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Biomedical subjects

T Mannen

Publications and source records attributed to T Mannen.

At least 37 records · Page 2Linked to original sources
PubMed1993

Inherited amyloid polyneuropathy type IV (gelsolin variant) in a Japanese family.

We describe a Japanese family with familial amyloid polyneuropathy type IV. The family originates from central Japan, Nagano prefecture, and is unrelated to Finnish or other Caucasian populations. Of 42 members in three generations, 14 individuals (5 men, 9 women) are affected by corneal lattice dystrophy, cranial neuropathy, mild peripheral neuropathy, and skin changes. Polarizing microscopy and immunohistochemistry studies of skin biopsy samples demonstrated abundant amyloid deposits, which bound an antigelsolin monoclonal antibody. Direct sequence analysis of a DNA fragment spanning codon 187 of plasma gelsolin complementary DNA and restriction analysis using a modified polymerase chain reaction demonstrated a single base substitution, guanine to adenine, at nucleotide position 654, which is identical to the mutation in Finnish familial amyloid polyneuropathy type IV. This strongly suggests that the mutation causes the familial amyloid polyneuropathy type IV phenotype regardless of ethnic background.

Adult↗
PubMed1993

The interval between the positive peak of premyoclonus spike and the onset of myoclonus is shorter than the cortical latency in cortical myoclonus.

In 6 patients with cortical myoclonus, the intervals between the positive peak of the premyoclonus cortical spike and the onset of the myoclonic jerk [interval (Sp-Mcl)] were precisely measured by the computer-assisted jerk-locked averaging method. The intervals (Sp-Mcl) were 13.0-15.8 ms for the abductor pollicis brevis, 8.0-10.4 ms for the extensor digitorum communis and 5.5 ms for the biceps brachii, respectively. These were apparently shorter than the normal latencies of the responses elicited by transcranial cortical stimulation. The difference between the interval (Sp-Mcl) and the spinal latency was -0.8 to 3.5 ms, which was not long enough for the time required for the descending volleys going down from the cortex to the spinal cord. We have concluded that the motor cortex is activated before the positive peak of the premyoclonus spike in cortical myoclonus.

Adult↗
PubMed1992

Paired helical filaments in astrocytes: electron microscopy and immunohistochemistry in a case of atypical Alzheimer's disease.

A 54-year-old man who had cerebellar ataxia and pseudobulbar palsy at the age of 29 years, and soon developed dementia, myoclonus and convulsions, died after about 20 years in a vegetative state. Histological examination of the extensively atrophic and devastated brain (680 g) revealed the almost total loss of cerebral cortical neurons associated with numerous beta-protein amyloid plaques, many extracellular tangles and a large number of hypertrophic astrocytes, and prominent amyloid angiopathy. The astrocytes were frequently immunopositive for anti-human tau antibody (anti-htau) and anti-ubiquitin antibody (anti-ubi). Double immunostaining with anti-htau and anti-glial fibrillary acidic protein (GFAP) antibody clearly demonstrated htau-positive domains within the GFAP-positive perikarya/and processes of several astrocytes. Electron microscopy of the hippocampal CA1, which was completely devoid of pyramidal neurons, revealed, in astrocytes, abnormal filaments indistinguishable from the paired helical filaments (PHFs) seen in neurons. On immunoelectron microscopy, the filaments were observed to be labeled with anti-htau and anti-ubi, exhibiting the same immunohistochemical features as neuronal PHFs. This is the first demonstration of clearly constricted and both tau- and ubiquitin-positive PHFs in astrocytes, indicating that, in some special conditions like in our case, processes similar to those that attack neurons also affect astrocytes and ultimately make the latter form PHFs.

Adult↗
PubMed1992

Immunohistochemical study of kuru plaques using antibodies against synthetic prion protein peptides.

Prion protein (PrP) is a protein closely associated with the transmission of scrapie and Creutzfeldt-Jakob disease (CJD). Kuru plaques are composed of this protein. PrP33-35 is converted to protease-resistant PrP27-30 by proteinase K digestion. It has not yet been determined which of these PrPs is present in kuru plaques in vivo. Accordingly we synthesized two peptides (peptide-N and peptide-M) that, respectively, corresponded to the protease-sensitive and protease-resistant portions of PrP33-35, based on the amino acid sequence deduced from human PrP cDNA. These two synthetic peptides were used to immunize rabbits and produce antisera (anti-N and anti-M). Both antisera stained kuru plaques in a patient with Gerstmann-Sträussler syndrome and one with CJD. Peptide-N has an amino acid sequence which does not exist in PrP27-30. Staining of kuru plaques by the antiserum against peptide-N indicated that the entire molecule, including the N-terminal portion of PrP33-35, was deposited in the kuru plaques.

