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Biomedical subjects

E Nikoskelainen

Publications and source records attributed to E Nikoskelainen.

At least 55 records · Page 3Linked to original sources

Fundus findings in Leber's hereditary optic neuroretinopathy. III. Fluorescein angiographic studies.

Fluorescein angiography was performed in nine members of families with Leber's disease. Serial studies were obtained in four men observed from asymptomatic to atrophic stages. Peripapillary microangiopathy was observed in six of nine asymptomatic eyes. Arteriovenous shunting occurred in the telangiectatic vascular bed. These changes remained stable in some eyes and progressed in others. In the acute stage, arteries and telangiectatic vessels were maximally dilated and flow was rapid. Angiography showed florid shunting in lower and upper vascular arcades and reduced filling of papillomacular capillaries. Vessels of the shunting vascular bed gradually narrowed irregularly. In the atrophic stage, disc vascularity diminished and arteriovenous circulation time increased markedly. Arterioles narrowed and peripapillary microangiopathy disappeared. These findings support our contention that Leber's disease is a hereditary vascular neuroretinopathy.

Adult↗

Monoclonal IgG immunoglobulinemia with subsequent myeloma in psoriatic arthritis.

A 42-year-old man had suffered from skin psoriasis for 27 years, psoriasis arthritis for 6 years and corneal crystalline deposits for 1 year, when an IgG-kappa monoclonal immunoglobulin was detected in his serum electrophoretic pattern. All the diagnostic studies for multiple myeloma were negative. However, 16 months later he developed this disease.

Adult↗

Ophthalmoscopic findings in Leber's hereditary optic neuropathy. II. The fundus findings in the affected family members.

Eighteen men and four women had Leber's disease. Fundus photographs obtained in three cases showed the following: Peripapillary microangiopathy, present from the beginning, slowly increased during the presymptomatic stage. At the end of the presymptomatic stage, the nerve fiber layer became swollen. During the acute stage, retinal vessels on and around the disc were dilated, tortuous, and telangiectatic. Nerve fiber layer hemorrhages occurred in two eyes. As atrophy appeared first in the papillomacular bundle and then in the remaining retina, the vascular bed involuted, leaving a capillary-poor retina with attenuated arterioles and a pale optic disc. The final degree of atrophy varied. Fifteen of the affected persons were blind with severe optic atrophy, but in seven optic atrophy was only partial, causing less visual handicap. Our results suggest that Leber's disease is primarily an intraocular, not a retrobulbar, optic neuropathy. It should be redesignated as Leber's hereditary neuroretinopathy.

Adolescent↗

Interferon-alpha and interferon-gamma production capacity of idiopathic isolated optic neuritis patients.

Interferon-alpha and interferon-gamma production by idiopathic isolated optic neuritis (ON) patients was studied. The production capacity was compared with that in two control groups: patients with iritis and healthy control subjects. A sensitive and reliable interferon bioassay was applied for interferon level measurements. Statistically significant differences were not found between patients and control groups in either interferon-alpha production or interferon-gamma production.

Adult↗

Ophthalmoscopic findings in Leber's hereditary optic neuropathy. I. Fundus findings in asymptomatic family members.

A neuro-ophthalmological examination was performed on 47 asymptomatic offspring in the female line and 13 offspring in the male line in families with Leber's hereditary optic neuropathy. Fourteen (61%) of the 23 asymptomatic males and nine (38%) of the 24 asymptomatic females in the female line had telangiectatic microangiopathy of the peripapillary arterioles and capillaries. Increased tortuosity of capillaries, medium-sized arterioles, and venules were also seen in the fundi of several asymptomatic family members. The 13 offsprings in the male line did not have microangiopathy. Nerve fiber abnormalities were not seen in asymptomatic persons. The photographically verified findings are described, classified, and discussed. It is suspected that Leber's disease is primarily a microvascular disorder. The vascular abnormalities detected in the asymptomatic family members probably indicate increased risk of Leber's disease developing. During the prospective follow-up of three years, three asymptomatic boys with microangiopathy have experienced Leber's disease.

Adolescent↗

Do visual evoked potentials give relevant information to the neuro-ophthalmological examination in optic nerve lesions?

The visual evoked potentials (VEPs) and neuro-ophthalmological examinations of 134 patients were compared. The VEPs were abnormal in 95% of the eyes with optic neuritis. Defective color vision was found in 99%, visual field defects in 88%, decreased vision in 66% and an afferent pupillary defect in 55%. 29 patients with optic neuritis were followed up with repeated tests. VEPs and color vision recovered more slowly than visual acuity and visual field. Abnormal VEPs were observed in 68% of 50 MS patients. An analysis of symptomatic and asymptomatic eyes showed that testing of color vision, visual field and red-free ophthalmoscopy were equally as useful diagnostic tools as VEPs. 4 (8%) of the MS patients had abnormal VEPs despite a normal neuro-ophthalmological examination; 94% of MS patients with symptoms and 47% of MS patients without visual symptoms had abnormal VEPs. VEPs were pathological in 59% of 24 patients with traumatic or compressive optic nerve diseases or optic atrophies of unknown etiology. The neuro-ophthalmological examination was more sensitive than VEPs in the diagnosis of these disorders. A neuro-ophthalmological examination is in most cases sufficient to diagnose optic nerve lesions. VEPs are of diagnostic aid especially in mild optic nerve lesions.

Diagnosis, Differential↗

Prognosis of optic neuritis with special reference to cerebrospinal fluid immunoglobulins and measles virus antibodies.

