-
[show abstract]
[hide abstract]
ABSTRACT: Involvement of cranial nerves caused by cranial base lesions manifests diverse symptoms according to the localization of lesions. These localization-related symptoms are classified into syndromes such as orbital apex syndrome, cavernous sinus syndrome, and jugular foramen syndrome. Since the lesions may have various etiologies including infection, inflammation and tumor, etiological diagnosis should be performed simultaneously if possible. However, previous reports have described poor and/or fatal outcome following inappropriate treatment mainly due to difficulties in making a definitive pathological diagnosis.Nineteen patients with multiple cranial nerve involvement caused by skull base lesions were enrolled over the past 12 years. The patients were divided into an "infectious" group (n=11) and a "noninfectious" group (n=8) based on the final diagnosis. Chi-square analysis was conducted to examine the sensitivity and specificity of various factors including patient characteristics, clinical course and treatment response in infectious and noninfectious groups. Consequently, we identified some patients with good outcome irrespective of infectious or noninfectious etiology, even though a pathological diagnosis was not reached before the initial treatment. These patients with good outcome consistently received antifungal therapy in the early stage if infectious etiologies were suspected. We recommend conducting diagnostic therapy with antifungal drugs in patients with skull base lesions of unknown origin although infection cannot be excluded, when a pathological diagnosis is difficult due to various patient conditions.
Rinsho shinkeigaku = Clinical neurology. 01/2013; 53(1):9-18.
-
Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 10/2012; · 3.12 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Tacrolimus (FK506) is a macrolide T-cell immunomodulator used to treat myasthenia gravis (MG). Besides immunosuppression, tacrolimus has been reported to have the potential to increase muscle strength by enhancing ryanodine receptor (RyR) function. However, few attempts have been made to demonstrate the early effect of tacrolimus as an RyR enhancer in clinical investigation.
In 20 MG patients, masseteric compound muscle action potential (CMAP) and mandibular movement-related potentials (MRPs) were recorded simultaneously after stimulating the trigeminal motor nerve with a needle electrode. The excitation-contraction (E-C) coupling time (ECCT) was calculated by the latency difference between CMAP and MRP. Bite force was measured using a pressure-sensitive sheet. Serial assessments of % decrement in masseteric repetitive nerve stimulation (RNS), ECCT and bite force were performed before and within 4 weeks of tacrolimus (3 mg day(-1)) treatment. The median (mean, range) interval of assessment was 2 (2.4, 1-4) weeks. We also measured serum antibodies against RyR, acetylcholine receptor and muscle-specific receptor tyrosine kinase.
Bite force increased after tacrolimus treatment accompanying clinical improvement assessed by Myasthenia Gravis Foundation of America classification, but the bite force difference did not reach statistical significance. Wilcoxon matched-pairs signed-ranks test detected a significant ECCT shortening in 12 patients assessed after 1-2 weeks of tacrolimus treatment as well as in eight patients assessed after 3-4 weeks. In contrast, masseteric CMAP and % decrement showed no significant changes after short-term tacrolimus treatment.
Tacrolimus induces ECCT shortening accompanying clinical improvement despite no improvement in % decrement within 2 weeks.
This early effect of tacrolimus may imply a pharmacological enhancement of RyR function to improve E-C coupling in MG.
Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 02/2012; 123(9):1886-90. · 3.12 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study was to elucidate the relationship between the impairment of excitation-contraction (E-C) coupling and anti-ryanodine receptor (RyR) antibody in patients with myasthenia gravis (MG).
Masseteric compound muscle action potential (CMAP) and mandibular movement-related potentials (MRPs) were recorded simultaneously after stimulating the trigeminal motor nerve with a needle electrode. The E-C coupling time (ECCT) was calculated as the latency difference between CMAP and MRP. For each patient, we selected a representative data set when there was no abnormal decrement in response to repetitive nerve stimulation. The 26 data sets were divided into an anti-RyR-positive group (n=12) and an anti-RyR-negative group (n=14).
Masseteric ECCT was significantly longer (p=0.017) in anti-RyR-positive group (median, mean, range; 3.6, 3.8, 3.0-5.9 ms) than in anti-RyR-negative group (3.1, 3.1, 2.7-4.0) although there were no significant differences in masseteric CMAP amplitude and % decrement between the two groups. The bite force was significantly lower in anti-RyR-positive group than in normal controls.
