupillographic results registered, are indicated and discussed in detail in the report. An analysis of the findings leads to the conclusion that this disturbance has a central, prenuclear location. Apart from a stenosis at the junction of the arteria vertebralis, which was determined angiographically, further examination did not reveal any pathological findings.

In a survey of 46 patients with haemagioblastoma of the CNS (Neurology Dept. University Hamburg, 1950-1980) most (n = 40) were found to have angioblastomas of the cerebellum (Lindau tumors). Of these patients 21 were re-examined in 1983. Headache was the most frequent initial symptom (43%), and within this group one-third (10%-15%) had dizziness, sensorymotor deficits and cerebellar gait disturbances. Signs of elevated intracranial pressure much more often led to the correct diagnosis than dizziness or dystaxia. After the introduction of CCT to the diagnostic procedure the combined evaluation of angiography of the vertebral arteries and CCT always permitted the correct diagnosis. The low neurosurgical mortality rate (13.5%) has decreased to 0% within the last decade. No relapses were found in 21 re-examinations including CCT and EOG compared to a frequency of 9.7% in all 46 cases. Significantly less often than expected from other data we found: signs of possible hereditary influence (0%), multiple tumor localization combined with angiomatosis retinae (0%), polyglobulia (10.8%). Psychopathologically relevant signs (45.6%) were, in all cases, combined with signs of increased intracranial pressure. The results of our re-examination demonstrate that late postoperative deficits as well as possible relapses are earlier and more precisely evaluated by the combined use of clinical examination, CCT and EOG.

A case of anterior cleavage syndrome in a 27-year-old female patient is described. It is remarkable for the unusual completeness and prominence of the symptoms. In addition to medodermal anomalies of the anterior segment (e.g., prominent ring of Schwalbe, posterior embryotoxon, peripheral anterior synechiae, hypoplasia of the iris stroma) the case also manifested microcornea, cataract, glaucoma, excessive myopia, partial anodontia vera with maxillary hypoplasia and telecanthus. It is the first time that Rieger's syndrome has been described in association with endocrine orbitopathy, caused by hyperthyroidism with isolated elevation of T3 in the blood serum and a soft struma. Possible genetic correlations and the role of the secondary glaucoma in the two syndromes are discussed.

Botulinus poisoning generally ends fatally after an only short course, mild forms being rare. In four members of a family who had eaten deep-frozen grilled sausages symptoms of botulinus poisoning developed, predominantly marked by disturbances of the autonomic nervous system. The course was so mild that hospitalization was not necessary nor was specific antitoxin administration. All clinical symptoms had disappeared after nine weeks. But special clinical and neurophysiological studies (electromyography, pupillography, electroencephalography) demonstrated abnormalities for very much longer. Botulinus poisoning was proven in animal tests and neutralization tests with botulinus antitoxin. The epidemiology, source of infection, and clinical symptoms suggest botulinus type B poisoning.

A report is given on a 47-year-old woman with periarteritis nodosa. A unilateral retinopathy with disseminated cotton-wool-spots surrounded by retinal haemorrhages and edema was observed. Fluoresceinangiography showed a multifocal constriction of the retinal capillaries. This lesion of the retinal vessels can be distinguished from those caused by hypertension. An acute immunological reaction has been suggested as the pathogenesis. Some authors have described this retinal lesion in collagen diseases as a "toxic" retinopathy. We prefer the term acute immunological microangiopathy (AIM).

Blunt trauma can lead to intra-orbital haemorrhage, which cause motility and sensibility disturbances typically found also in orbit-floor fracture. When there is also a fracture present, it can be problematic to give the function deficiencies their right causes. Spontaneous improvement of the defiencies can be expected in a reasonable percentage of cases. Operative revision therefore should not be performed in cases of doubt, or in any case not earlier than 14 days after the injury. In a severe stove-in orbit with enophthalmos and double vision the operation should be preformed within the first week. In some cases, however, residual diplopia can be expected in spite of optimal early therapy.

1. 37 patients with pigmentary retinopathy, seeming otherwise healthy, were thoroughly examined for fine symptoms in other parts of the body, in particular for neuropathies and for blood chemistry peculiarities. 2. In 19 cases some constitutional pathology was discovered, in 4 cases hearing was affected by inner ear changes. 3. Clinically patients behaviour was changed and with electroencephalgraphy an encephalopathy was found in 7 cases. 4. Electroencephalograms were in more than 50% of cases slightly abnormal. 5. A myastenic reaction, confirmed by electromyography, occurred in one case (but not in its sibling who had also a pigmentary retinopathy.) 6. In 14 cases electromyogram was showing a neurogenic damage. Motor nerve conduction speed was lowered in 2 cases, in 5 cases it was at the lower limit of normal. In 6 cases very long distal latence times were noted. In 2 cases action potential was shortened but this could not be considered as a myopathy for certain. 7. In 6 cases a hyperlipoproteinaemia occurred. In 2 cases a mild diabetes mellitus, in 7 cases uric acid level in serum was raised. The number of cases was too small to give a statistical importance to those findings. 8. Cerebrospinal fluid was found normal in all cases. 9. This study should lead to further investigations. In spite of the small number of cases one can presume that pigmentary retinopathy is not an independent defect but is a phenomenon of a polygenetic entity.

Four cases of mild botulinus type B intoxication are reported with the clinical neurophysiological studies. Electromyographically tetanic stimulation of the N. ulnaris initially led to tetanic potentiation of the muscle action potential. After an interval without any symptoms tetanic reduction became apparent between the fourth and seventh week of the illness. The electroneurographic finding was normal during the observation period of three months. Infrared reflex pupillographic studies revealed that the cholinergic autonomous synapses were disturbed for a period much longer than the symptoms clinically established. The speed at which the pupillographic findings returned to normal varied considerably according to the patient. Electroencephalographic results indicated generalized groups of slow high amplitude waves in three of the four cases during the period of observation. The pathophysiology of botulinus intoxication is discussed with a consideration of the literature and the results of this study.

In cases of homonymous hemianopsia, with the aid of pupillographic perimetry it is possible to differentiate between infra- or suprageniculate lesions. Principally the validity of Wernicke's theory remains unchanged. However in processes in the central visual pathways a hemihypokinesia of varying degree may be found. In the blind visual fields so far in our cases a congruence between pupillographic and perimetric thresholds has not been confirmed. The phenomenon of hemihypokinesia of the pupil is discussed.

Clinical-neurological, electrocephalographic, ophthalmological and genetic observations of a family with related persons with Leber's optic atrophy is described. 6 male subjects of the altogether ca. 100 members of the family tree stretching over 4 generations were manifestly affected relatives - that is possible carriers, an unusually large number of EEG's were abnormal, and, less often, colour vision disturbances were seen. The results suggest that Leber's optic atrophy is a hereditary neuro-ophthalmological systemic condition, whose most striking symptom is the optic atrophy. The genetic factors of the condition are also discussed.