Nerve ultrasound characteristics of immunoglobulin M neuropathy associated with anti-myelin-associated glycoprotein antibodies.

INTRODUCTION/AIMS: Immunoglobulin M neuropathy associated with anti-myelin-associated glycoprotein antibody (IgM/anti-MAG) neuropathy typically presents with chronic, distal-dominant symmetrical sensory or sensorimotor deficits. Ultrasonographic studies of IgM/anti-MAG neuropathy are limited, and were all performed on Western populations. We aimed to characterize the nerve ultrasonographic features of IgM/anti-MAG neuropathy in the Japanese population and evaluate whether they differ from the findings of the common subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: In this cross-sectional study, we retrospectively reviewed medical records and extracted the cross-sectional areas (CSAs) of C5-C7 cervical nerve roots and median and ulnar nerves of 6 IgM/anti-MAG neuropathy patients, 10 typical CIDP (t-CIDP) patients, 5 multifocal CIDP (m-CIDP) patients, and 17 healthy controls (HCs). RESULTS: Cervical nerve root CSAs were significantly larger at every examined site on both sides in IgM/anti-MAG neuropathy than in m-CIDP and HCs but were comparable to those in t-CIDP. Peripheral nerve enlargements were greatest at common entrapment sites (ie, wrist and elbow) in IgM/anti-MAG neuropathy, a pattern shared with t-CIDP but not with m-CIDP. The degree of nerve enlargement at entrapment sites compared to non-entrapment sites was significantly higher in IgM/anti-MAG neuropathy than in t-CIDP. DISCUSSION: Our study delineated the ultrasonographic features of IgM/anti-MAG neuropathy in the Japanese population and observed similar characteristics to those of t-CIDP, with subtle differences. Further studies comparing results from various populations are required to optimize the use of nerve ultrasound worldwide.

Cervical Root Enlargement in Segmental Zoster Paresis: A Study with Magnetic Resonance Imaging and Nerve Ultrasound.

A 72-year-old woman presented with acute-progressive muscle weakness after a rash in the left upper limb. Muscle weakness was restricted to the left C5 innervated muscles. Short inversion time inversion recovery magnetic resonance imaging (MRI) showed a high-intensity signal in the left C5 nerve root, and nerve ultrasound showed its enlargement. She was diagnosed with segmental zoster paralysis (SZP) and treated with acyclovir and methylprednisolone. Her muscle strength gradually recovered, and the abnormal signal and enlargement in the left C5 nerve root improved. This is the first SZP case of confirmed improvement of abnormal findings on MRI and nerve ultrasound in association with muscle power recovery.

Multifocal motor neuropathy and visual pathway impairment: A case report.

BACKGROUND: Multifocal motor neuropathy (MMN) occasionally presents with cranial nerve involvement. However, no MMN cases with visual pathway impairment demonstrated by visual evoked potential (VEP) have been reported. CASE REPORT: A 36-year-old man was admitted to our hospital with progressive muscular weakness. On admission, neurological findings revealed bilateral muscle weakness and atrophy of the distal upper limbs. The blood tests were positive for GM-1 ganglioside antibodies. Nerve conduction studies revealed bilateral conduction block in the median nerve. He was diagnosed with MMN. Intravenous immunoglobulin treatment improved muscle weakness and blurred vision, which was not a complaint when he was first seen. Moreover, VEP showed a post-treatment shortening of P100 latency. These treatment effects were consistently observed for 3.5 years. SIGNIFICANCE: Our findings suggested that MMN could affect the visual pathway through autoimmune mechanisms.

[A case of suspected migraine with aura: transient cortical venous dilatation visualized by susceptibility-weighted imaging].