Presence of anti-RyR antibodies is associated with significantly prolonged masseteric ECCT compared to absence of the antibodies in MG.
Anti-RyR antibody contributes to E-C coupling impairment in the masseter muscle in patients with MG.
Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 11/2011; 123(6):1242-7. · 3.12 Impact Factor
-
Norito Kokubun,
Masahiro Sonoo, Tomihiro Imai,
Yumiko Arimura,
Satoshi Kuwabara,
Tetsuo Komori,
Masahito Kobayashi,
Takahide Nagashima,
Yuki Hatanaka,
Emiko Tsuda,
Sonoko Misawa,
Tatsuya Abe,
Kimiyoshi Arimura
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study is to establish reference values for single-fibre electromyography (SFEMG) using concentric needles in a prospective, multicentre study.
Voluntary or stimulated SFEMG at the extensor digitorum communis (EDC) or frontalis (FRO) muscles was conducted in 56-63 of a total of 69 normal subjects below the age of 60years at six Japanese institutes. The cut-off values for mean consecutive difference (MCD) of individual potentials were calculated using +2.5 SD or 95% prediction limit (one-tail) of the upper 10th percentile MCD value for individual subjects.
The cut-off values for individual MCD (+2.5 SD) were 56.8μs for EDC-V (voluntary SFEMG for EDC), 58.8μs for EDC-S (stimulated SFEMG for EDC), 56.8μs for FRO-V (voluntary SFEMG for FRO) and 51.0μs for FRO-S (stimulated SFEMG for FRO). The false positive rates using these cut-off values were around 2%.
The +2.5 SD and 95% prediction limit might be two optimal cut-off values, depending on the clinical question. The obtained reference values were larger than those reported previously using concentric needles, but might better coincide with conventional values.
This is the first multicentre study reporting reference values for SFEMG using concentric needles. The way to determine cut-off values and the statistically correct definition of the percentile were discussed.
Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 08/2011; 123(3):613-20. · 3.12 Impact Factor
-
Meng Han,
Hirofumi Ohnishi,
Michio Nonaka,
Rika Yamauchi,
Takayoshi Hozuki,
Takashi Hayashi,
Masaki Saitoh,
Shin Hisahara, Tomihiro Imai,
Shun Shimohama,
Mitsuru Mori
[show abstract]
[hide abstract]
ABSTRACT: Aspiration pneumonia related to dysphagia is known to be the leading cause of death in patients with Parkinson's disease (PD). We investigated the relationship between depressive states and dysphagia in patients with PD. A hundred and twenty-seven PD patients gave their informed consent and were enrolled in this study. We used the Beck Depression Inventory (BDI) questionnaire to determine the participants' depressive states, and also used a questionnaire to assess participants' state of dysphagia. Participants were divided into four groups according to their BDI score. We compared the PD patients with Swallowing Disturbances Questionnaire (SDQ) scores of more than or equal to 11 with the SDQ scores of less than 11 regarding depressive categories. A logistic regression analysis was conducted to calculate odds ratios (ORs) and their 95% confidence intervals (95%CI) adjusting for age, sex, disease duration, wearing-off phenomenon and severity of movement disorder. OR (95%CI) of depressive categories, in which the trivial class was set as a reference group, were 3.28 (0.93-11.55), 13.44 (3.10-58.16), 30.35 (5.65-162.97) in the mild class, the moderate class and the severe class, respectively. This study suggests that there may be a strong relationship between depressive states and dysphagia in patients with PD.
Parkinsonism & Related Disorders 03/2011; 17(6):437-9. · 3.80 Impact Factor
-
Muscle & Nerve 02/2011; 43(2):294-5. · 2.37 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study was to elucidate the relationship between the impairment of excitation-contraction (E-C) coupling of masseter and the bite force in patients with myasthenia gravis (MG).
In 20 patients with MG, masseteric compound muscle action potential (CMAP) and mandibular movement-related potentials (MRP) were recorded simultaneously after stimulating the trigeminal motor nerve with a needle electrode. The E-C coupling time (ECCT) was calculated by the latency difference between CMAP and MRP. Bite force was measured using a pressure-sensitive sheet. Serial assessments of % decrement in masseteric repetitive nerve stimulation (RNS), ECCT, and bite force were performed before and after corticosteroid therapy alone or in various combinations with FK506, cyclosporin A, intravenous immunoglobulin and immunoabsorption.
