Biomedical subjects

T Ibi

Publications and source records attributed to T Ibi.

At least 55 records · Page 3Linked to original sources

PubMed1991

[HTLV-I associated myelopathy (HAM) and sarcoid myopathy].

A 65-year-old house-wife developed dirty erythematous rash on her face in April, 1989. Almost simultaneously, she complained of muscle soreness and weakness on both lower extremities. Pathological findings of the skin biopsy at that time was consistent with those of sarcoidosis with moderate inflammatory cell infiltration. In December, 1989, when she was admitted to our hospital, her lower extremities were paretic with marked spasticity, and mild bladder dysfunction was noted. HTLV-I antibody titers in serum and cerebrospinal fluid were significantly elevated. Biopsied limb skeletal muscle revealed the findings of the sarcoid myopathy with small inflammatory cell infiltration in endomysium. HLA haplotypes showed A24, B7, BW61, CW7, CW8, DR1 and DR4 which show relatively common types of those in HAM. Corticosteroid treatments including the methylprednisolone pulse therapy healed the skin lesion, but did not improve her neurological signs. Paraplegia and urinary disturbance were progressive. It is concluded that the inflammatory sarcoid myopathy with HAM in this patient may be caused by a common abnormal immunological background.

Aged↗

PubMed1991

[Myoglobinuria caused by multiple deletions of mitochondrial DNA].

We report two brothers with inherited recurrent myoglobinuria associated with distinct morphological abnormalities of muscle mitochondria and multiple deletions of muscle mitochondrial DNA. Patient 1 (26 years old) and Patient 2 (21 years old) had recurrent episodes of myoglobinuria provoked by strenuous exercise or alcohol intake. Histochemistry of their biopsied limb muscles showed ragged-red fibers and cytochrome c oxidase-negative fibers as well as degenerating and regenerating fibers. Electron microscopy showed a pronounced accumulation of abnormal mitochondria containing paracrystalline inclusions and moderate increases of glycogen particles. Southern blot analysis revealed multiple deletions of mitochondrial DNA, some of which were common to both patients. By the primer shift polymerase chain reaction method, we detected multiple abnormal fragments indicating mitochondrial DNA deletions. Nucleotide sequencing of the deleted regions disclosed directly repeated sequences of 1 to 12 bp on each side of the deletions. Since the end points of mitochondrial DNA deletions were within 20 bp of the major non-coding region, probable mutations in this region contribute to the pathogenesis of multiple mitochondrial DNA deletions found in these patients. We propose that a defect of the mitochondrial energy-transducing system due to multiple mitochondrial DNA deletions is a novel genetic cause of inherited recurrent myoglobinuria.

Base Sequence↗

PubMed1990

Cytoplasmic body and mitochondrial DNA deletion.

A patient with chronic progressive external ophthalmoplegia (CPEO) who had abundant cytoplasmic bodies in muscle fibers and a deletion of mitochondrial DNA is reported. The patient was a 26-year-old male suffering from ophthalmoplegia from age 21. He had a marfanoid skeletal abnormality and perceptive hearing loss, but had neither retinopathy, ataxia, nor dementia. In the mitochondria isolated from the biopsied skeletal muscle, NADH-ubiquinone oxidoreductase activity was slightly decreased, succinate-cytochrome c reductase activity was slightly increased, and cytochrome c oxidase activity remained normal. Southern blot analysis of the muscle DNA identified heteroplasmy composed of a normal-sized mitochondrial DNA and a mutant mitochondrial DNA with a 4.2-kilobase deletion. The PCR plus S1 analysis showed that the deletion extended from nucleotide position 7860 +/- 60 to 12,090 +/- 70. The histological studies of the biopsied muscle revealed ragged-red fibers and cytochrome c oxidase-negative fibers in 15.7% and 18.6% of the muscle fibers, respectively. Other conspicuous histological change was abundant cytoplasmic bodies surrounded by clusters of abnormal mitochondria. The cytoplasmic bodies were found preferentially in type 1 fibers, and exclusively in cytochrome c oxidase-negative fibers and in ragged-red fibers. Focal existence of cytoplasmic bodies in muscle fibers with abnormal mitochondria suggests that segregated distribution of the abnormal mitochondria with deleted mitochondrial DNA is involved in the pathogenesis of cytoplasmic bodies.