A 41-year-old man was admitted with proper name anomia and headache of sudden onset. He had a history of migraine without aura from the age of 35. Neurological examination on admission showed acalculia, proper name anomia, left-right disorientation and severe left-sided headache with nausea. Susceptibility-weighted MRI revealed dilatation of cortical veins of the left hemisphere. MR angiography and contrast CT revealed no cerebral arterial or venous occlusion. The patient's proper name anomia was improved at 5 hours from the onset and acalculia and left-right disorientation were improved at 17 hours from the onset. At 42 hours from the onset, he had recovered from his headache, and the dilatation of cortical veins of the left hemisphere had disappeared. Acalculia and left and right disorientation are rare presentations of migraine with aura. Susceptibility-weighted imaging may be a useful tool to distinguish migraine with aura from stroke and stroke mimics.

Sonographic Multifocal Cranial Nerve Enlargement in Multifocal Acquired Demyelinating Sensory and Motor Neuropathy.

Multifocal enlargements with the alteration of a normal fascicular pattern are considered to be sonographic peripheral nerve features in multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), a subtype of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We herein present the case of an 18-year-old patient with MADSAM in whom intensive sonological assessments revealed multifocal nerve enlargement within clinically affected cranial nerves. Our case demonstrated that, if systematically investigated with ultrasound, morphological changes similar to those in the peripheral nerves may be detected in a large proportion of clinically affected cranial nerves in MADSAM, boosting the future applications of cranial nerve ultrasound in CIDP.

Postoperative cerebral air embolism with delayed abnormal brain MRI findings.

Cerebral air embolism (CAE) is a rare but well-known complication resulting from invasive medical procedures; however, previous studies have not examined the postoperative longitudinal MRI changes in CAE. In particular, the likelihood that such changes may be observed after an initial delay when using magnetic resonance imaging (MRI) has not been explored. We herein report a case of CAE with no MRI abnormalities 4 h after a pulmonary vein isolation (PVI) procedure and where the first abnormality was found 22 h after the procedure. A 65-year-old man underwent PVI for paroxysmal atrial fibrillation and showed no signs of recovery from anesthesia after the procedure; thus, he was transferred to our emergency department for further examination. Neurological examination revealed conjugate eye deviation to the right and quadriplegia. Although initial computed tomography (CT) and MRI revealed no abnormalities, CAE was suspected, and a high-concentration oxygen treatment was administered. MRI performed 22 h after the procedure revealed restricted diffusion affecting the cortical areas. At the same day, he was transferred for hyperbaric­oxygen chamber treatment. After 7 days of treatment, the patient recovered clinically and neurologically. He regained consciousness and was able to communicate. As suggested by this case, CT and MRI findings may fail to reveal CAE abnormalities initially. In such cases, as urgent treatment is necessary, it is important to consider diagnosing CAE based on the patient's history and administering a high concentration of oxygen. Finally, to reach a correct diagnosis, repeated brain MRI should be considered for patients with suspected CAE.

Paraneoplastic Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome with SOX-1 Antibodies.

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. He was diagnosed with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS). He died from aspiration pneumonia on day 9. Small-cell lung carcinoma (SCLC), which had not been obvious on computed tomography, was found during the autopsy. Patients with PCD-LEMS who test positive for SOX-1 antibodies should be carefully evaluated for SCLC.

A Case of Invasive Aspergillus Rhinosinusitis Presenting with Unilateral Visual Loss and Subsequently Associated with Meningitis, Subarachnoid Hemorrhage, and Cerebral Infarction.

Visual impairment can occur because of several mechanisms, including optic nerve disease and occasionally fungal sinusitis. An 87-year-old man presented with the loss of right visual acuity; he was diagnosed with optic neuritis. Steroid pulse therapy was not effective. One month later, he became unconscious because of meningitis, following which treatment with ceftriaxone and acyclovir was initiated. However, his consciousness deteriorated because of a subarachnoid hemorrhage caused by a ruptured aneurysm. Meningitis and vascular invasion caused by fungal rhinosinusitis were suspected, and the sinus mucosa was biopsied. He was pathologically diagnosed with invasive Aspergillus rhinosinusitis. Despite continuous liposomal amphotericin B administration, he died of cerebral infarction, following a right internal carotid artery occlusion. It is important to consider the possibility of Aspergillus as an etiological agent, especially when cerebrovascular events are associated with visual impairment.

