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Background: We evaluated whether the Walkaide® device could effectively improve walking ability and lower extremity function in post-stroke patients with foot drop. Patients aged 20–85 years with an initial stroke within ≤6 months and a functional ambulation classification score of 3 or 4 were eligible. Materials and Methods: Patients were randomly allocated to the functional electrical stimulation (FES) or control group at a 1:1 ratio. A 40 min training program using Walkaide was additionally performed by the FES group five times per week for 8 weeks. The control group received the 40 min training program without FES. Results: A total of 203 patients were allocated to the FES (n = 102) or control (n = 101) groups. Patients who did not receive the intervention or whose data were unavailable were excluded. Finally, the primary outcome data of 184 patients (n = 92 in each group) were analyzed. The mean change in the maximum distance during the 6-MWT (primary outcome) was 68.37 ± 62.42 m and 57.50 ± 68.17 m in the FES and control groups (difference: 10.86 m; 95% confidence interval: −8.26 to 29.98, p = 0.26), respectively. Conclusions: In Japanese post-stroke patients with foot drop, FES did not significantly improve the 6 min walk distance during the convalescent phase. The trial was registered at UMIN000020604.

A 47-year-old right-handed man was admitted to our hospital for rehabilitation after right basal ganglion hematoma. On day 57, he noticed a supernumerary motor phantom limb (SPL) involving his right arm, originating at the level of the elbow. The most notable finding of his SPL was the motor characteristic. When the subject had the intention to move the upper paralyzed limb simultaneously with the trainer’s facilitating action, he said “there is another arm.” The intention to move the paralyzed arm alone or passive movement of the paralyzed arm did not induce the SPL. He showed a severe left sensorimotor impairment and mild hemineglect, but no neglect syndromes of the body (e.g., asomatognosia, somatoparaphrenia, personification and misoplegia, or anosognosia) were observed. Brain MRI demonstrated a hematoma in the right temporal lobe subcortex, subfrontal cortex, putamen, internal capsule, and thalamus. Single-photon emission computed tomography images showed more widespread hypoperfusion in the right hemisphere in comparison to the lesions on MRI. However, the premotor cortex was preserved. Our case is different from Staub’s case in that SPL was not induced by the intention to move the paralyzed limb alone; rather, it was induced when the patient intended to move the paralyzed limb with a trainer’s simultaneous facilitating action. The SPL may reflect that an abnormal closed-loop function of the thalamocortical system underlies the phantom phenomenon. However, despite the severe motor and sensory impairment, the afferent pathway from the periphery to the premotor cortex may have been partially preserved, and this may have been related to the induction of SPL.

Background and Purpose— Our aim was to study the efficacy of robotic therapy as an adjuvant to standard therapy during poststroke rehabilitation. Methods— Prospective, open, blinded end point, randomized, multicenter exploratory clinical trial in Japan of 60 individuals with mild to moderate hemiplegia 4 to 8 weeks post stroke randomized to receive standard therapy plus 40 minutes of either robotic or self-guided therapy for 6 weeks (7 days/week). Upper extremity impairment before and after intervention was measured using the Fugl–Meyer assessment, Wolf Motor Function Test, and Motor Activity Log. Results— Robotic therapy significantly improved Fugl–Meyer assessment flexor synergy (2.1±2.7 versus −0.1±2.4; P P P Conclusions— Robotic therapy as an adjuvant to standard rehabilitation may improve upper extremity recovery in moderately impaired poststroke patients. Results of this exploratory study should be interpreted with caution. Clinical Trial Registration— URL: http://www.umin.ac.jp/ . Unique identifier: UMIN000001619.

A 66-year-old, right-handed male, was admitted to our hospital with difficulty in recognizing faces and colors. He had suffered a stroke in the right occipital region three years earlier that had induced left homonymous hemianopsia, but not prosopagnosia. A neurological examination revealed prosopagnosia, color agnosia, constructional apraxia, and topographical disorientation, but not either hemineglect or dressing apraxia. The patient was unable to distinguish faces of familiar persons such as his family and friends, as well as those of unfamiliar persons such as doctors and nurses. Brain MRI demonstrated an old infarction in the right medial occipital lobe and a new hemorrhagic infarction in the left medial occipital lobe, including the fusiform and lingual gyrus. It is unclear whether a purely right medial occipital lesion can be responsible for prosopagnosia, or whether bilateral medial occipital lesions are necessary for this occurrence. The current case indicated that bilateral medial occipital lesions play an important role in inducing porsopagnosia.

