Campylobacter coli infection causes spinal epidural abscess with Guillain-Barré syndrome: a case report.

BACKGROUND: Guillain-Barré syndrome (GBS) and spinal epidural abscess (SEA) are known as mimics of each other because they present with flaccid paralysis following an infection; however, they differ in the main causative bacteria. Nevertheless, the two diseases can occur simultaneously if there is a preceding Campylobacter infection. Here, we report the first case of SEA with GBS following Campylobacter coli infection. CASE PRESENTATION: A 71-year-old Japanese man presented with progressive back pain and paralysis of the lower limbs following enteritis. Magnetic resonance imaging showed a lumbar epidural abscess that required surgical decompression; therefore, surgical drainage was performed. Blood cultures revealed the presence of C. coli. Despite surgery, the paralysis progressed to the extremities. Nerve conduction studies led to the diagnosis of GBS. Anti-ganglioside antibodies in the patient suggested that GBS was preceded by Campylobacter infection. Intravascular immunoglobulin therapy attenuated the progression of the paralysis. CONCLUSIONS: We report a case of SEA and GBS following Campylobacter infection. A combination of the two diseases is rare; however, it could occur if the preceding infection is caused by Campylobacter spp. If a cause is known but the patient does not respond to the corresponding treatment, it is important to reconsider the diagnosis based on the medical history.

Slow vital capacity as a useful indicator of the prognosis after percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis.

OBJECTIVES: Forced vital capacity (FVC) is recommended as a respiratory function test in patients with amyotrophic lateral sclerosis (ALS). However, in ALS associated with orofacial palsy, FVC may be an unreliable test. Slow vital capacity (SVC) is an easier and more reliable test even in cases with bulbar symptoms. However, it remains unclear whether respiratory function tests using SVC and FVC are associated with prognosis after percutaneous endoscopic gastrostomy (PEG) placement. This study aimed to confirm whether both SVC and FVC are related to prognosis after PEG placement in patients with ALS. MATERIALS AND METHODS: We conducted this retrospective observational cohort study of 69 consecutive patients diagnosed with sporadic ALS who underwent PEG placement between July 2007 and February 2020. We analyzed the association with mortality 6 months after PEG placement and evaluated long-term prognosis. RESULTS: Forty-four patients met the inclusion criteria. In cases with decreased SVC (p < .01) and FVC (p < .01), a significant difference was observed in mortality 6 months after PEG placement, with an optimal cut-off of SVC ≤57.4% (sensitivity, 0.828; specificity, 0.867) and FVC ≤57.3% (sensitivity, 0.828; specificity, 0.867). Multivariate analysis showed that onset age ≥ 65 years (p < .05), SVC ≤57.4% (p < .01), and FVC ≤57.3% (p < .01) were associated with survival after PEG placement. CONCLUSIONS: SVC, like FVC, is an important prognostic factor after PEG placement in patients with ALS, and there is a possibility that evaluation using SVC can complement respiratory function testing even in cases where the evaluation of FVC is limited.

Explosive lithium production in the classical nova V339 Del (Nova Delphini 2013).

The origin of lithium (Li) and its production process have long been uncertain. Li could be produced by Big Bang nucleosynthesis, interactions of energetic cosmic rays with interstellar matter, evolved low-mass stars, novae, and supernova explosions. Chemical evolution models and observed stellar Li abundances suggest that at least half the Li may have been produced in red giants, asymptotic giant branch (AGB) stars, and novae. No direct evidence, however, for the supply of Li from evolved stellar objects to the Galactic medium has hitherto been found. Here we report the detection of highly blue-shifted resonance lines of the singly ionized radioactive isotope of beryllium, (7)Be, in the near-ultraviolet spectra of the classical nova V339 Del (Nova Delphini 2013) 38 to 48 days after the explosion. (7)Be decays to form (7)Li within a short time (half-life of 53.22 days). The (7)Be was created during the nova explosion via the alpha-capture reaction (3)He(α,γ)(7)Be (ref. 5). This result supports the theoretical prediction that a significant amount of (7)Li is produced in classical nova explosions.