Adult↗

PubMed1990

[Computed tomographic analyses on skeletal muscles in bulbar spinal muscular atrophy].

We analysed the patterns of skeletal muscular involvement in 18 patients with bulbar-spinal muscular atrophy (BSMA) of the Kennedy-Alter-Sung type by using the computed tomographic scanner. Fatty infiltrations were prominent in various skeletal muscles of extremities and trunk, and its degree was severe in the following numerical orders; the gluteal muscles, flexors of the thighs, flexors of the lower extremities, extensors and the adductors of the thighs, the paraspinal muscles, and extensors of the lower extremities. There was statistically significant correlation between fatty infiltrations in flexors of the lower extremities and duration of illness. And were noted findings that the skeletal muscle lesion progressed with the preserved fasciae and sectional areas, fatty infiltrations in the lower extremities were more conspicuous in flexors than in extensors, and compensatory hypertrophic muscles in the thigh were apparent in 50% of cases. In conclusions, the computed tomographic analyses on skeletal muscle of BSMA may be useful to detect distributions, progression of the muscle lesion, in addition to a profile of the myopathic alterations of the disease.

Adult↗

PubMed1990

[Myasthenia gravis associated with tonic pupil].

A 41 year-old female patient with acquired autoimmune myasthenia gravis (MG, IIa type) and tonic pupil (Adie's pupil) of the right side was reported. Adie's pupil was pointed out at the same time of the clinical diagnosis of MG in September, 1988. She had the solitary thymoma identified by pneumo-mediastinograph and mediastinal CT scan. Results of the positive anti-nicotinic AChR antibody titer, the positive edrophonium test and the electrophysiological examination gave unambiguous evidences of acquired autoimmune MG with thymoma. Instillations to the eyes of cocaine, pilocarpine and epinephrine, and the pupillary response to the intravenous injection of edrophonium revealed the postganglionic abnormality mainly in the parasympathetic system. There were no other overt symptoms due to dysautonomia. She refused thymectomy. Her myasthenic and pupillary signs became gradually improving without any immunosuppressive medications. It is concluded that two diseases of the patient may be probably caused by multiple organ- or tissue-specific autoantibodies, in addition to pathogenetic significance of Adie's pupil.

Adie Syndrome↗

PubMed1989

[Clinical features of the peripheral nerve involvement in necrotizing angitis--characteristics in polyarteritis nodosa and allergic granulomatous angitis].

Thirteen patients with peripheral neuropathy caused by necrotizing vasculitis were clinico-pathologically analyzed. These patients consisted of nine classical periarteritis nodosa (PN), four allergic granulomatous angitis (Churg-Strauss syndrome, AGA). All of them were proven to have a necrotizing vasculitis by sural nerve biopsy. The characteristics of peripheral neuropathy of these patients were summarized as follows. 1) Mononeuritis multiplex was a principal features in all patients preferentially localized in common peroneal, sural, radial median and ulnar nerves, with all modality of sensory impairment. 2) Radiation or diffuse deep-pain was a major initial symptom. Since this pain occurs frequently in the manner of sudden onset, the patient can tell the day of onset. 3) Local edema on the skin of involved region was initially observed. 4) Muscular atrophy and weakness was distributed more widely than sensory impairment. 5) Morphometric and teased-fiber study of biopsied sural nerves revealed axonal degeneration as a major pathological process. As compared to myelinated fibers, unmyelinated fibers were likely to be well preserved in morphology and population, which suggests that unmyelinated fibers are relatively resistant to ischemia. 6) Motor and sensory conduction study showed greatly decreased sensory and motor action potentials frequently resulting in absent of recordings. Conduction velocity is almost within normal range or just below the normal. Routine EMG recordings showed active denervation potentials in the involved muscles. 7) Protein in CSF was rarely elevated which suggested involvement of the spinal roots is infrequent. 8) Hypereosinophilia, thrombocythemia, fever, increased erythrocyte sedimentation rate, positive CRP and RA, and polyclonal hypergammaglobulinemia (IgG, IgA) were observed in most cases.