Forty-eight patients with optic neuritis (ON), first seen in 1970 to 1973, were neurologically and neuroophthalmologically reexamined after 7 to 10 years. Twenty-seven patients (56%) had probable MS, and 9 (19%) had possible MS. During the attack of ON, the cerebrospinal fluid (CSF) samples and serum/CSF measles antibody ratios were studied. Twenty patients had increased relative immunoglobulin G (IgG) (% of total protein) in their CSF; 19 of these had probable or possible MS. However, 17 of 28 patients with a normal relative IgG value had also developed MS. CSF electrophoresis was abnormal in 20 patients with ON; reexamination showed that 19 had probable or possible MS. Sixteen of 27 patients with normal electrophoresis had also developed MS. Serum/CSF measles antibody ratio had decreased in 19 patients; 13 of these had probable MS and 3 had possible MS. Of 29 patients with a normal measles antibody ratio, 14 had probable MS and 6 had possible MS. The conclusion is that examination of the CSF in ON gives valuable prognostic information because increased relative IgG, abnormal electrophoresis, or a decreased measles antibody ratio implies a high risk of developing MS. A normal CSF does not, however, rule out the possibility of dissemination.

Antibodies, Viral↗

Parotid carcinoma and posterior fossa schwannoma following irradiation. Report of a patient treated in infancy for benign ear disease.

A 23-year-old man had received irradiation for a benign ear problem at the age of 1 year. Twenty year later, a malignant left parotid carcinoma and a right jugular foramen schwannoma developed. Earlier reports in the literature suggest that radiation can induce tumors in the head and neck. The localization of both tumors and the histological features of the parotid tumor suggest that they were radiation-induced.

Adenocarcinoma↗

The early phase in Leber hereditary optic atrophy.

Clinical, ophthalmoscopic, perimetric, and color vision tests and visual evoked responses were recorded in symptomatic and asymptomatic eyes in five members of a family with Leber hereditary optic atrophy. The presymptomatic eyes showed abnormalities in the retinal nerve fiber layer. Optic nerve dysfunction was found before central vision failed. The appearance of degenerating retinal nerve fibers, particularly in the papillomacular bundle, was documented photographically in the weeks after the onset of visual symptoms. Treatment with prednisone and hydroxocobalamin did not reverse or halt serious impairment of vision.

Adolescent↗

Computerized tomography of the orbits. A report of 196 patients.

The value of orbital CCT scans was studied by grouping the CCT scans of 196 patients into clinical diagnostic categories. In 83% of patients with suspected Graves' ophthalmopathy, CCT confirmed the diagnosis. The CCT scan differentiated patients with Graves' disease from those with orbital pseudotumour or malignancies. The CCT scan was positive in 92% or patients with proven orbital malignancies. In 73% of patients with proptosis of unknown aetiology, and in 56% of patients with ocular motility problems, the CCT scan led to the diagnosis. The CCT scan ruled out orbital mass lesions in cases of optic neuritis, optic atrophy or vascular malformations. The greatest value of the CCT scan was in the localization and determination of the size and shape of various orbital lesions and in the accurate follow-up of those lesions utilizing serial scans.

Adult↗

Virus antibody levels in serum and cerebrospinal fluid specimens from patients with optic neuritis.

Virus antibody levels in serum specimens from 77 patients with optic neuritis (ON) were compared with those in healthy controls which had been matched with regard to sex, age and place of residence. A group of 58 patients with various neurological diseases other than multiple sclerosis (MS) or infectious diseases of the central nervous system (CNS) served as a second control group. The ON patients had significantly higher measles antibody titers in serum than the two control groups in both measles HI and HLI tests. Tests of cerebrospinal fluid (CSF) specimens revealed similar differences between ON patients and controls. Tests for antibodies to other viruses showed no statistically significant differences between ON patients and controls. There were several ON patients with normal serum/CSF albumin ratios but low serum/CSF IgG and measles antibody ratios. An increase in measles antibody CSF titers was observed during the study time in two ON patients. The results support the hypothesis that local production of measles antibodies takes place in the CNS of some patients with ON as has been earlier suggested to occur in patients with MS.

Antibodies, Viral↗

Orbital involvement by plasmacytoma. Report of two cases.

Two patients with orbital involvement of plasma cell myeloma are presented. The first patient presented an isolated plasmacytoma in the orbit; the second patient had generalized plasma cell myeloma. In both cases X-rays and computed tomographic scanning gave valuable information and biopsy confirmed the diagnosis.

Aged↗

Virus antibody levels in serum specimens from patients with optic neuritis and from matched controls;.

Virus antibody levels in serum specimens taken in acute and convalescent phases from 77 patients with optic neuritits were tested by measles hamagglutination inhibition (HI), measles hemolysis inhibition (HLI), rubella HI, parainfluenza-1 HI, Epstein-Barr immunofluorescence (IF), and against 11 other viruses and mycoplasma pneumoniae with the complement fixation (CF) technique. The virus antibody levels were indicated to be usually very stable, and a fourfold change in virus antibody levels was demonstrated in only eight patients. The virus antibody levels were compared with specimens from two carefully selected control groups. The first control group consisted of 71 healthy persons matched in age, sex and place of residence with the patients with optic neuritis. The other control group consisted of 58 patients with various neurological diseases other than multiple sclerosis (MS) or infectious diseases of the central nervous system. The patients with optic neuritis had significantly higher measles antibody titres than the two control groups in both measles HI and measles HLI tests. Also in 33 patients with optic neuritis of unknown cause, the measles antibody levels were higher than in the control groups. On the other hand, various other antibody tests showed no statistically significant differences between patients with optic neuritis and the control group.

Acute Disease↗