GABA storage and release in the medial globus pallidus in L-DOPA-induced dyskinesia priming.

Levo-dihydroxyphenylalanine (L-DOPA) is the most effective treatment for Parkinson's disease; however, most patients develop uncontrollable abnormal involuntary movements known as L-DOPA-induced dyskinesia. L-DOPA-induced dyskinesia can be reduced by pallidotomy of the medial globus pallidus or pallidal deep brain stimulation, suggesting that the medial globus pallidus plays a significant role in the development of L-DOPA-induced dyskinesia. In the present study, the pathological changes of the medial globus pallidus in L-DOPA-induced dyskinesia were studied in rat models of Parkinson's disease (unilateral 6-hydroxydopamine lesioning) and L-DOPA-induced dyskinesia (L-DOPA injection in Parkinson's disease-model rats twice daily for 2 weeks, confirmed by display of dyskinesia-like abnormal involuntary movements). L-DOPA-induced dyskinesia-model rats displayed medial globus pallidus hypertrophy, enlarged axon terminals surrounding the dendrites of medial globus pallidus neurons, and increased density of synaptic vesicles in enlarged axon terminals on the lesioned side. Synaptic terminal enlargement reversed after discontinuation of L-DOPA. Histological studies revealed the enlarged synaptic terminals were those of GABAergic striatal (direct pathway) neurons. A single injection of L-DOPA enhanced GABA release in the medial globus pallidus on the lesioned side in L-DOPA-induced dyskinesia-model rats compared to Parkinson's disease-model rats. In addition, microinjection of muscimol, a GABAA receptor agonist, into the medial globus pallidus on the lesioned side of Parkinson's disease-model rats induced dyskinesia-like abnormal involuntary movements. Microinjection of bicuculline, a GABAA receptor antagonist, into the medial globus pallidus on the lesioned side alleviated L-DOPA-induced dyskinesia in Parkinson's disease-model rats that had received L-DOPA prior to the microinjection. These results indicate that priming for L-DOPA-induced dyskinesia comprises excessive GABA storage in axon terminals of the direct pathway and that expression of L-DOPA-induced dyskinesia is associated with enhanced GABA release into the medial globus pallidus after L-DOPA dosing and the resultant excessive stimulation of GABAA receptors.

Assessing the relationship between non-motor symptoms and health-related quality of life in Parkinson's disease: a retrospective observational cohort study.

OBJECTIVES: Non-motor symptoms (NMSs) negatively impact the health-related quality of life (HrQOL) of patients with Parkinson's disease (PD). The Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) is a comprehensive scale for evaluating PD. It remains unclear whether the NMSs evaluated with MDS-UPDRS are predictive of HrQOL. This study aimed to investigate whether NMSs, as evaluated with the MDS-UPDRS, could predict the HrQOL of patients with PD. MATERIALS AND METHODS: We conducted a 2-year retrospective observational cohort study assessing 108 patients with PD who were recruited from a single tertiary center between January 2015 and December 2017. MDS-UPDRS was used to assess NMSs and motor symptoms and Parkinson's Disease Questionnaire-39 (PDQ-39) to measure patients' HrQOL. RESULTS: The median age of patients was 69 years, and 65.7% were female. The median MDS-UPDRS part I, part II, part III, and PDQ-39-summary index scores were 8, 10, 22, and 25, respectively. The final stepwise multiple linear regression model showed that female sex (standard partial regression coefficient ß = 0.131, P < 0.05) and baseline MDS-UPDRS part I (ß = 0.272, P < 0.01) and part II (ß = 0.571, P < 0.01) scores significantly predicted the PDQ-39-SI scores at the 2-year follow-up. CONCLUSIONS: In addition to motor symptoms, NMSs at the 2-year follow-up may be useful for predicting the HrQOL of patients with PD. In clinical practice, MDS-UPDRS-guided assessment and treatment of motor symptoms and NMSs may contribute to improving HrQOL in patients with PD.