Aged↗
PubMed1992

Developmental changes of fucosylated glycoconjugates in rabbit dorsal root ganglia.

Developmental changes of the fucosylated glycoconjugates in the dorsal root ganglia (DRG) of the rabbit were investigated histochemically using anti-fucosyl GM1 antibody and Ulex europaeus agglutinin 1 (UEA-1) lectin. Neither anti-fucosyl GM1 antibody nor UEA-1 lectin bound to the neural tubes or to the neural crest on embryonic day 14 (E14). Anti-fucosyl GM1 antibody binds diffusely to the DRG of E25. Large neurons unreactive with anti-fucosyl GM1 antibody appeared at 1 month and increased within 6 months after birth. Schwann cells immunoreactive with anti-fucosyl GM1 antibody came to be limited to the satellite cells surrounding the positive neurons. No staining with UEA-1 lectin was observed in the DRG of E25. Some small neurons became reactive with UEA-1 lectin within 1 month and remained to be so at 6 months after birth. Schwann cells including satellite cells were unreactive with this lectin. Since fucosyl GM1 was detected in the lipid fraction of DRGs from 1-month-old and 6-month-old rabbits, fucosyl GM1 itself should be the antigen molecule recognized by the anti-fucosyl GM1 antibody. Further study is necessary to elucidate the association between these developmental changes of the fucosylated glycoconjugates in DRG and their possible functional roles.

Animals↗
PubMed1992

Stimulation of corticospinal pathways at the level of the pyramidal decussation in neurological disorders.

A newly developed technique of brainstem stimulation was applied in 14 normal subjects and 23 patients with various neurological disorders. The electromyographic (EMG) responses of limb muscles following cortical, brainstem and cervical stimulation were recorded. The cortical-brainstem conduction time and brainstem-cervical conduction time were then calculated from the difference in latency between the two sites of stimulation. From the regression lines of the relationship between the normal conduction times in the first dorsal interosseous muscle and the length of the descending tracts, the site of activation by brainstem stimulation was estimated to lie near the cervical-medullary junction. The most distal lesion causing prolongation of cortical-brainstem conduction time was a small cerebral infarction in the lower pons. Herniation of the third cervical spinal disc was the most rostral lesion resulting in delayed brainstem-cervical conduction time and normal cortical-brainstem conduction time. These observations suggest that activation occurs at the level of the cervical-medullary junction where the pyramidal decussation lies. The conduction velocities of the activated tracts estimated from the regression lines for normal individuals were 57-92 m/s. In patients with supratentorial lesions, the threshold for brainstem stimulation was abnormally high. The abnormal findings in this test were correlated significantly with the clinical pyramidal signs. This suggests that the EMG responses elicited by brainstem stimulation are mediated mainly by the corticospinal tract. We conclude that the brainstem stimulation technique would be clinically useful for localization of lesions in the corticospinal tract; the primary lesion can be localized whether above or below the pyramidal decussation.

Adult↗
PubMed1992

Nation-wide collaborative study on the long-term effects of bromocriptine in the treatment of parkinsonian patients. Final report.

Final results of the 5-year multicentric collaborative study on the long-term effects of bromocriptine in the patients with Parkinson's disease are reported. This prospective study started in May 1985 in order to see whether the early combination therapy with bromocriptine and levodopa is really superior to the levodopa monotherapy with regard to the late side effects of levodopa in the treatment of parkinsonian patients. Another project of the study was to see the therapeutic efficacy of bromocriptine monotherapy without concomitant use of levodopa. For these purposes, a total of 702 patients with Parkinson's disease were enrolled into three groups: Group 1 (n = 286) with bromocriptine monotherapy, Group 2A (n = 216) with early combination of bromocriptine and levodopa, and Group 2B (n = 200) with levodopa alone. At the end of the 5-year study, 48 patients in Group 1 (16.8%) were still continuing bromocriptine monotherapy with satisfactorily good therapeutic effects. About half (49.1%) of the Group 2A patients remained on the combined therapy, and the comparable number of the Group 2B patients (46.0%) were also kept on the initial mode of therapy, while 13.5% of the latter group with levodopa monotherapy needed bromocriptine to be added in order to assure the good therapeutic effects. Moreover, significant differences were seen between group 2A and Group 2B with regard to the incidence of wearing-off phenomenon and dyskinesias. Disappearance rate of dyskinesias which were present at the time of enrollment was significantly higher in Group 2A than in Group 2B. No significant difference was noted as to the incidence of untoward symptoms and the death rate among all three therapeutic groups. These results support the view that the early combination of bromocriptine with levodopa is superior to levodopa alone in the treatment of Parkinson's disease.

Activities of Daily Living↗
PubMed1992

Nation-wide collaborative study on the long-term effects of bromocriptine in the treatment of parkinsonian patients: analysis on the maintenance and the change of the original mode of treatment.

