Posterior Interosseous Neuropathy with Peripheral Dystonia: A Case Report.

Background: Posterior interosseous neuropathy is an uncommon cause of peripheral dystonia. Case Report: A 62-year-old man awakened and noticed right finger drop. A neurological examination revealed posterior interosseous neuropathy with dystonia-like finger movements. Abnormal movements were predominantly observed in the right thumb, ring finger, and little finger. Within 2 weeks, the muscle weakness in the right fingers had completely improved. However, a brief abnormal posture of the right thumb was persistent. Discussion: The residual abnormal posture of the right thumb may reflect pre-existing motor control abnormalities, which may have contributed to the onset of posterior interosseous neuropathy-associated peripheral dystonia.

Hand-foot synkinesis in a patient with phenytoin intoxication.

Here we report the first case of phenytoin intoxication that was closely associated with hand-foot synkinesis. This case suggests a close association between cerebellar dysfunction and hand-foot synkinesis. In patients with hand-foot synkinesis, lesions of not only the secondary motor areas but also the cerebellum should be considered.

Bilateral Vocal Cord Paralysis Associated with Meningeal Carcinomatosis from Lung Adenocarcinoma.

Cranial neuropathy is a clinical manifestation of meningeal carcinomatosis (MC); however, the glossopharyngeal and vagus nerves are rarely impaired. Therefore, dysphagia and bilateral vocal cord paralysis (BVCP) are extremely rare manifestations of MC. Here, we present a case of MC from a lung adenocarcinoma presenting with dysphagia and BVCP. An 84-year-old man with a 4-year history of left lung adenocarcinoma developed dysphagia and hoarseness. Flexible nasopharyngoscopy revealed BVCP. Ten days later, the patient developed stridor and respiratory distress. A tracheotomy was performed to prevent airway obstruction. Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain showed enhancement of the bilateral glossopharyngeal and vagus nerves, and several enhancing lesions in the right internal auditory canal, left cerebellum, fourth ventricle, pons, cerebral aqueduct, and right frontal lobe, suggesting MC and brain metastasis. Based on the clinical history of malignancy and the MRI findings, the patient was diagnosed with MC. As the patient refused additional treatment, including chemotherapy and radiation, only palliative care was provided. To the best of our knowledge, this was the first case of MC from a solid tumor presenting with BVCP. When patients with malignancy present with BVCP, MC should be considered.

Occipital Neuralgia Secondary to C2 Spinal Cord Infarction.

We herein report the first case of occipital neuralgia secondary to spinal cord infarction. A 74-year-old woman suddenly developed numbness and dysmetria in her right arm. Two days later, she developed a paroxysmal shooting pain in the right posterior part of the scalp three to five times per day. Magnetic resonance imaging revealed a hyperintense lesion in the right posterior column and dorsal root entry zone at the C2 level. The patient was subsequently diagnosed with occipital neuralgia secondary to spinal cord infarction. Diverse etiologies need to be considered in occipital neuralgia secondary to spinal cord lesions.

A case of chronic inflammatory demyelinating polyneuropathy following COVID-19 vaccine.

We report a 44-year-old woman who presented with bilateral weakness of the hands and distal paresthesia of the arms on the next day of the second COVID-19 vaccine, and gradually progressed ascending weakness of the arms and legs, and sensory ataxia beyond 2 months. She was diagnosed as a chronic inflammatory demyelinating polyneuropathy (CIDP) following COVID-19 vaccine on the basis of clinical and electrophysiological findings. This is a first case diagnosed as a CIDP following COVID-19 vaccine alone.

Index finger pointing posture in a patient with drug-induced foot tremor.

A 41-year-old woman who had been taking paroxetine began taking tramadol for bilateral ankle pain. A few days later, the patient presented acutely with both feet tremors. During a mental arithmetic task, index-finger pointing posture briefly appeared on the left side. Co-administration of paroxetine and tramadol increases the risk of serotonin toxicity. This is the first reported case of index-finger pointing posture which was associated with serotonin toxicity.