Subarachnoid Hemorrhage Caused by Ruptured Aneurysm of the Artery of Adamkiewicz: a Case Report.

A 66-year-old man presented with a 6-day history of progressive posterior cervical pain that included lower back pain and fever. Neurological examinations revealed neck stiffness. Computed tomography demonstrated convexity subarachnoid hemorrhage. A spinal T2-weighted image revealed a hypointense signal lesion with contrast enhancement of the intradural extramedullary space at Th12-L1. Digital subtraction angiography showed a fusiform aneurysm with a 10 mm diameter in the artery of Adamkiewicz. We diagnosed the patient's condition as a ruptured aneurysm of the artery of Adamkiewicz. By day 41 the aneurysm had disappeared following conservative treatment. Aneurysms arising from the artery of Adamkiewicz are extremely rare and can cause both convexity and spinal subarachnoid hemorrhages. Clinicians should therefore look for spinal lesions if patients with convexity subarachnoid hemorrhage of an unknown origin have lower back pain as their initial symptom.

Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report.

A 51-year-old Japanese woman was admitted to our hospital because of speech difficulty following severe headache. Neurological examination showed dysarthria and tongue weakness on the right side, indicating right hypoglossal nerve palsy. Needle electromyography of the right side of the tongue showed fibrillation potentials. Magnetic resonance angiography and computed tomography angiography revealed a right, persistent, primitive hypoglossal artery (PPHA) that met Lie's diagnostic criteria. Digital subtraction angiography showed an extended PPHA with irregular caliber in the portion running through the right hypoglossal canal. We diagnosed compression neuropathy of the hypoglossal nerve due to PPHA enlargement based on the findings of ipsilateral hypoglossal nerve palsy, fibrillation that indicated peripheral nerve palsy, and the enlarged diameter of the portion of the PPHA running through the right hypoglossal canal. We prescribed antihypertensive therapy. At 1 year after onset, her tongue weakness was alleviated. Clinicians should consider compression neuropathy due to a PPHA as one of the possibilities in the differential diagnosis of hypoglossal nerve palsy.

Atypical globular glial tauopathy with a combination of types I and II pathology.

Globular glial tauopathy (GGT) is a group of 4-repeat tauopathies characterized by widespread globular glial inclusions (GGIs). GGT is now classified into three subtypes based on the distribution and morphology of the GGIs. We report an autopsy case of GGT in an 85-year-old woman who presented with semantic dementia, a rare phenotype in GGT. Postmortem examination revealed marked atrophy of the frontotemporal and motor cortices and corticospinal tract degeneration with widespread occurrence of globular neurofibrillary tangles and GGIs. The distribution of the pathology was similar to that seen in GGT type III. However, the morphology of astrocytic inclusions in the present case differed from that in type III. Moreover, the tau burden in the primary motor area was more severe in the gray than in the white matter, and globular oligodendroglial inclusions were more numerous than astrocytic inclusions, corresponding to GGT type II. By contrast, the tau pathology in the temporal lobe was chiefly globular oligodendroglial inclusions in the white matter, corresponding to GGT type I. Thus, the present case exhibited a combination of GGT types I and II pathology. Our findings appear to extend the pathological heterogeneity of GGT.

Helicopter Transport for Patients with Cerebral Infarction in Rural Japan.