A nation-wide collaborative study to evaluate the long-term effects of bromocriptine in patients with Parkinson's disease was completed as described in the accompanying paper. The present study analysed the same data by paying attention to a group of patients who maintained the original mode of therapy and to a group of patients who changed the mode of treatment by adding levodopa or bromocriptine to the original drug. Surprisingly, 48 among 286 patients in a group of bromocriptine monotherapy maintained the original mode of therapy. This group has particular features of a short duration of illness and a low grade of Hoehn-Yahr's scale. It is noteworthy that this group of patients did not show wearing-off phenomenon. The effects of additional bromocriptine to levodopa for a 5-year period were analysed by comparing two groups of combination therapy and levodopa alone therapy maintained for 5 years, with 106 and 92 patients, respectively. Results were essentially the same as those obtained from the accompanying paper, i.e., in general, treatment by combination with bromocriptine may be more suitable than treatment by levodopa alone. In order to find the best timing of the combination of levodopa and bromocriptine, results of 3 groups were compared, i.e. a group of patients who started with bromocriptine alone and later added with levodopa (82 patients), a group of patients who maintained the combination for 5 years (106 patients) and a group of patients who started with levodopa alone and later added bromocriptine (27 patients). The best results were obtained in the group of 5-year combination.(ABSTRACT TRUNCATED AT 250 WORDS)

Activities of Daily Living↗
PubMed1992

Accumulation of amyloid precursor protein and beta-protein immunoreactivities in axons injured by cerebral infarct.

To determine the distribution of amyloid precursor protein (APP), monoclonal antibodies against APP45-62 (APP1-28-9) and beta1-17 (4A18 and 4A61) were produced. In the sections of Alzheimer's disease (AD) brain, APP1-28-9 was reactive with neurites around senile plaques and a few neurons but not with amyloid cores. This antibody also immunostained the axons in the ischemic lesions of brain tissues from cases with cerebral infarct. 4A18 and 4A61 were reactive with amyloid plaques but not with neurites and neurons. The latter two antibodies also immunostained axons in ischemic lesions. These findings suggest that APP, transported by the fast axonal flows, accumulated in the injured axons in the central nervous system. The beta immunoreactivity appearing in those axons may provide a clue to the mechanism of amyloidogenesis.

Alzheimer Disease↗
PubMed1992

Lidocaine unmasks silent demyelinative lesions in multiple sclerosis.

Blockage of a small number of sodium channels may prevent impulse conduction in some demyelinated segments of nerve fibers with low safety factors, thereby unmasking subclinical demyelinative lesions. On the basis of this hypothesis, lidocaine, a sodium channel blocker, was administered intravenously to 28 MS patients and to 19 normal subjects and seven patients with nondemyelinating diseases. As predicted, lidocaine (mean plasma level, 2.7 micrograms/ml) elicited reversible subclinical symptoms in 23 of the MS patients, but it had not effect on the control subjects. We made a quantitative study of the visual functions (visual acuity, color vision, visual evoked potential [VEP]) that were impaired in 15 MS patients. Of the 23 affected eyes, nine showed normal VEPs, indicative of the test's sensitivity to focal lesions. This test should be useful in the diagnosis of MS and in the evaluation of the subclinical activity of MS as well.

Adult↗
PubMed1992

[Familial amyloidotic polyneuropathy type IV (Finnish type)--the first description of a large kindred in Japan].

Familial amyloidotic polyneuropathy type IV, one of the hereditary systemic amyloidoses with an autosomal dominant trait, is clinically characterized by cranial neuropathy and corneal lattice dystrophy. Recent biochemical studies have indicated that the amyloid fibril protein in FAP IV is related to gelsolin, an actin-modulating protein. Cases were clustered in the Finnish population and only a few cases have been reported from other populations. Here we described a large kindred with FAP IV as the first report in Japan. This family comprises 42 members in three generations with 14 affected individuals. We examined 7 patients at the age ranging from 43 to 80 years. All cases have corneal lattice dystrophy type II. The disease begins with slowly progressive facial weakness in the fifth or sixth decade of life and consequently the V, XII, IX and X cranial nerves become involved. Peripheral neuropathy of the extremities remained mild until late of life. Microscopy of skin biopsy samples showed deposits of amyloid around the eccrine glands, sebaceous glands, epidermal-dermal junction and blood vessel walls. Immunohistochemistry of the skin revealed the immunopositive material against a monoclonal antibody to gelsolin in the amyloid deposits. Molecular analysis of the gelsolin gene is now in progress.

Adult↗
PubMed1992

[Gene analysis of Japanese patients with familial amyloidotic polyneuropathy type IV].