Adult↗

PubMed1989

[Progressive hemifacial atrophy with sympathetic nerve dysfunction of central origin].

A 37-year-old unmarried man was admitted because of gait disturbance and right hemifacial atrophy. Family history was unremarkable. He had an unconscious attack at age 13 and had writer's cramp since age 15. He was thin and lipodystrophic. In reviewing his portraits, hemifacial atrophy was considered to develop in his early teens and to be progressive since then. Pigmented gum, high arched palate, mild mental retardation, pseudo-Argyll Robertson's pupil, sexual impotence, amyotrophy of the left thigh and the right calf, and a limp due to bony abnormalities was detected. Serological tests for syphilis were negative. Bone X-rays disclosed coxa-deformance. Cerebrospinal fluid. EMG, EEG, muscle biopsy and brain CT were normal. Hearing was decreased to 20-35 dB bilaterally. Plasma norepinephrine levels were 450 pg/ml in the supine position and 539 pg/ml in standing. Plasma renin activity was 5.1-5.4 ng/ml/hr. Microneurography revealed highly accentuated muscle and skin sympathetic nerve activities. Hypothermia on the feet, reduced CVR-R and decreased mydriatic response to 5% cocaine instillation were present. Intravenous infusion of norepinephrine and intradermal injection of either acetylcholine or histamine revealed normal results. In the case, sympathicotonia due to dysfunction in the central nervous system is considered to be related to the pathogenesis of hemifacial atrophy.

Adult↗

PubMed1989

[Superior oblique myokymia--a case report and pathogenetic consideration].

A 30-year-old male visited us with complaints of dizziness and diplopia of abrupt onset in September, 1988. Neurological studies demonstrated paroxysmal rotatory to-and-fro oscillations of the left eye. There has been the same clinical episode at 25 years of age, lasting approximately 6 months. The characteristics of his ocular involuntary movement with the mild IVth cranial nerve paresis were as follows: quick, nonrhythmic, initially counterclockwise-rolling, more prominent in moving the left eye to the lower medial side, persisting for 1-10 seconds and rapidly repeated oscillations. When oscillations were prominent, he complained of faintness in addition to double vision. Except for the ocular signs, other neurological and laboratory examinations including cerebrospinal fluid, brain MRI and brain stem auditory evoked response, failed to disclose the precise location and nature of the lesion. Abnormal ocular movement was fluctuating for 4 months and gradually disappeared without any medication. In conclusion, the faintness could be considered to result from a lesion of reticular activating system adjacent to the IVth cranial nerve nucleus and its rostral (excitatory or inhibitory) supranuclei, and it suggests that a responsible lesion of the abnormal ocular movement is located at a region of the dorsomedial midbrain.

Adult↗

PubMed1989

[HTLV-I associated encephalo-myelopathy resembling ALS with concurrence of acute promyelocytic leukemia in a member of the relatives].