OBJECTIVES: Physician-staffed helicopter transport is faster than ground transport and allows for prompt medical care of patients in rural areas. In this study, we evaluated the relationship between helicopter transport and the prognosis of patients with acute cerebral infarction in rural Japan. METHODS: This retrospective, observational study included 546 patients with acute cerebral infarction attending Aomori Prefectural Central Hospital, which serves a rural region of Japan. Patients were separated into 2 transport groups: physician-staffed helicopter emergency medical services and ground emergency medical services. Patients were assessed for stroke severity, treatment, and prognosis. RESULTS: Of the 546 patients, 11.2% were transported by physician-staffed helicopter emergency medical services and 88.8% by ground emergency medical services. Although the distance transported was significantly longer in the physician-staffed helicopter emergency medical services group, the time from onset to reaching our hospital was similar between the groups. National Institutes of Health Stroke Scale on admission and final prognosis were significantly worse with physician-staffed helicopter emergency medical services than with ground emergency medical services. Multivariate analysis showed no association between transport system and prognosis. CONCLUSIONS: In this study, patients transported by physician-staffed helicopter emergency medical services had more severe stroke symptoms and poorer functional outcomes than those transported by ground emergency medical services. However, the transport time was shorter for physician-staffed helicopter emergency medical services; thus, physician-staffed helicopter emergency medical services may be useful for reducing transport time for patients in rural Japan.

Association of survival and hyperthermia after rt-PA for ischemic stroke.

BACKGROUND: Hyperthermia in patients with acute ischemic stroke is associated with poor outcome. Although previous studies have shown a negative effect on functional outcome, even in patients treated with intravenous recombinant tissue plasminogen activator (rt-PA), the effect on survival remains unclear. AIMS OF THE STUDY: The aim of this study was to evaluate the association between the functional and survival prognosis and hyperthermia in patients with acute ischemic stroke treated with rt-PA. METHODS: We studied 120 patients treated with rt-PA from 2306 consecutive Japanese patients with acute cerebral infarction at Aomori Prefectural Central Hospital between December 2009 and March 2017. We defined hyperthermia as ≥38°C within 72 hours after rt-PA administration. Propensity score matching was used to compare 34 non-hyperthermia and hyperthermia patient pairs. RESULTS: Final modified Rankin Scale scores were higher in the hyperthermia group than in the non-hyperthermia group. In addition, the Kaplan-Meier model showed that the non-hyperthermia group had significantly better survival rates than the hyperthermia group (hazard ratio, 5.3; 95% confidence intervals, 1.2-24.8). CONCLUSIONS: Hyperthermia within 3 days after rt-PA is associated with poor functional prognosis and survival outcome in patients with acute cerebral infarction.

Rectus Sheath Hematoma Following Intravenous Thrombolysis With Recombinant Tissue Plasminogen Activator for Cerebral Infarction: A Case Report.

A 76-year-old Japanese man was admitted to our department because of aphasia and right hemiparesis. Brain magnetic resonance imaging showed cerebral infarction caused by occlusion of the left middle cerebral artery, and alteplase therapy was started. Thrombectomy was subsequently performed, resulting in recanalization. On day 4 after admission, he complained of epigastric pain when coughing. On day 8, contrast-enhanced computed tomography showed a left abdominal rectus sheath hematoma without extravasation. We carefully followed up the patient with conservative therapy, and the rectus sheath hematoma was ameliorated. Rectus sheath hematomas present as acute abdominal pain and are often misdiagnosed. Although several predisposing factors for rectus sheath hematomas have been identified, whether recombinant tissue-type plasminogen activator causes rectus sheath hematomas, remains unclear. This case highlights the need to consider a rectus sheath hematoma as a differential diagnosis of abdominal pain following treatment with recombinant tissue-type plasminogen activator.

Dermatomal Sensory Manifestations in Opalski Syndrome.

A 31-year-old Japanese woman presented with sudden-onset unstable gait followed by nuchal pain. A neurological examination revealed right-sided limb weakness and decreased pain and thermal sensation on the left side below the level of the L1 dermatome. A lower lateral medullary infarction with ipsilateral hemiplegia, known as Opalski syndrome, caused by spontaneous vertebral artery dissection was diagnosed by magnetic resonance imaging. The spinothalamic tract in the medulla oblongata has a topographic arrangement of sensory fibers, and the dermatomal sensory deficit in this case can be explained in relation to that. This is the first reported case of Opalski syndrome with dermatomal sensory manifestations. Opalski syndrome could be a differential diagnosis for dermatomal sensory manifestations.