Appropriate assessment method of 123I-MIBG myocardial scintigraphy for the diagnosis of Lewy body diseases and idiopathic REM sleep behavior disorder.

BACKGROUND: In 123I-metaiodobenzylguanidine (123I-MIBG) myocardial scintigraphy, the early heart-to-mediastinum (H/M) ratio is considered to reflect the density of the cardiac sympathetic nerve endings, washout rate (WR) is an indicator of the cardiac sympathetic tone, and the delayed H/M ratio reflects both. The Delayed H/M ratio is usually used to support the diagnosis of Lewy body diseases (LBDs) and idiopathic REM sleep behavior disorder (iRBD); however, which values should be used have not been specified. Here, we hypothesized that the combination of these values is appropriate for the diagnostic purpose. METHODS: In this single-center retrospective cohort study, we recruited 106 patients with LBDs or iRBD and 33 patients without those diseases, of whom we reviewed the 123I-MIBG myocardial scintigraphy results. RESULTS: Sensitivity/specificity to diagnose LBDs and iRBD were 0.77/0.94 for the early H/M ratio (≤ 2.0), 0.82/0.94 for the delayed H/M ratio (≤ 2.0), and 0.89/0.91 for WR (≥ 23.0). When patients were considered positive if at least either the early H/M ratio or WR was abnormal, the sensitivity significantly increased to 0.97, whereas the specificity remained similar at 0.91. Furthermore, our subgroup analyses revealed that WR enhancement preceded H/M ratio reduction, but, in patients with a severely reduced early H/M ratio, paradoxically normal WR could be observed. CONCLUSION: We propose the highly sensitive, combined early H/M ratio and WR assessments for 123I-MIBG myocardial scintigraphy. The temporal precedence of cardiac sympathetic dysfunction over denervation and the floor effect in 123I-MIBG uptake may underlie the sensitivity improvement.

[A rare case of inclusion body myositis associated with anti-PM/Scl-75 antibodies].

A 71-year-old man presented with progressive muscle weakness of the four limbs in November 2014. His symptoms had started from the left leg in 2008, resulting in frequent falls. In 2011, he became unable to stand up without a handrail due to weakness of the both legs. Physical examination showed almost symmetric muscle weakness of the arms and legs; MMT4. The CK level was slightly elevated of 304 IU/l. The patient was diagnosed as having inclusion body myositis based on the muscle biopsy findings showing many fibers with rimmed vacuoles in addition to mononuclear cell infiltrating into the endomysium, surrounding and sometimes invading into non-necrotic muscle fibers. Anti-PM/Scl-75 antibodies were positive. Muscle strength improved after intravenous immunoglobulin therapy, although the effect was only temporary. This rare case suggests the autoimmunological etiology in inclusion body myositis.

Antidepressants for Depression, Apathy, and Gait Instability in Parkinson's Disease: A Multicenter Randomized Study.

Objective Depression, apathy, and gait instability are cardinal symptoms in patients with Parkinson's disease (PD). Selective serotonin reuptake inhibitors (SSRIs) and serotonin and norepinephrine reuptake inhibitors (SNRIs) are used for treating the psychiatric symptoms of PD. This is the first prospective randomized study to compare the efficacy of an SNRI (duloxetine) with SSRIs (paroxetine, escitalopram) in improving depressive symptoms and apathy (primary) and freezing of gait (FOG; secondary) in patients with PD. Methods In this prospective, multicenter, open-label, randomized study, Japanese PD patients with a Quick Inventory of Depressive Symptomatology-Japanese (QIDS-J) score ≥6 were randomly assigned to receive an SSRI (27 enrolled, 25 analyzed) or duloxetine (28 enrolled, 27 analyzed) and were assessed at 6 and 10 weeks. Results The mean change (SD) in the QIDS J [SSRI -2.4 (3.6), p=0.015; SNRI -2.3 (3.9), p=0.029] and FOG-Questionnaire [SSRI -2.9 (4.2), p=0.012; SNRI -3.4 (4.7), p=0.010] scores (from baseline) at 10 weeks was statistically significant, while the mean change in the Apathy Scale scores was not [SSRI -2.7 (5.4), p=0.054; SNRI -1.5 (3.7), p=0.109]. No significant differences were observed between the SSRI and SNRI groups. The treatments were well-tolerated; however, gastrointestinal events were more common with SSRIs. Two SNRI-treated patients reported an exacerbation of tremor. Conclusion SSRIs and SNRIs improve the depressive symptoms and FOG in PD patients with mild to severe depressive symptoms. However, their effectiveness in treating apathy remains to be elucidated.