Percent amplitude decrement in RNS and ECCT decreased significantly accompanying an increase in bite force after treatment. Simple regression analysis demonstrated a linear correlation among % decrement, ECCT and bite force. However, ECCT shortening accompanying bite force recovery without reduction in % decrement was observed in 4 patients.
Masseteric E-C coupling is impaired in some MG patients, and functional recovery of E-C coupling contributes at least in part to the increase in bite force after treatment.
Impaired E-C coupling contributes to muscle weakness in patients with MG.
Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 03/2010; 121(7):1051-8. · 3.12 Impact Factor
-
Takayoshi Hozuki, Tomihiro Imai,
Emiko Tsuda,
Akihiro Matsumura,
Daisuke Yamamoto,
Takanobu Toyoshima,
Syuuichiro Suzuki,
Rika Yamauchi,
Takashi Hayashi,
Shin Hisahara,
Shun Shimohama
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study was to investigate the responses of serum osteocalcin (OC), undercarboxylated osteocalcin (ucOC) and N-terminal telopeptide of type I collagen (NTx) to corticosteroids, and to examine the effects of risedronate therapy with or without vitamin K(2) supplementation on bone metabolic markers in corticosteroid-treated patients.
Sixteen patients on corticosteroid therapy for neuromuscular disorders were assigned randomly to 2 groups (A: risedronate monotherapy, n=8; B: combined risedronate and vitamin K(2) therapy, n=8) and treated for 1 year. Another 6 patients who received intravenous steroid pulse therapy were assigned to group C for investigation of the effects of corticosteroids on OC and ucOC 1 month after pulse therapy.
Serial measurements revealed that significant decreases of OC, ucOC and NTx persisted with a similar time course profile during 1 year of treatment in groups A and B, and between-group analysis failed to demonstrate any additional effects of vitamin K(2) on risedronate therapy. Intravenous steroid pulse therapy induced a transient depression of OC and ucOC within 1 week in group C.
These results indicate that serum concentrations of OC and ucOC become consistently low during corticosteroid administration despite risedronate therapy with or without vitamin K(2) supplementation, and the serum ucOC level may not be a reliable indicator of vitamin K status under corticosteroid administration.
Internal Medicine 01/2010; 49(5):371-6. · 0.94 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 40-year-old woman was admitted to our hospital because of double vision combined with left ptosis. Although at 25 years of age she had already been diagnosed with limb weakness associated with chronic inflammatory demyelinating polyneuropathy (CIDP), she had never experienced double vision until her latest condition. Neurological examination revealed left oculomotor palsy without other cranial nerve involvement. Serial magnetic resonance imaging (MRI) studies demonstrated a temporal correlation between clinical severity of oculomotor palsy and segmental enhancement of the oculomotor nerve. Gadolinium enhancement on MRI may be a significant finding indicating relapse of oculomotor involvement of CIDP.
Internal Medicine 01/2009; 48(22):1985-7. · 0.94 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 41-year-old man with progressive limb weakness manifested fluctuating muscle weakness as seen in myasthenia gravis (MG). Laboratory investigations revealed hyperthyroidism without the complication of MG. Electrophysiological studies demonstrated abnormal features of neuromuscular transmissions resembling those of the Lambert-Eaton myasthenic syndrome rather than those of MG. A CT scan showed a mediastinal mass that suggested thymic hyperplasia which often complicates MG or hyperthyroidism. Medical treatment of hyperthyroidism resulted in resolution of MG-like symptoms and regression of thymic hyperplasia on CT concomitant with normalization of thyroid function. This case highlights the fact that careful investigations are needed to differentiate MG-like symptoms from genuine MG in cases of hyperthyroidism with thymic lesions.
Internal Medicine 02/2008; 47(5):445-7. · 0.94 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Interferons (IFNs)-inducible myxovirus resistance protein A (MxA) has recently been used as an indirect marker of neutralizing antibody against IFN in patients with multiple sclerosis (MS). On the other hand, MxA inhibits the replication of viruses by means of modifying cellular function, including apoptotic pathway. Our objective is to investigate the genetic and pathological role of MxA in patients with MS.