Clinical investigation in non-liver cirrhosis portosystemic shunt encephalopathy-four case series.

We reported here four cases presenting with disturbance of consciousness over long periods of time and hyperammonemia. Two patients were on maintenance hemodialysis. Contrast-enhanced computed tomography (CT) of abdomen and balloon-occluded retrograde contrast venography revealed existence of a non-cirrhotic portosystemic shunt. Conservative treatment such as intravenous branched-chain amino acid administration and oral lactulose administration had only a modest effect in all patients. Improvements in symptoms were observed following the occlusion of the shunt path in three patients. Measurements of ammonia values would be the most important test for screening, but changes in Fischer's ratio or indocyanine green (ICG) test values were also correlated with clinical symptoms. Neurologists should keep in mind the possibility of non-cirrhotic portosystemic shunts when they encounter patients with disturbance of consciousness. They should also remember that occlusion of the shunt pathway is an effective treatment.

[Lambert-Eaton myasthenic syndrome associated with pulmonary squamous cell carcinoma and circulating anti-P/Q-type voltage-gated calcium channel antibody].

We report a 64-year-old man diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) associated with pulmonary squamous cell carcinoma. Circulating anti-P/Q-type voltage-gated calcium channel (VGCC) antibody was detected, and the patient was treated with 3,4-diaminopyridine. At age 61, chest radiograph revealed a tumor shadow in the right upper lung field. This was surgically removed, and a histological diagnosis of moderately differentiated pulmonary squamous cell carcinoma was obtained. After about 1 year, mediastinal metastasis was detected and 5-FU was administered. Eight months later, metastasis was noted in the left frontal hemisphere, and radiosurgical therapy was performed. The brain tumor gradually shrank but generalized fatigue, thirst, and gait disturbance developed after 4 months. A diagnosis of LEMS was made on the basis of neurological findings including proximal muscle weakness and absent tendon reflexes; autonomic symptoms (thirst, constipation, and impotence); characteristic electromyographic findings; and circulating anti-P/Q-type VGCC antibody. He has been treated with 3,4-diaminopyridine at a dose of 30 mg/day, resulting in marked improvement in symptoms but little change in electromyographic findings. The present case is very rare and suggests that anti-P/Q-type VGCC antibody may be involved in the mechanism of LEMS associated with pulmonary squamous cell carcinoma.

Analysis of DNA variations in promoter region of HCNP gene with Alzheimer's disease.

Hippocampal cholinergic neurostimulating peptide (HCNP), which enhances acetylcholine synthesis and induces cholinergic phenotype development of the septohippocampal system, is derived from HCNP precursor protein (HCNPpp), also known as phosphatidylethanolamine binding protein (PEBP) and Raf kinase inhibitor protein (RKIP). Our previous study demonstrated that expression of HCNPpp mRNA was decreased in the hippocampi of autopsied brains of Alzheimer's disease (AD) patients, indicating the association of HCNP with the pathogenesis of AD. To clarify the involvement of gene variations in the promoter region of the gene encoding HCNPpp in this mRNA reduction, we analyzed DNA polymorphisms or mutations within this gene promoter region in AD patients by direct sequencing. The promoter was found to contain a CpG island without a TATA box, an element of housekeeping gene promoters. Moreover, no disease-specific polymorphisms or mutations were identified, suggesting that the decrease of mRNA can be ascribed to transcriptional or posttranscriptional changes in activity.

Posterior Reversible Encephalopathy Syndrome after a Variety of Combined Chemotherapies Containing Bevacizumab for Metastatic Colon Cancer.