Large fasciculation can clinically manifest as spinal myoclonus; electromyographic and dynamic echomyographic studies of four cases with motor neuron disease.

OBJECTIVE: Patients with motor neuron disease rarely present with fasciculation which is large enough to be clinically recognized as myoclonus. This study is aimed at elucidating the features of large fasciculation manifesting as myoclonus by using surface electromyography (surface EMG) and dynamic echomyography (dynamic Echo). METHODS: Four patients with amyotrophic lateral sclerosis, two of whom clinically presented with both fasciculation and myoclonus, were studied by using the surface EMG and the dynamic Echo. RESULTS: At rest, all patients had fasciculation in atrophic muscles, and the surface EMG showed occasional discharges of different waveforms corresponding to fasciculation. During voluntary gentle muscle contraction, the surface EMG showed repetitive discharges in the contracting muscle, which were constant in size and waveform within each muscle. The muscle Echo at rest revealed occasional contractions of a small number of muscle fibers corresponding to fasciculation. During voluntary muscle contraction, the number of muscle fibers involved in the involuntary motor phenomena was larger in the patients who clinically presented with myoclonus compared with other patients who clinically presented only with fasciculation. In a patient who presented with myoclonus, there was no contraction in the antagonist muscle. CONCLUSIONS: Fasciculation involving a large number of muscle fibers clinically manifests as spinal myoclonus. SIGNIFICANCE: Fasciculation involving a large number of muscle fibers can be a cause of spinal myoclonus.

Rate of augmentation and risk factors with long-term follow-up in Japanese patients with restless legs syndrome.

OBJECTIVES: Preventing augmentation is the critical clinical issue for RLS treatment. As for augmentation in Asian RLS patients, there have been only four studies and the follow-up durations of these studies were not long. We investigated Japanese RLS patients with longer duration of treatment in a clinical setting. METHODS: This study is a retrospective assessment of 42 RLS patients with follow-up durations of longer than 18 months (78.4 ± 29.2, range 19-139) at two urban sleep centers in Osaka, Japan from May 2004 to April 2014. RESULTS: The mean age of first visit was 63.5 ± 14.1 years old and the estimated age of RLS onset was 47.9 ± 16.5 years old. Twenty-eight out of 42 patients were female. At initial evaluation, the mean International Restless Legs Scale score (IRLS score) was 22.0 ± 5.9. Thirty-one of 42 had already visited other clinics before coming to our sleep centers, and the number of clinics visited was 1.3 ± 0.6. Augmentation developed in two patients (4.8%), and the dosage of dopamine equivalent in patients with and without augmentation was 12.5 and 18.8 mg vs. 15.8 ± 17.7 mg. In the two RLS patients with augmentation, ferritin was 113.1 and 114.1 ng/mL, respectively, and the number of clinics before coming to our sleep centers was both three. CONCLUSIONS: The augmentation rate of Japanese RLS patients from our study is low compared with previous Western and Asian studies. It might be attributable to racial difference, lower dosage of dopaminergic treatment, and the level of ferritin.

[Improvement of human T-lymphotropic virus type 1 associated demyelinating neuropathy with corticosteroid therapy].

