Deep white matter hyperintensities, decreased serum low-density lipoprotein, and dilative large arteriopathy.

Deep white matter hyperintensities (DWMHs) seen on magnetic resonance imaging (MRI) are thought to reflect small-vessel diseases (SVDs) and may have a background that differs from that of stenotic large-vessel diseases. We assessed risk factors for DWMHs and investigated the association between DWMHs and dilative changes in the basilar artery (BA) on MRI in nonstroke patients. We reviewed clinical information and MRI findings for 149 outpatients aged 46-90 years, excluding those with a previous symptomatic cerebrovascular event. DWMHs were graded 0-3, and the maximal BA diameter and area were measured from the flow void on axial T2-weighted MRI to assess dilatation. We divided the patients into groups with and without DWMH grade 2 or 3, and compared clinical information and BA parameters in these groups. The two groups demonstrated significant differences in age, serum low-density lipoprotein (LDL) level, estimated glomerular filtration rate (eGFR), and BA parameters. An adjusted logistic regression analysis including BA diameter found that age (odds ratio [OR], 1.974 per 10 years; 95% confidence interval [CI], 1.030-1.112; P = .0006), LDL (OR, 0.811 per 10 mg/dL; 95% CI, 0.964-0.965; P = .0085), eGFR (OR, 0.835 per 10 mL/min/1.73 m(2); 95% CI, 0.967-0.998; P = .0229), and BA diameter (OR, 2.515 per 1 mm; 95% CI, 1.191-4.098; P = .0119) were independently associated with the presence of DWMHs. An analysis including the BA area yielded similar results. DWMHs are manifestations of SVDs and show a strong association with lower serum LDL level, lower eGFR, and BA dilatation.

Relationship between cognitive function affecting motor Functional Independence Measure and hypnotics.

Otsubo H, Kishimoto K, Hirano I, Nakano H, Itaya K, Kumaki R, Osumi H. Relationship between cognitive function affecting motor Functional Independence Measure and hypnotics. Jpn J Compr Rehabil Sci 2022; 13: 4-11. Purpose: The purpose of this study was to examine the relationship between cognitive dysfunction affecting motor Functional Independence Measure (FIM) and hypnotics. Methods: This was a retrospective study involving 509 patients aged ≥ 65 years who were discharged from a convalescent rehabilitation ward. Results: Multiple regression analysis was performed with motor FIM efficiency and motor FIM effectiveness (motor FIM-e) as independent variables and the presence or absence of cognitive dysfunction as the dependent variable. The use of hypnotics in patients with cognitive dysfunction showed a positive relationship with motor FIM efficiency (ß = 0.147, P = 0.019) and motor FIM-e (ß = 0.141, P = 0.026). Multiple regression analyses were performed after further classifying hypnotics by therapeutic class into hypnotics with new mechanisms, non-benzodiazepine (BZ) hypnotics, and BZ hypnotics. Non-BZ hypnotics (ß = 0.141, P = 0.021) showed a positive relationship with motor FIM efficiency. Non-BZ hypnotics (ß = 0.158, P = 0.009) and BZ hypnotics (ß = 0.178, P = 0.003) showed a positive relationship with motor FIM-e, whereas hypnotics with new mechanisms of action did not. In contrast, none of the three combinations of hypnotics showed any significant relationship with either motor FIM efficiency or motor FIM-e in patients without cognitive dysfunction. Conclusion: The results suggested that the use of hypnotics in patients with cognitive dysfunction increases motor FIM efficiency and motor FIM-e.

[Analysis of the metallo-beta-lactamase gene in multidrug-resistant Pseudomonas aeruginosa isolated at Showa University Hospital].

We conducted a molecular epidemiological analysis of multidrug-resistant Pseudomonas aeruginosa (MDRP) isolated at Showa University Hospital. The ratio of MDRP to total P. aeruginosa was 0.8% (1/132 strains) from September to December 1993. This ratio was increased to 1.8% (18/1000 strains) from October 2003 to October 2004. We analyzed the genome type and drug-resistant gene of 18 MDRPs isolated from October 2003 to October 2004, and 6 MDRPs isolated from January to April 2005 in a follow-up survey. Genome-type analysis using pulsed-field gel electrophoresis after SpeI restriction enzyme digestion revealed that 12 of the 24 MDRP strains had an identical genome type, indicating a possible nosocomial infection. In order to analyze the drug resistance mechanism, the 2-mercaptopropionic acid inhibition test was performed. Twenty of 24 MDRP strains were positive for metallo-beta-lactamase. Of all the metallo-beta-lactamase-positive strains, IMP type beta-lactamase was detected by PCR. Sequence analysis of the PCR product of IMP type betalactamase identified that 2 strains were IMP-1 and 18 were IMP-10. Of the 12 strains having an identical genome type, IMP-1 was detected in 1 strain and IMP-10 was detected in 11 strains. From our results, we conclude that P. aeruginosa with the same genome type was continuously colonized in the hospital and independently acquired a drug-resistant gene.

