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Purpose: The aim of this study was to investigate a structured approach for effective speech therapy (ST) for dysarthria and speech-related quality of life in patients with sporadic spinocerebellar degeneration (SCD), including cerebellar-type multiple-system atrophy and cerebellar cortical atrophy. Method: Twenty-two patients with SCD (cerebellar-type multiple system atrophy, 15 patients; cerebellar cortical atrophy, seven patients) who underwent intensive ST were examined. Dysarthria was evaluated using the Scale for Assessment and Rating of Ataxia Speech Dysfunction, Assessment of Motor Speech for Dysarthria Articulation, oral diadochokinesis (OD), and Voice Handicap Index-10 (VHI-10). Respiratory muscle strength (inspiratory and expiratory pressure) and respiratory-phonatory coordination (maximum phonation time) were measured. Cognitive function was evaluated using the Montréal Cognitive Assessment and the word fluency test. Mood was evaluated using the Hospital Anxiety and Depression Scale. The relationships between dysarthria scales (particularly, VHI-10) and clinical data were analyzed using stepwise regression. The differences in outcomes after intensive ST were analyzed using the Wilcoxon signed-rank test. The alpha level (p) for statistical significance was set at 0.0125 by Bonferroni correction. Results: For both pre- and post-ST, the patient's OD (p = 0.002) and maximum phonation time (p = 0.002) significantly improved, except for Speech Dysfunction scores of the Scale for Assessment and Rating of Ataxia (p = 0.705) and the VHI-10 (p = 0.018). The Assessment of Motor Speech for Dysarthria Articulation, OD, and inspiratory pressure were identified as independent variables of VHI-10 (adjusted R[subscript 2] = 0.820) for speech-related quality of life; no correlations among the Montréal Cognitive Assessment, word fluency test, and Hospital Anxiety and Depression Scale scores were observed. Conclusion: OD and VHI-10 showed improvements due to changes in speech function and respiratory-phonatory coordination, justifying intensive ST treatment for dysarthria in patients with SCD.

Tumor necrosis factor (TNF)-α is a potent proinflammatory cytokine involved in the pathogenesis of diabetic neuropathy. We inactivated TNF-α to determine if it is a valid therapeutic target for the treatment of diabetic neuropathy. We effected the inactivation in diabetic neuropathy using two approaches: by genetic inactivation of TNF-α (TNF-α-/- mice) or by neutralization of TNF-α protein using the monoclonal antibody infliximab. We induced diabetes using streptozotocin in wild-type and TNF-α-/- mice. We measured serum TNF-α concentration and the level of TNF-α mRNA in the dorsal root ganglion (DRG) and evaluated nerve function by a combination of motor (MNCV) and sensory (SNCV) nerve conduction velocities and tail flick test, as well as cytological analysis of intraepidermal nerve fiber density (IENFD) and immunostaining of DRG for NF-κB p65 serine-276 phosphorylated and cleaved caspase-3. Compared with nondiabetic mice, TNF-α+/+ diabetic mice displayed significant impairments of MNCV, SNCV, tail flick test, and IENFD as well as increased expression of NF-κB p65 and cleaved caspase-3 in their DRG. In contrast, although nondiabetic TNF-α-/- mice showed mild abnormalities of IENFD under basal conditions, diabetic TNF-α-/- mice showed no evidence of abnormal nerve function tests compared with nondiabetic mice. A single injection of infliximab in diabetic TNF-α+/+ mice led to suppression of the increased serum TNF-α and amelioration of the electrophysiological and biochemical deficits for at least 4 wk. Moreover, the increased TNF-α mRNA expression in diabetic DRG was also attenuated by infliximab, suggesting infliximab's effects may involve the local suppression of TNF-α. Infliximab, an agent currently in clinical use, is effective in targeting TNF-α action and expression and amelioration of diabetic neuropathy in mice. [ABSTRACT FROM AUTHOR]

A 26-year-old woman presented with a seven-month history of weakness in her left upper limb, progressing to difficulty lifting her arms within a few weeks. Her symptoms progressed with fluctuations. For the past three months, she has been unable to stand due to weakness in her proximal lower limbs. Nerve conduction studies did not show any definite conduction block or abnormal sensory conduction, but motor conduction studies showed a slight prolongation of the terminal latency and a decrease in the frequency of the F-wave. A magnetic fatigue test indicated a proximal conduction block. Her symptoms were rapidly resolved with intravenous immunoglobulin treatment, leading to a diagnosis of chronic immune-mediated neuropathy, met both criteria for multifocal motor neuropathy (MMN) and motor chronic inflammatory demyelinating polyneuropathy (CIDP). Our case highlights the utility of the magnetic fatigue test in detecting conduction blocks and its role in differentiating between MMN and motor CIDP.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disease associated with the presence of anti-glycine receptor (GlyR) antibodies. We herein report an autopsy case of an 80-year-old man diagnosed with anti-GlyR antibody-positive PERM who presented with symptoms of oculomotor dysfunction and autonomic failure. Despite intensive immunotherapy, the neurological symptoms showed almost no improvement, and the patient succumbed to aspiration pneumonia and bacterial translocation. Postmortem pathology revealed mild inflammatory changes and neuronal loss that were disproportionate to a severe clinical presentation. These results suggest that the clinical symptoms of PERM may result from antibody-mediated GlyR internalization, leading to neuronal disinhibition, rather than a neuroinflammatory signature.

A 42 years old female suffered from systemic lupus erythematosus (SLE) about 20 years ago. While steroid was tapered for a steroid-induced psychiatric disorder, she presented with an acute confusional state and was diagnosed with neuropsychiatric SLE (NPSLE). MRI showed acute infarction mainly in the cortex of the right temporal lobe and MRA demonstrated dynamic subacute morphological changes such as stenosis and dilation in several major intracrainal arteries. The right vertebral artery diffusely dilated and subsequently formed an aneurysm in a week. Contrast-enhanced MRI vessel-wall imaging showed a remarkable enhancement of the aneurysm wall, which might indicate an unstable unruptured aneurysm. The prompt introduction of intravenous cyclophosphamide improved both clinical and radiological signs. Our case indicates that intensive immunosuppressive treatments should be considered in NPSLE patients with varying vasospasm and aneurysm, indicating exacerbated disease activity.

A 70-year-old woman was admitted to our hospital because of fever, numbness in her extremities and right drop foot. Because her hip prosthesis had loosened as a result of infection, she had been taking 100 mg of minocycline orally for eight months. Three months before admission, she had had melena several times and body weight loss and pyrexia developed. A month before admission, asymmetrical paresthesia and numbness appeared in her extremities and finally right drop foot developed. Laboratory tests showed elevated C-reactive protein and positive anti-nuclear antibody. Abnormalities found in nerve conduction study were compatible with mononeuritis multiplex. Sural nerve biopsy revealed an occluded medium-size artery in the epineurium and axonal degeneration in the nerve fascicles, confirming the diagnosis of vasculitic neuropathy. These manifestations met the American Congress Rheumatology criteria for polyarteritis nodosa. However, her clinical conditions markedly improved after discontinuing minocycline and therefore she was diagnosed as having minocycline-induced vasculitic neuropathy. Although minocycline-induced vasculitis is a well known adverse effect of the drug, peripheral neuropathy with biopsy findings has rarely been reported. Drug induced-vasculitis is important as a differential diagnosis for mononeuritis multiplex because the symptoms can be improved by the discontinuation of an offending drug.