The origin of patchy white matter hyperintensities commonly seen in the elderly on magnetic resonance (MR) images with long repetition time (TR) is still controversial. We describe MR findings in older patients in whom white matter hyperintensities were attenuated by compression of the cerebral hemisphere from a chronic subdural hematoma. These sequential MR findings substantiate the hypothesis that leukoaraiosis may arise when drainage of the bulk flow of brain interstitial fluid is disturbed.

Bunina bodies (BBs) are small eosinophilic neuronal cytoplasmic inclusions (NCIs) found in the remaining lower motor neurons (LMNs) of patients with sporadic amyotrophic lateral sclerosis (SALS), being a specific feature of the cellular pathology. We examined a case of SALS, unassociated with TDP-43 or C9ORF72 mutation, of 12 years duration in a 75-year-old man, who had received artificial respiratory support for 9 years, and showed widespread multisystem degeneration with TDP-43 pathology. Interestingly, in this patient, many NCIs reminiscent of BBs were observed in the oculomotor nucleus, medullary reticular formation and cerebellar dentate nucleus. As BBs in the cerebellar dentate nucleus have not been previously described, we performed ultrastructural and immunohistochemical studies of these NCIs to gain further insight into the nature of BBs. In each region, the ultrastructural features of these NCIs were shown to be identical to those of BBs previously described in LMNs. These three regions and the relatively well preserved sacral anterior horns (S1 and S2) and facial motor nucleus were immunostained with antibodies against cystatin C (CC) and TDP-43. Importantly, it was revealed that BBs exhibiting immunoreactivity for CC were a feature of LMNs, but not of non-motor neurons, and that in the cerebellar dentate nucleus, the ratio of neurons with BBs and TDP-43 inclusions/neurons with BBs was significantly lower than in other regions. These findings suggest that the occurrence of BBs with CC immunoreactivity is intrinsically associated with the particular cellular properties of LMNs, and that the mechanism responsible for the formation of BBs is distinct from that for TDP-43 inclusions.

Bunina bodies (BBs) are small eosinophilic neuronal cytoplasmic inclusions (NCIs) found in the remaining lower motor neurons (LMNs) of patients with sporadic amyotrophic lateral sclerosis (SALS), being a specific feature of the cellular pathology. We examined a case of SALS, unassociated with TDP-43 or C9ORF72 mutation, of 12 years duration in a 75-year-old man, who had received artificial respiratory support for 9 years, and showed widespread multisystem degeneration with TDP-43 pathology. Interestingly, in this patient, many NCIs reminiscent of BBs were observed in the oculomotor nucleus, medullary reticular formation and cerebellar dentate nucleus. As BBs in the cerebellar dentate nucleus have not been previously described, we performed ultrastructural and immunohistochemical studies of these NCIs to gain further insight into the nature of BBs. In each region, the ultrastructural features of these NCIs were shown to be identical to those of BBs previously described in LMNs. These three regions and the relatively well preserved sacral anterior horns (S1 and S2) and facial motor nucleus were immunostained with antibodies against cystatin C (CC) and TDP-43. Importantly, it was revealed that BBs exhibiting immunoreactivity for CC were a feature of LMNs, but not of non-motor neurons, and that in the cerebellar dentate nucleus, the ratio of neurons with BBs and TDP-43 inclusions/neurons with BBs was significantly lower than in other regions. These findings suggest that the occurrence of BBs with CC immunoreactivity is intrinsically associated with the particular cellular properties of LMNs, and that the mechanism responsible for the formation of BBs is distinct from that for TDP-43 inclusions.

Neonatal rats born of and nursed by mothers immunized with Narke japonica acetylcholine receptor protein had elevated serum anti-acetylcholine receptor antibodies that reached the mother's level on day 10 after delivery and decreased rapidly after weaning. IgG was present at the motor end-plates up to day 170, and the motor end-plate fine structure remained abnormal up to day 80. Miniature end-plate potential amplitudes in the diaphragm were at the control levels within 10 days of birth, but were lower than those of the controls up to day 80 after birth. We could not obtain the direct evidence that transient synthesis of antibodies occurs in experimental autoimmune myasthenia gravis pups. This model can serve as an experimental model of transient neonatal myasthenia gravis in humans, exception for the route of antibody transfer and the time of the onset of illness.

The new serological tests for candidemia were evaluated. In nine cases of candidemia, fungal index (Endotoxin D-Endospecy) was 146.8 +/- 116.8 pg/ml and D-arabinitol/creatinine 1.10 +/- 1.04 mumol/mg. Fungal index was significantly (p less than 0.01) higher in candidemia than suspected infections. All candidemia were positive for Cand-Tec latex agglutination, but twelve of the sixteen cases of suspected infections were also positive. Mannan latex agglutination was very specific for candidemia. Four cases of candidemia was positive for Mannan latex agglutination and only one case was positive in suspected infections. Fungal index and D-arabinitol/creatinine was decreased after treatment of antifungal agents. In poor clinical efficacy case, these titer remained high even after treatment. These data suggested the usefulness of these serological tests for candidemia.