[Listeria monocytogenes meningoencephalitis lacking meningeal signs].

We report a fatal case of meningoencephalitis due to Listeria monocytogenes. A 74-year old alcoholic man presented with high-grade fever lasting for four days without headache and meningeal signs. Routine blood analysis showed leukocytosis, but serum C-reactive protein (CRP) was not significantly elevated. He developed altered consciousness with focal seizure, and cerebrospinal fluid (CSF) examination showed a minor degree of pleocytosis, elevated protein, and hypoglycorrhachia. Repeated CSF examination four days later showed greater increases in cells and proteins as well as severely decreased glucose level. Bacterial culture from the initial CSF showed a growth of L. monocytogenes. Meningoencephalitis caused by L. monocytogenes may have atypical clinical and laboratory features, and should be listed in the differential diagnosis of immunocompromised or elderly patients presenting with fever of unknown origin associated with altered consciousness.

A case of a 17-year-old male with neurofascin-155 antibody-positive chronic inflammatory demyelinating polyradiculoneuropathy presenting with tremor and ataxia.

A 17-year-old male with no medical history noticed weakness of his limbs with imbalance and subsequent finger tremors. Physical examination revealed features of polyneuropathy, including diffuse weakness, distal symmetrical numbness with impaired deep sensation and areflexia in all limbs. Postural tremor was present in fingers. Ataxia was apparent in both lower limbs, causing a wide-based gait with a positive Romberg sign. Cerebrospinal fluid contained elevated total protein without pleocytosis. A nerve conduction study disclosed demyelinating features with prolonged terminal latencies, slow velocities with delayed F-wave latencies, and prominent temporal dispersion. These findings led to diagnosis of typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with notable feature of postural finger tremor and ataxia of unknown cause. These atypical features prompted us to examine neurofascin-155 (NF155) antibodies, which were positive. No significant improvement occurred after initial administration of intravenous immunoglobulin and subsequent plasma exchange. However, corticosteroids with intravenous pulse therapy followed by oral prednisolone significantly improved the symptoms. Patients with CIDP with anti-NF155 antibodies may have similar clinical features and constitute a CIDP subgroup. In such patients, corticosteroids may be more effective than intravenous immunoglobulin. Further studies are needed to define the features of this subgroup and determine effective therapy for CIDP.

[A case of chronic myopathy associated with an antibody to signal recognition particle (SRP) following long-term asymptomatic hypercreatinekinasemia].

A 65-year-old man first visited our hospital due to hypercreatinekinasemia (hyperCKemia) (669 IU/l) 12 years ago at age 53. At that time, he had normal muscle strength without other neurological deficits, electromyography (EMG) was normal, and a muscle biopsy obtained from the biceps brachii was intact in routine histochemical studies. These findings led to a diagnosis of idiopathic hyperCKemia that lasted for over a decade. At age 65, the patient became aware of muscle weakness and serum CK was elevated to 4,846 IU/l. Neurological examination revealed very mild atrophy in both thighs, proximal muscle weakness in the left upper and right lower limbs without myalgia, grasping pain, joint pain, and skin lesions. A typical myogenic pattern was detected on EMG exclusively in proximal limb muscles, and fat-suppressed MRI showed high intensity signal areas in adductor magnus muscles. The clinical diagnosis was limb-girdle muscular dystrophy, but MRI findings suggestive of an inflammatory process prompted us to perform muscle biopsy at the rectus femoris. The pathology had characteristic features of necrotizing myopathy containing necrotic and regenerating fibers without prominent inflammatory cell infiltration. Serum anti-signal recognition particle (SRP) antibodies were found to be positive and the final diagnosis was anti-SRP antibody myopathy. Muscle weakness progressed slowly despite therapy with oral corticosteroids. Addition of intravenous high-dose immunoglobulin therapy led to an apparent improvement of muscle weakness in parallel with lowering of the serum CK level. In those who were thought to be idiopathic hyperCKemia or hereditary muscle disorders, potential immunotherapy-effective group does exist. We suggest considering such cases including anti-SRP antibody myopathy during diagnosis, and non-invasive MRI study may be useful to differentiate immunotherapy-effective group from hereditary muscle disorders.