We examined SNPs of MxA promoter region in 67 patients with MS. Moreover, to elucidate the functional roles of SNPs, we conducted Luciferase assay with pGL3-basic vector including patient-derived or artificially mutated MxA promoter region.
A significantly higher frequency of the haplotype with -88T and -123A, which correlates with over-expression of MxA, was observed in MS. Moreover, we elucidated novel findings showing that nt -88 played a leading part with type I IFNs and that nt -123 played the same role independently without type I IFNs, respectively.
SNPs on MxA promoter region may play an important role in the pathophysiology of MS and provide a novel strategy for the therapeutic resolutions of MS.
Journal of the Neurological Sciences 12/2006; 249(2):153-7. · 2.35 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We examined the usefulness of non-invasive positive pressure ventilation (NPPV) in the management of nocturnal laryngeal stridor associated with vocal cord dysfunction in five cases of multiple system atrophy (MSA). First, the patients were investigated during sleep induced by a minimal dose of propofol. Laryngoscopy showed paradoxical vocal cord movement resulting in inspiratory stridor. Electromyographic (EMG) study revealed synchronized bursts in the thyroarytenoid muscles and diaphragm in every inspiratory phase whenever the stridor emerged. NPPV was initiated after paradoxical movement was recognized with laryngoscopy. The NPPV mask was equipped with an additional channel for laryngoscopic monitoring. The optimal pressure for treatment was determined according to laryngoscopic and EMG findings. Next, NPPV was applied to natural sleep using the conditions determined in propofol-induced sleep. In all cases, NPPV eliminated nocturnal stridor and oxygen desaturation during natural sleep. Laryngoscopic observation during induced sleep is recommended as a useful procedure to titrate the optimal pressure for NPPV therapy. Since central hypoventilation progresses in the course of MSA, the choice of NPPV rather than continuous positive airway pressure should be encouraged to treat laryngeal contraction disorder associated with MSA.
Journal of the Neurological Sciences 09/2006; 247(1):53-8. · 2.35 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 49-year-old woman with amyotrophic sequelae of poliomyelitis experienced progressive left upper limb weakness and breathing discomfort while walking that had developed over one year prior to presentation. She had flaccid quadriplegia which was more marked in the left upper limb, with the C4- and C5-innervated muscles being most severely affected. Chest radiographs double exposed at maximal inspiration and expiration revealed poor respiratory movements in the left hemidiaphragm. Phrenic nerve conduction study demonstrated conspicuous diaphragmatic paralysis on the left side. The electrophysiological study supports radiological findings and provides useful evidence for insidious phrenic nerve involvement in postpolio syndrome.
Internal Medicine 02/2006; 45(8):563-4. · 0.94 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We elucidated the possible relationship between age and conduction parameters of phrenic nerve in subjects above the sixth decade, comparing with the data from middle-age controls.
Diaphragmatic action potentials (DAPs) were recorded on bilateral hemithoraces of 41 volunteers aged 60-101 years (old group) and 25 volunteers aged 35-55 years (middle-age group). Statistical analyses were performed to assess the effects of aging on latency, latency corrected by size (Lat/Dist), amplitude, and the right-left difference of these DAP parameters.
In all 61 subjects, age showed a significant quadratic correlation with latency and with Lat/Dist, and a linear correlation with amplitude. The right-left differences ranged from 0.0 to 14.5% for latency and from 6.5 to 112.4% for amplitude in the elderly.
The normal ranges of DAP parameters should be determined according to age. The left-right difference may be a useful reference in diagnosing unilateral phrenic nerve lesion.
The precise normal ranges of phrenic nerve conduction parameters presented will encourage investigations of neuropathies in subjects aged above 60.
Clinical Neurophysiology 12/2005; 116(11):2560-4. · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We conducted spinal MR imaging on a 35-year-old man with Lhermitte's sign that had manifested over the previous 4 years. He had consumed more than 500 ml of whisky daily for at least 10 years. However, he did not show any evidence of severe liver disease with hepato-systemic blood shunting. Neurologic examination revealed markedly depressed sense of vibration in the feet and mild spasticity in the lower limbs, together with Lhermitte's sign. MR imaging revealed abnormal signal intensity in the posterior column spanning the whole length of the upper cervical cord, which is consistent with Lhermitte's sign.