Elevated pain threshold in patients with asymptomatic diabetic neuropathy: an intraepidermal electrical stimulation study.

INTRODUCTION: The loss of epidermal nerve fibers is regarded as an early pathological change in human diabetes. We investigated epidermal Aδ nerve fiber function by examining pain threshold by means of intraepidermal electrical stimulation (IES) in early diabetic neuropathy. METHODS: We recruited 20 asymptomatic diabetic patients. Eighteen age-matched, healthy subjects served as controls. We placed the IES electrode onto the skin of the foot dorsum and delivered weak electrical stimulation. We defined pain threshold as the minimum electrical intensity at which a subject felt a pricking sensation. RESULTS: The mean pain thresholds in the patient group were significantly higher (0.053 ± 0.036 mA; P < 0.01) than in the control group (0.027 ± 0.006 mA). CONCLUSION: We confirmed that the pain threshold was elevated in early diabetic neuropathy. We conclude that the IES electrode is a useful tool to evaluate early diabetic polyneuropathy. Muscle Nerve 54: 146-149, 2016.

Effect of educational television commercial on pre-hospital delay in patients with ischemic stroke.

Administering intravenous recombinant tissue plasminogen activator (r-tPA) within 4.5 h or endovascular procedures within 8 h of ischemic stroke onset may reduce the risk of disability. The effectiveness of media campaigns to raise stroke awareness and shorten pre-hospital delay is unclear. We studied 1144 consecutive ischemic stroke patients at Aomori Prefectural Central Hospital, Japan, between March 2010 and February 2014. From March 2012, the government sponsored an educational campaign based on a television commercial to improve knowledge of stroke symptoms and encourage ambulance calls for facial palsy, arm palsy, or speech disturbance. For the 544 and 600 patients admitted before and during the intervention, respectively, we recorded the National Institutes of Health Stroke Scale score, stroke type, the time when patients or bystanders recognized stroke symptoms, and hospital arrival time. Pre-hospital delay, as the time interval from awareness of stroke to hospital arrival, was categorized as 0-3, 3-6, and 6+ h. The mean pre-hospital delay was shorter (12.0 vs 13.5 h; P = 0.0067), the proportion of patients arriving within 3 h was larger (55.7 vs 46.5 %; P = 0.0021), and the proportion arriving after 6 h was smaller (32.7 vs 39.5 %; P = 0.0162) in the intervention group than in the pre-intervention group. There was no significant difference in the proportion of patients treated with r-tPA (6 and 7.5 % of the intervention and pre-intervention groups, respectively). A television-based public education campaign potentially reduced pre-hospital delay for ischemic stroke patients, but the r-tPA treatment rate was unchanged.

Morphological and electrophysiological changes in intratelencephalic-type pyramidal neurons in the motor cortex of a rat model of levodopa-induced dyskinesia.