A 76-year-old man who rapidly developed quadriparesis was admitted to our hospital. MRI showed an epidural mass extending from C4 to C6, displacing the spinal cord anteriorly. It showed isointensity on the T1-weighted imagines, hyperintensity on the T2-weighted images, and diffuse hyperintensity with gadolinium enhancement. Plain radiographs, CT and MRI showed no evidence of bone involvement. Serum immunoelectrophoresis disclosed M-components of IgA and lambda light chains. This is the first report that an epidural myeloma in the cervical spinal cord caused compression of the cord without evidence of bone involvement.

To evaluate the clinical characteristics of Machado-Joseph disease (MJD) with reference to CAG repeat length and disease duration, we analyzed neurologic findings in 108 patients from 84 families. The majority of MJD patients presented with an ataxic gait as the initial symptom. Dysarthria and nystagmus were observed from an early stage. Bulging eyes, muscle atrophy and bradykinesia developed later. Patients with a shorter CAG repeat length or later onset had more frequent involvement of proprioceptive sensory deficit. Incidence of abnormal reflexes, tones, and proprioceptive sensation was not associated with disease duration, but with CAG repeat length. Based on these results, we propose a new clinical classification: type A (juvenile type), with hyperreflexia and dystonia, but without a proprioceptive sensory deficit; type C (adult type), with hyporeflexia and a proprioceptive sensory deficit, but without dystonia; and type B (intermediate type), the remaining patients with a mixed presentation.

Machado--Joseph disease (MJD) is an autosomal dominant spinocerebellar degeneration mapped to chromosome 14q32.1. The CAG expansions of the MJD1 gene was identified as the cause of the disease. We have analyzed 90 MJD individuals from 62 independent MJD families and found that the MJD1 repeat length is inversely correlated with the age of onset (r = -0.87). The MJD chromosomes contained 61-84 repeat units, whereas normal chromosomes displayed 14-34 repeats. In the normal chromosomes, 14 repeat units were the most common and the shortest. In association with the clinical anticipation of the disease, a parent--child analysis showed the unidirectional expansion of CAG repeats and no case of diminution in the affected family. The differences in CAG repeat length between parent and child and between siblings are greater in paternal transmission than in maternal transmission. Detailed analysis revealed that a large degree of expansion was associated with a shorter length of MJD1 gene in paternal transmission. On the other hand, the increments of increase were similar for shorter and longer expansion in maternal transmission. Among the three clinical subtypes, type I of MJD, with dystonia, showed a larger degree of expansion in CAG repeats of the gene and younger ages of onset than the other types.

The relationship between arteriosclerosis and osteoporosis (OP) was assessed in 20 elderly women. The aortic pulse wave velocity (PWV) was used as the index of arteriosclerosis and bone mineral content (BM) of the vertebral body, which was measured by quantitative computed tomography, was used as an index of OP. PWV and BM showed a significant correlation with aging (PWV and age: r = 0.466, p less than 0.05, BM and age: r = -0.487, p less than 0.05). In the group in which the serum alkaline phosphatase (ALP) was higher than 180 IU/l (n = 11), there was a significant inverse correlation between PWV and BM (r = -0.728, p less than 0.02). On the other hand, there was no correlation between PWV and BM in the group in which ALP was lower than 180 IU/l (n = 9). These results suggest that in cases with OP and high levels of ALP, calcium (Ca) seems to be released from the bone system and transferred to the wall of the aorta. The mechanism and pathogenesis of this Ca transference is unknown and should be investigated.

Creutzfeldt-Jakob disease (CJD) is a subacute spongiform encephalopathy characterized clinically by progressive dementia with myoclonus and extrapyramidal symptoms. If no characteristic EEG findings are found, clinical diagnosis is difficult. CT is of little use. MRI has been reported both to be of little value1 and to be helpful in the diagnosis of CJD.2-4 A few CJD patients with a point mutation at codon 232 of prion protein gene have been reported.5-7 We describe MRI changes in the cerebral cortex of a patient with a mutation at codon 232. Case report. A 64-year-old woman who had about a year's history of difficulty in dressing was admitted to our hospital. At that time, she had developed abnormalities in her cortical functions (dressing apraxia, constructional apraxia, limb-kinetic apraxia, idiomotor apraxia, ideational apraxia, and apraxic agraphia). She showed no disturbance of recent or remote memory. Her past medical and family histories were unremarkable, and she …

Two novel coordination polymer compounds, [Cu(DMTPN)2]X(DMTPN)(thf) (DMTPN = 2,5-dimethylterephthalonitrile, X = BF4 (l) and ClO4 (2)) are prepared and characterized by single crystal X-ray analysis; both are isostructural and have three-fold interpenetrated diamondoid structure with π–π stacking column of alternate coordinated and uncoordinated DMTPN.