A 36 year-old woman beginning with spastic paraparesis at her age of 11 visited us for evaluation of progressive muscular weakness of the distal portions of the upper extremities and difficulty in speaking at her age of 33. The neurological features at the present are as follows; fine horizontal gaze-nystagmus, impaired smooth pursuit ocular movement, highly spastic paraplegia with pes equino-varus necessitating canes and the wheel-chair, highly accentuated PTRs and ATRs associated with positive Babinski's sign, diminished or absent deep reflexes in the upper extremities, moderate muscular wasting with fasciculation on the tongue and distal portions of the upper extremities (rt less than lt). Sensory or cerebellar functions remain normal. No autonomic finding has been manifested. The HTLV-I antibody titers of serum (eg. PA method: x8192 ) and cerebrospinal fluid are highly positive in various methods. That of her mother (no blood-transfusion history) is positive. The provirus genome analysis on peripheral lymphocytes using the Southern blotting method by the cleaving enzyme Psi I was unable to discriminate that of an ATL patient. MRI of the central nervous system revealed higher signal area (short SE) at the C5/6 region and atrophy of C7/8 region. Neither a definite lesion in the lower brain stem, cerebellum nor cerebral hemispheres was identified. The skeletal muscle X-ray CT on the lower extremities revealed the atrophic flexor thighs and the anterior tibial and peroneal muscles. Needle EMG showed the prominent neurogenic changes in the atrophic muscles. Oral prednisolone therapy for four months relieved nystagmus and difficulty in walking, slightly. She, however, discontinued because of its side effect.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1989

[Two cases of myasthenia gravis associated with transient amenorrhea].

Two cases of acquired autoimmune myasthenia gravis (MG) presenting transient amenorrhea were reported. Case 1, 28 years old, developed blepharoptosis and generalized fatigability at the age of 20 years. She had been treated only by anti-cholinesterase. Amenorrhea appeared at the age of 26 years. Then, physical examinations showed normal secondary sexual development and moderate myasthenic features. On laboratory examinations, SLE findings such as leucopenia (1,600/mm3), biologically false positivity in the serological tests for syphilis, negative Mantoux reaction, positive anti-nuclear and -DNA antibodies, were noted. Anti-AChR antibody was highly positive (max.: 353 nmol/l). Decreased E2 (13-15 pg/ml) and progesterone (0.21-0.29 ng/ml) values in serum, elevated LH (110-160 mIU/ml) and FSH (78-90 mIU/ml) and highly reactive LH-RH loading test were consistent with the hypergonadotropic hypogonadism. Thymus pathology of thymectomy which was done during amenorrhea, showed hyperplasia. Bilateral ovarian biopsy revealed a number of arrested primordial follicles, but neither inflammatory changes nor fibrosis. Immune complexes were not localized in the ovarian biopsy. The Kaufmann's therapy aggravated myasthenic symptoms. Menstruation recurred after 13 months of thymectomy. Amenorrhea continued for 18 months. Case 2, 37 years old, has had anti-epileptic regimens since the age of 4 years. She has been highly myasthenic for 15 years and treated by thymectomy, steroid hormone, plasmapheresis and some other therapies for 10 years. Amenorrhea occurred at the age of 34 years. Sexual development was normal. Myasthenia was very severe. On laboratory examinations, anti-AChR antibody was positive (max.: 941 nmol/l). Transient elevation of serum LH (37 mIU/l) and FSH (14 mIU/l) values was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1989

[Clinical analyses on moving toes in "painful legs and moving toes"].

Clinical analyses on moving toes were done through two cases of painful legs and moving toes, who had been ill for more than 5 years. Either cases had degenerated lower lumber to upper sacral spines. Clinico-physiological data suggested the presence of polyneuropathy of the sensory type. They showed the lowered skin temperature on the distal portions of lower extremities and acrocyanosis. They, in addition, showed an irregular respiration frequently associated with apnea episodes. Frequency and amplitude of moving toes were increased during Valsalva's maneuver or apnea. Further more, findings were obtained that both the muscle sympathetic nerve activity recorded microneurographically from the tibial nerve and the surface electromyographic activity on the extensor digitorum longs were increased well concomitantly by the occurrence of moving toes. The finding may relevant that moving toes, a type of dyskinesia, may be probably generated by a direct noxious stimulus to the peripheral sensory or autonomic fibers, being strongly influenced by the higher central autonomic (at brain stem?), the extrapyramidal (at corpus striatum?) or some other structures. Moving toes might be a compensatory mechanism to lessen the paucity of blood flow in the lower extremities.

Aged↗

PubMed1988