[Case of acute ischemic stroke due to cardiac myxoma treated by intravenous thrombolysis and endovascular therapy].

A 48-year-old woman with no previous neurological diseases was transferred to our hospital because of sudden-onset unconsciousness. On arrival, she showed consciousness disturbance (E1V1M3 on the Glasgow Coma Scale), tetraplegia, right conjugate deviation and bilateral pathological reflexes. These symptoms resulted in a NIH stroke scale score of 32. Brain diffusion-weighted MR imaging (DWI) showed multiple hyper-intense lesions, and MR angiography revealed occlusions of the basilar artery (BA) and superior branch of the right middle cerebral artery (MCA). Transthoracic echocardiography disclosed a 51 × 24 mm myxoma in the left atrium. These findings led to diagnosis of acute ischemic stroke due to embolization from cardiac myxoma. Thrombolytic therapy with intravenous tissue plasminogen activator (IV tPA) was started 120 min after onset because there were no contraindications for this treatment. However, the symptoms did not resolve, and thus endovascular therapy was performed immediately after IV tPA. Angiography of the left vertebral artery initially showed BA occlusion, but a repeated angiogram resulted in spontaneous recanalization of the BA. However, the left posterior cerebral artery remained occluded by a residual embolus. Subsequently, occlusion found in the superior branch of the right MCA was treated by intra-arterial local thrombolysis using urokinase and thrombectomy with a foreign body retrieval device, but the MCA remained occluded. DWI after endovascular therapy showed new hyper-intense lesions in the bilateral medial thalamus and left occipital cortex. Clinically, neurological status did not improve, with a score of 5 on the modified Rankin Scale. IV tPA can be used for stroke due to cardiac myxoma, but development of brain aneurysms and metastases caused by myxoma is a concern. Given the difficulty of predicting an embolus composite from a thrombus or tumor particle, aspiration thrombectomy may be safer and more effective for stroke due to cardiac myxoma to avoid delayed formation of brain aneurysms and metastases.

[Epilepsy with higher brain dysfunction].

Acquired higher brain dysfunction is for the most part due to cerebral vascular disease, but epilepsy may also be a cause. In this study with five patients, we discuss the advantages of anti-epileptic drugs (AEDs) for persistent higher brain dysfunction. The patients showed chronic amnesia or acute aphasia, with associated symptoms like personality change. All five cases affected automatism or convulsive attack, though only after the emergence of higher brain dysfunction and administration of AEDs. There were underlying diseases like cerebral arteriovenous malformation in four cases, but the other patient had none. Electroencephalogram and single photon emission computed tomography revealed one case of aphasia epilepsy with higher brain dysfunction. These results suggest the potential therapeutic efficacy of AEDs for persistent higher brain dysfunction, and we must differentiate epilepsy with higher brain dysfunction from dementia or cerebral vascular disease.

Unilateral Abducens Nerve Palsy as an Early Feature of Multiple Mononeuropathy Associated with Anti-GQ1b Antibody.

Patients with anti-GQ1b antibody syndrome show various combinations of ophthalmoplegia, ataxia, areflexia, or altered sensorium as clinical features. We describe herein a unique case with unilateral abducens nerve palsy as an early feature of multiple mononeuropathy involving dysfunctions of the inferior dental plexus and the ulnar nerve, which was thought to be associated with anti-GQ1b antibody. A 27-year-old man presented with acute-onset diplopia. He subsequently experienced numbness not only in the right lower teeth and gums but also on the ulnar side of the left hand. Neurological examinations revealed dysfunctions of the right abducens nerve, the right inferior dental plexus, and the left ulnar nerve, suggesting multiple mononeuropathy. Serum anti-GQ1b antibody was positive. This is a rare case report of a patient with unilateral abducens nerve palsy as an early feature of multiple mononeuropathy associated with anti-GQ1b antibody. We suggest that anti-GQ1b antibody syndrome should be taken into consideration as a differential diagnosis of acute multiple mononeuropathy if ophthalmoplegia is present unilaterally.