Article abstract Heart tissues of patients with PD or incidental Lewy body (LB) disease (ILBD) were examined by light and electron microscopy. LBs and α-synuclein–positive neurites were identified in the hearts from 9 of 11 patients with PD and from 7 of 7 patients with ILBD. LBs were present in both tyrosine hydroxylase-positive and -negative nerve processes, which are nerves of extrinsic sympathetic and intrinsic origin, respectively. These findings provide histologic evidence that the postganglionic sympathetic and intrinsic neurons in the heart are involved in the PD disease process.

To clarify the role of Ia antigen positive macrophages which invade motor end-plates in the induction of the chronic phase, experimental autoimmune myasthenia gravis (EAMG) rats were injected intraperitoneally with silica dust on day 6 after immunization. Silica injection partially inhibited the invasion of motor end-plates with macrophages as compared with a saline injection. The titer of antibodies to the Narke acetylcholine receptor (AChR) in the chronic phase did not differ in either the saline or the silica groups, while the titer of antibodies to rat-AChR (non-crossreactive antibodies) was lower in the silica group than in the saline group. The silica group survived longer than the saline group. These results suggest that Ia antigen positive macrophages in the acute phase act as antigen-presenting cells and play an important role in the production of antibodies to self-AChR in the chronic phase.

症例. 27才,女性. 11才初潮, 16才より11年間無月経. 25才眼瞼下垂,複視出現.テンシロンテスト陽性,尺骨神経反復刺激でwaning現象,血中抗アセチルコリン受容体抗体価の上昇,筋生検所見より重症筋無力症と診断.一方, luteinizing hormone, follicular stimulating hormoneの著明な上昇, estradiolの低値, 1週間連続のhuman menopausal gonadotropin負荷に対する無反応よりhypergonadotropic hypogonadismと診断された.血清中の抗卵巣抗体,抗透明帯抗体は陰性.卵巣生検では,卵胞は全くなかった.両者の合併例の報告は過去11例とまれであり,両者が共通の機序により引き起こされているという確固たる証拠は現在のところ得られていない.

SETO, M., MOTOMURA, M., TAKEO, G., YOSHIMURA, T., TSUJIHATA, M. and NAGATAKI, S. Treatment of Myasthenia Gravis: A Comparison of the Natural Course and Current Therapies. Tohoku J. Exp. Med., 1993, 169 (1), 77-86 - Ninety-five patients with generalized myasthenia gravis were followed for 10 years to evaluate the long term effects of prednisolone, thymectomy, or both, and they were compared with a group only with anticholinesterase treatment. Only 15.0% of the patients with anticholinesterase alone had showed improvement 10 years after the onset, but more than 60% of those treated with prednisolone, thymectomy, or both showed improvement. Especially thymectomy induced complete remissions at 10 years after surgery in more than 20% of the patients. There was no difference between the histology of the thymi and clinical severity, or anti-acetylcholine receptor (AChR) antibody titer. In patients who showed improvement classified “good” of higher 10 years after thymectomy with or without prednisolone, anti-AChR antibody titers swiftly decreased to 37.8% of the value before surgery, and remained low thereafter. This result suggests that the marked decrease in anti-AChR antibody titers within 1 month after thymectomy is a favorable prognostic sign in myasthenia gravis patients who have undergone thymectomy. - myasthenia gravis; prognosis; anti-AChR antibodies; thymectomy; prednisolone

小出血を繰り返し,手掌口症候群,視性幻覚, MLF症候群,外転神経麻痺,小脳失調症など多彩な神経症状を呈した特発性橋出血の33才,男性例を報告した.最初左手掌口症候群,右MLF症候群,右外転神経麻痺が出現, CT上橋被蓋部に出血を認めた.血腫の縮小と平行して症状は改善したが,再度橋出血が増大するとともに,左MLF症候群,左外転神経麻痺,左顔面神経麻痺,視性幻覚が出現し,血腫の再縮小とともに,これらの症状は消退した.血管造影上は, AVM, aneurysmなどは証明されなかった.限局した突発性橋出血を繰り返したために,意識障害なしに,種々の神経症状の出現をみたものと考えた.