A 44-year-old woman with advanced metastatic colon cancer received chemotherapies comprising oxaliplatin and capecitabine (XELOX), irinotecan hydrochloride, leucovorin calcium and fluorouracil irinotecan (FOLFIRI)/panitumumab and mFOLFOX6/bevacizumab. Fifteen months later, she presented with the acute onset of a headache, drowsiness and seizure with a fever and hypertension. Brain magnetic resonance imaging (MRI) indicated bilateral regions of signal hyperintensity in the white matter with spasms of bilateral cerebral arteries apparent on magnetic resonance angiography. Posterior reversible encephalopathy syndrome (PRES) was diagnosed, and treatments resulted in improvement of the MRI findings, but the patient experienced cerebral infarction and ultimately died of deterioration of cancer on day 26 after the onset of PRES.

Cognitive event-related potentials: useful clinical information in Alzheimer's disease.

Cognitive event-related potentials (ERPs) have been used as a marker of cognitive function in patients with psychiatric and neurological disorders. In particular, the P300 potential has been widely utilized to study dementia and aging, because the P300 ERP component is easily observed and reflects attention and memory processing. However, the relationship between parameters of the P300 potential and the severity or type of cognitive impairment in patients with Alzheimer's disease (AD) remains controversial. Because specific and effective anti-dementia treatments have recently become available for AD, more useful information is needed about the clinical aspects of this disease, including methods of making an accurate and early diagnosis, differentiation from vascular dementia and other degenerative dementias, assessment of severity, assessment of disease progression, evaluation of the response to treatment, and prediction of the prognosis. This mini-review described new discoveries on recent clinical application of ERPs in AD with respect to the above-mentioned areas. Although the definition of normal ERP values and the most appropriate method of ERP recording in AD patients have not been well defined, recent findings suggest that ERP analysis may be a clinically useful, inexpensive, noninvasive, and reliable method of assessing AD.

Long-term effects of donepezil on P300 auditory event-related potentials in patients with Alzheimer's disease.

The P300, one of the cognitive event-related potentials (ERPs) of the cerebral cortex, reflects the functioning of the neurochemical system involved in cognitive processes. We investigated clinical significance of the components of auditory P300 ERPs, in comparison with neuropsychologic tests including the Mini-Mental State Examination and the Japanese version of the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-J cog), for evaluating of the effect of donepezil (DPZ) (5 mg daily for 6 months), an acetylcholinesterase inhibitor, in patients with Alzheimer's disease (AD). Reduction of P300 latency associated with a parallel improvement of ADAS-J cog scores was observed after administration of 5 mg/day of DPZ in patients with AD. P300 latency gives very useful information on the progression of AD, especially in the longitudinal follow-up of patients with AD during treatment with DPZ acting on cholinergic pathways.

[A case of cavernous sinus cavernous hemangioma presenting with cavernous sinus syndrome].

The authors report an unusual case of a 50-year-old woman presenting with cavernous sinus syndrome, who had a cavernous sinus cavernous hemangioma (CSCH). The acute onset of her symptoms including pain of the right eye, blephaloptosis of the right eye, diplopia, and sensory disturbance of the right face was similar to those of Tolosa-Hunt syndrome. Magnetic resonance imaging and angiography showed a tumor in the right cavernous sinus. Although she showed improvement of the symptoms after receiving oral corticosteroids, follow-up neuroradiological investigations after a year from the onset revealed the mass in the right cavernous sinus which grew up in size. The histopathological findings obtained from the biopsy of the mass demonstrated a CSCH. Our findings suggest that the growth mechanism of CSCH could be progressive ectasia of vessels or their autonomous development at the edges of the lesions.

Comparison of effects of valsartan and amlodipine on cognitive functions and auditory p300 event-related potentials in elderly hypertensive patients.