The patient was a 58-year-old man, who was hospitalized with progressive bilateral leg weakness, and sensory impairment in all four extremities. Chronic inflammatory demyelinating polyneuropathy (CIDP) was suspected based on an electrophysiological examination, and intravenous immunoglobulin therapy (IVIg) was initiated. However, his symptoms progressed. The serum and cerebrospinal fluid were positive for human T-lymphotropic virus type 1 (HTLV-1) antibodies; hence the patient was diagnosed as having HTLV-1 associated neuropathy. After administration of corticosteroid, muscle strength markedly improved. Thus, our findings suggest that rather than IVIg, corticosteroid therapy is recommended for the treatment of CIDP like neuropathy in HTLV-1 infected patients.

Rheumatoid Meningitis Occurring during Etanercept Treatment.

We report a 65-year-old man who had repetitive seizures 6 months after receiving etanercept, methotrexate, and prednisolone for rheumatoid arthritis. Mononuclear cells were mildly increased in the cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) showed high intensity along sulci of the frontal and parietal lobes. Brain biopsy revealed lymphocyte and plasma cell infiltration in the meninges, confirming the diagnosis of rheumatoid meningitis. After steroid pulse therapy, seizures resolved and clinical findings improved. When etanercept was replaced by tocilizumab, rheumatoid meningitis did not recur. Although TNF-α inhibitors can control joint symptoms of rheumatoid arthritis, they may induce rheumatoid meningitis.

Non-granulomatous myositis in a patient with ulcerative colitis who showed symptoms resembling gastrocnemius myalgia syndrome.

The patient was a 36-year-old man. His initial symptom was bilateral thigh and calf pain. When he developed ulcerative colitis in the following year, he also noticed wasting of the calf muscles. The clinical feature is similar to gastrocnemius myalgia syndrome, although the left upper limb was also involved. A high-intensity lesion in the left calf and soleus muscles was observed on MRI, which was lead to the diagnosis of non-granulomatous myositis with infiltration of CD68-positive cells based on muscle biopsy. After steroids were administered, his pain subsided. Evaluation with needle EMG, MRI, and muscle biopsy is important when muscle pain accompanies inflammatory bowel disease.

A case of bulbospinal muscular atrophy with large fasciculation manifesting as spinal myoclonus.

OBJECTIVE: This paper reports a patient with bulbospinal muscular atrophy (BSMA) who presented with spinal myoclonus, documented by video and surface electromyography. CASE REPORT: A 66-year-old man had progressive gait disturbance, dysphagia, and easy fatigability of all extremities over a period of 4 years. Neurologically, muscle atrophy, fasciculation, and weakness were observed in the bulbar and limb muscles. When the knees were kept in mild flexion in the supine position, fasciculation of the thigh adductor muscles was so large that it caused shock-like involuntary movements of the legs, corresponding to spinal myoclonus. A genetic test revealed 41 repeats of CAG in the androgen receptor gene, and the diagnosis of BSMA was made. SIGNIFICANCE: The present case suggests that extremely large fasciculation can cause spinal myoclonus.

Peripheral neuropathies during biologic therapies.

Peripheral neuropathies should be recognized as the adverse effects of biological agents, especially anti-TNF agents. However, no solid clinical databases for biological agent-associated peripheral neuropathies (BAPN) have been established in Japan. Here we report two cases of peripheral neuropathy associated with anti-TNF agents. One was peroneal motor neuropathy. The other case was chronic inflammatory demyelinating polyradiculoneuropathy. In addition, we summarize the previous reports on BAPN and discuss their prevalence rate, pathogenesis and management.

[A case of left hemifacial metamorphopsia by a right retrosplenial infarction].

We report a 70-year-old women with left hemifacial metamorphopsia due to an infarction in the right retrosplenial region. She firstly noticed that the left half of her face reflected in the mirror was distorted. She complained of the same kind of distortion when she looked at the face of humans. Neurological examination on admission showed no other symptoms. Her visual acuity and visual field were normal. Diffusion weighted images of the brain revealed a high intense lesion in the right retrosplenial region, which was considered to account for her symptom. This case contributes to clarify the pathogenesis of hemifacial metamorphopsia.