Levodopa-induced dyskinesia (LID) is a major complication of long-term dopamine replacement therapy for Parkinson's disease, and becomes increasingly problematic in the advanced stage of the disease. Although the cause of LID still remains unclear, there is accumulating evidence from animal experiments that it results from maladaptive plasticity, resulting in supersensitive excitatory transmission at corticostriatal synapses. Recent work using transcranial magnetic stimulation suggests that the motor cortex displays the same supersensitivity in Parkinson's disease patients with LID. To date, the cellular mechanisms underlying the abnormal cortical plasticity have not been examined. The morphology of the dendritic spines has a strong relationship to synaptic plasticity. Therefore, we explored the spine morphology of pyramidal neurons in the motor cortex in a rat model of LID. We used control rats, 6-hydroxydopamine-lesioned rats (a model of Parkinson's disease), 6-hydroxydopamine-lesioned rats chronically treated with levodopa (a model of LID), and control rats chronically treated with levodopa. Because the direct pathway of the basal ganglia plays a central role in the development of LID, we quantified the density and size of dendritic spines in intratelencephalic (IT)-type pyramidal neurons in M1 cortex that project to the striatal medium spiny neurons in the direct pathway. The spine density was not different among the four groups. In contrast, spine size became enlarged in the Parkinson's disease and LID rat models. The enlargement was significantly greater in the LID model than in the Parkinson's disease model. This enlargement of the spines suggests that IT-type pyramidal neurons acquire supersensitivity to excitatory stimuli. To confirm this possibility, we monitored miniature excitatory postsynaptic currents (mEPSCs) in the IT-type pyramidal neurons in M1 cortex using whole-cell patch clamp. The amplitude of the mEPSCs was significantly increased in the LID model compared with the control. This indicates that the IT-type pyramidal neurons become hyperexcited in the LID model, paralleling the enlargement of spines. Thus, spine enlargement and the resultant hyperexcitability of IT-type pyramidal neurons in M1 cortex might contribute to the abnormal cortical neuronal plasticity in LID.

Morphologic changes of dendritic spines of striatal neurons in the levodopa-induced dyskinesia model.

Maladaptive plasticity at corticostriatal synapses plays an important role in the development of levodopa-induced dyskinesia. Recently, it has been shown that synaptic plasticity is closely linked to morphologic changes of dendritic spines. To evaluate morphologic changes of dendritic spines of two types of striatal medium spiny neurons, which project to the internal segment of globus pallidus or the external segment of globus pallidus, in the levodopa-induced dyskinesia model, we used 6-hydroxydopamine-lesioned rats chronically treated with levodopa. Dendritic spines were decreased and became enlarged in the direct pathway neurons of the model of levodopa-induced dyskinesia. The same levodopa treatment to normal rats, in which no dyskinesia was observed, also induced enlargement of dendritic spines, but not a decrease in density of spines in the direct pathway neurons. These results suggest that a loss and enlargement of dendritic spines in the direct pathway neurons plays important roles in the development of levodopa-induced dyskinesia.

Pitfalls of voxel-based amyloid PET analyses for diagnosis of Alzheimer's disease: artifacts due to non-specific uptake in the white matter and the skull.

Two methods are commonly used in brain image voxel-based analyses widely used for dementia work-ups: 3-dimensional stereotactic surface projections (3D-SSP) and statistical parametric mapping (SPM). The methods calculate the Z-scores of the cortical voxels that represent the significance of differences compared to a database of brain images with normal findings, and visualize them as surface brain maps. The methods are considered useful in amyloid positron emission tomography (PET) analyses to detect small amounts of amyloid-ß deposits in early-stage Alzheimer's disease (AD), but are not fully validated. We analyzed the (11)C-labeled 2-(2-[2-dimethylaminothiazol-5-yl]ethenyl)-6-(2-[fluoro]ethoxy)benzoxazole (BF-227) amyloid PET imaging of 56 subjects (20 individuals with mild cognitive impairment [MCI], 19 AD patients, and 17 non-demented [ND] volunteers) with 3D-SSP and the easy Z-score imaging system (eZIS) that is an SPM-based method. To clarify these methods' limitations, we visually compared Z-score maps output from the two methods and investigated the causes of discrepancies between them. Discrepancies were found in 27 subjects (9 MCI, 13 AD, and 5 ND). Relatively high white matter uptake was considered to cause higher Z-scores on 3D-SSP in 4 subjects (1 MCI and 3 ND). Meanwhile, in 17 subjects (6 MCI, 9 AD, and 2 ND), Z-score overestimation on eZIS corresponded with high skull uptake and disappeared after removing the skull uptake ("scalping"). Our results suggest that non-specific uptakes in the white matter and skull account for errors in voxel-based amyloid PET analyses. Thus, diagnoses based on 3D-SSP data require checking white matter uptake, and "scalping" is recommended before eZIS analysis.