OBJECTIVE: We compared the antihypertensive effect of valsartan (VAL) and amlodipine (AML) treatments in elderly hypertensive patients by examining the long-term changes in cognitive function and auditory P300 event-related potentials. METHODS: We enrolled 20 outpatients, including 12 men and 8 women in the age group of 56 to 81 years who had mild to moderate essential hypertension. The subjects were randomly allocated to receive either 80 mg VAL once a day (10 patients) or 5 mg AML once a day (10 patients). Neuropsychological assessment and auditory P300 event-related potentials were obtained before initiation of VAL or AML treatment and after 6 months of the treatment with VAL or AML. Neuropsychological assessment was evaluated by conducting the Mini-Mental State Examination, the verbal fluency, word-list memory, word-list recall test, word-list recognition, and Trails B tests. RESULTS: Both the groups showed significantly reduced-blood pressure after 6 months of treatment, and the intergroup difference was not significant. The mean baseline Mini-Mental State Examination scores of the VAL and AML groups were not significantly different. Amlodipine treatment did not significantly affect any test score, but VAL treatment significantly increased the word-list memory and word-list recall test scores. Valsartan, and not AML, significantly reduced the mean P300 latency after 6 months. CONCLUSIONS: These results suggest that VAL exerts a positive effect on cognitive functions, independent of its antihypertensive effect.

Longitudinal study on MRI intensity changes of Machado-Joseph disease: correlation between MRI findings and neuropathological changes.

To disclose the neuropathological progression course of Machado-Joseph disease (MJD), magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four males and two females, including an autopsied female, all unrelated to one another) were further investigated on neurodegeneration. Brain MRI studies were repeated in all cases at different stages of the disease. Ages at the first MRI study ranged from 47 to 65 years (55.2 ± 7.1), with observation periods from 4.5 to 10.6 years (6.95 ± 2.48). We paid particular attention to two types of previously reported MRI findings detectable using T2-weighted images. One is located just outside the internal capsules, and another in the pons. A linear high-intensity change along the internal capsules was detected in all cases, and showed progression during the observation period. A comparison between MRI and autopsy findings suggested that the lesion might reflect degeneration with neuronal loss, astrocytosis, and gliosis in the internal segment of the globus pallidus. A cross-shaped high-intensity change in the pons was well advanced but still incomplete in all cases. In this region, pontine transverse fibers were atrophic, while longitudinal fibers remained intact. Pontine nuclei showed apparent nerve cell loss, and the remaining cells had many 1C2-positive intranuclear inclusions. Since these findings were detected both by lifetime images and by postmortem examination, MRI intensity changes could track the progression of neurodegeneration. Based on a comparison between MRI findings and neuropathology, the degeneration of an internal segment of the globus pallidus (one of the pathological features of MJD) had progressed following the initial symptoms.

Effects of ascorbic acid on ambulatory blood pressure in elderly patients with refractory hypertension.

The increased production of reactive oxygen species plays a role in the etiology of hypertension, but the effects of antioxidants on blood pressure are controversial. However, antioxidants possibly lower blood pressure in elderly patients with hypertension, because vascular aging is also closely related to oxidative stress. Effects of chronic treatment with ascorbic acid (CAS 50-81-7; 600 mg/day for 6 months) on blood pressure and levels of C-reactive protein, 8-isoprostane, and malondialdehyde-modified low-density lipoproteins were examined in elderly patients (n = 12, six males/six females, age 78.3 +/- 5.0 years, mean +/- SD [range, 67 to 84 years]; elderly group) and adult patients (n = 12, five males/seven females, age 54.6 +/- 6.7 years [range, 39 to 621; adult group) with refractory hypertension. Chronic treatment with ascorbic acid markedly reduced systolic blood pressure and pulse pressure in ambulatory blood pressure monitoring in the elderly group (from 154.9 +/- 21.6 to 134.8 +/- 19.7 mmHg, p < 0.001; and from 79.1 +/- 22.1 to 63.4 +/- 18.7, p < 0.05; respectively), which was accompanied by an increase in the serum levels of ascorbic acid and decreases in the levels of C-reactive protein, 8-isoprostane, and malondialdehyde-modified low-density lipoproteins. In contrast, ascorbic acid did not affect blood pressure in the adult group. These results suggest that ascorbic acid is useful for controlling blood pressure in elderly patients with refractory hypertension.