Familial amyloidotic polyneuropathy type IV (FAP IV) is clinically characterized by slowly progressive cranial neuropathy and corneal lattice dystrophy. More than 300 cases were clustered in the Finnish population. Recent biochemical studies have demonstrated that the amyloid fibril protein in FAP IV is related to Asn-187 variant gelsolin, and the corresponding missense mutation, a G to A substitution at nucleotide 654 of plasma gelsolin cDNA, cosegregates with the disease phenotype in Finnish families. Here we analyzed the gelsolin gene of the Japanese family with FAP IV which we described as the first Japanese case. Direct sequence analysis of PCR-amplified DNA fragments spanning the codon 187 of plasma gelsolin cDNA from the 2 affected family members demonstrated a single base substitution, G to A at nucleotide 654, which is identical to the mutation of Finnish FAP IV. Restriction analysis using a modified PCR revealed that three unaffected family members and three unrelated healthy controls were homozygous for the normal allele, whereas the seven affected family members were heterozygous for the normal and the mutated alleles. This indicates the cosegregation of the mutation with the disease phenotype in this Japanese family, suggesting that the mutation causes the FAP IV phenotype regardless of ethnic background.

Alleles↗
PubMed1992

[Wallenberg's syndrome due to vertebral artery dissection following minimal neck injury--report of two cases].

We described two cases of the lateral medullary syndrome (Wallenberg's syndrome) due to vertebral artery dissection following minimal neck injuries. The first case was a 45-year-old man, who hit his head and often rotated his head because of posterior neck discomfort. Two years after the injury, he suffered from sudden sharp neck pain, nausea, and vertigo, which was followed by left hand numbness and difficulty in walking due to the right lateral medullary syndrome. Angiography showed right vertebral artery dissection at the fourth segment. The second case, a 48-year-old man, suffered from neck pain immediately after he hyperextended his neck for painting a wall. Within several hours, he experienced left hand numbness and difficulty in walking due to the lateral medullary syndrome. Angiography showed a saccular aneurysm and dissection of the right vertebral artery at the fourth segment. In both cases, minor traumas were thought to be the causes of vertebral artery dissection. We surveyed previously reported 84 cases (men: 50, women: 34) of the vertebral artery dissection due to minor traumas. Seventy per cent of patients were in their third or fourth decade of life. The main causes of trauma preceding the dissection were neck manipulation especially chiropractics (52%). The third segment was most vulnerable. Delay in onset following neck trauma could be more than a week, but in most cases the delay was less than 24 hours. Cervical rotation and extension were thought to precipitate dissection.

Aortic Dissection↗
PubMed1992

[An autopsy case of Degos disease with neurological symptoms--neuropathological observations and increased platelet aggregation].

We reported clinical and neuropathological observations of a 41-year-old man with Degos disease. He first noted painless skin lesions over the upper extremities in January, 1982. Three years later he was diagnosed as Degos disease by skin biopsy, and treatment with aspirin was started. In September, 1985, he complained of paresthesia on his right arm, followed by a series of new neurological manifestations suggesting multifocal spinal cord lesions. On October 28, examination of admission showed papules with central umblication over the whole body except the head, face, palms, soles and scrotum. Neurological examination revealed no weakness, diminished right biceps reflex, exaggerated patellar reflexes and Achilles reflexes, left extensor plantar reflex, hypesthesia and hypalgesia to the level of Th8, mild left spastic gait, and retention of urine. In November, he had paraparesis, loss of vibration sense of lower extremities, hypesthesia and hypalgesia to the level of TH4, and weakness of right upper extremity. In December, he showed tetraplegia, left-sided facial palsy, and hypesthesia and hypalgesia to the level of C5. In January, 1986, he showed right facial palsy, left facial hypesthesia, pseudobulbar palsy. In February, he had bilateral abducens nerve palsy and hiccups. On February 18, he died of intracranial hemorrhages. He had episodic abdominal pain several times during admission. His condition deteriorated progressively in four months after the first manifestation of neurological symptoms, despite the therapy with heparin, urokinase, ticlopidine, dipyridamole, and prednisolone. Laboratory studies showed gradual increase of CSF proteins (from 156 mg/dl to 602 mg/dl) and extremely increased platelet aggregation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

Fucosylated glycoconjugates in human dorsal root ganglion cells with unmyelinated axons.

The same subset of small neurons in human dorsal root ganglia (DRG) was recognized by both of the two probes binding to fucosylated residue. Ulex europaeus agglutinin I (UEA-1) lectin and anti-fucosyl GM1 antibody, although these probes bind to different glycoconjugates. UEA-1 lectin also bound to unmyelinated axons in DRG and in biopsied sural nerve, but not to any neurons or unmyelinated axons in the sympathetic ganglia. Thus UEA-1 lectin and anti-fucosyl GM1 antibody may be used as specific probes for primary sensory neurons with unmyelinated axons.

Antibodies↗