Internal Medicine 03/2005; 44(2):153-4. · 0.94 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study is to elucidate the significance of electrophysiological data of the palmar cutaneous branch of the median nerve (PCBm) in the diagnosis entrapment of the PCBm concomitant with carpal tunnel syndrome (CTS).
Clinical and electrophysiological studies were conducted for abnormal sensation of the thenar eminence in eight CTS patients. Sensory nerve action potentials (SNAPs) of the PCBm were recorded from bipolar surface electrodes over the forearm using an orthodromic method. The preoperative SNAP evaluation was compared with the operative findings.
The eight patients were divided into two groups based on the characteristics of SNAPs of the PCBm; five patients had normal SNAPs of the PCBm and three patients had delayed or absent SNAPs. Open surgery confirmed entrapment of the PCBm in one patient in the first group and all patients in the latter group.
Although clinical symptoms were not always reliable to diagnose entrapment of the PCBm in CTS, electrophysiological study of the PCBm was highly sensitive (75%) and entirely specific (100%) to demonstrate the entrapment of the PCBm preoperatively.
Clinical Neurophysiology 12/2004; 115(11):2514-7. · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We report a 42-year-old woman of non-herpetic acute limbic encephalitis (NHALE) whose CT perfusion (CTP) images revealed abnormalities of the limbic system at the early stage. The patient had high fever, convulsion and memory disturbance soon after having caught a common cold, and was admitted to a hospital where she developed progressive disturbance of consciousness. She was then transferred to our hospital 7 days after the initial manifestations. Although enhanced CT images of the brain failed to find any lesion, CTP images revealed a focal increase in the cerebral blood flow and shortening of mean transit time in the bilateral hippocampi and amygdalae. MRI of the subsequent day showed high signal intensity lesions on diffusion, T2-weighted and FLAIR images at the same area. Her consciousness improved by intravenous administration of high-dose methylprednisolone together with other combination therapies. Her CTP images apparently improved by 5 weeks after the onset, but she was left with mild memory disturbance, amenorrhea secondary to hypothalamic failure, hyperosmia, and hypogeusia. In conclusion, CTP is sensitive enough to detect the lesions of the limbic system even in the early stage of NHALE.
Rinsho shinkeigaku = Clinical neurology 09/2004; 44(8):537-40.
-
[show abstract]
[hide abstract]
ABSTRACT: The aims of this study were to prove the presence of transient phrenic nerve palsy in children after cardiac surgery by successive recordings of diaphragmatic action potentials (DAPs), and to decide the indication of diaphragmatic plication in infants with postoperative phrenic nerve palsy.
The DAPs were recorded from 11 infants (age 0-54 months) under artificial ventilation after cardiac surgery. The successive DAP recordings were performed within 3-4 days (0W), 1 week (1W) and 2 weeks (2W) after operation to make a final decision for diaphragmatic plication to wean artificial ventilation.
The patients were divided into 3 groups according to the DAP changes in successive recordings, namely, patients with normal DAPs at 0W, patients with transient depression of DAPs at 0W followed by recovery to normal DAPs by 1W and/or 2W, and patients with persistent depression of DAPs of the affected side necessitating plication of hemidiaphragm.
In infants with phrenic nerve palsy after cardiothoracic surgery, persistently abnormal DAPs in repeated electrophysiologic examinations for at least 2 weeks after surgery are a useful guidance to support clinical and radiological evidence for an indication of diaphragmatic plication.
Clinical Neurophysiology 07/2004; 115(6):1469-72. · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is a benign and non-progressive motor neuron disease. Application of a cervical collar is believed to prevent progression of symptoms in the early stages, but there is no effective therapy for the advanced disease. We found that tendon transfer improved the activities of daily living (ADL) of a patient with advanced Hirayama disease. An operative reconstruction can be valuable, even in patients with Hirayama disease who have developed impaired ADL due to extensive intrinsic hand muscle atrophy.
Internal Medicine 02/2004; 43(1):79-81. · 0.94 Impact Factor
