Ultrasonographic data of cervical nerve roots diameter in 100 healthy adults.

Clinically significant evaluation of the diameters of nerve roots by ultrasonography requires the establishment of a normal reference range. Although there are multiple reports of nerve root diameters in normal subjects, none of them describe how to normalize and compare data derived from different facilities that may differ in their methodology, equipment, techniques, and recording sites during data acquisition. The aim of the present investigation was to establish a dataset of normal values using 100 healthy subjects, and to identify the factors that affect the normal ranges of cervical nerve root diameters with regard to age, sex, laterality, and root segments. Compared to previous reports, smaller standard deviations (0.07-0.21) were obtained, and the coefficient of variation ranged from 0.02 to 0.08, which facilitated the precise evaluation of cervical nerve roots. Age had a significant effect on the sixth cervical nerve root (C6) in male participants, and sex had a significant effect at C6 in participants in their 60s. To establish the normal values suitable for use across different facilities, acquired using different equipment, further development of various aspects, including the sophisticated recording techniques and data-sharing capabilities, is essential.

The natural history of hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P) in 97 Japanese patients.

Hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P) is a motor and sensory neuronopathy with autosomal dominant inheritance, adult onset, slowly progressive course, and is associated with TRK-fused gene (TFG) mutation. At advanced stages, respiratory failure and dysphagia becomes life-threatoning, and patients typically die by their 70s. Although there is currently no evidence for effective treatment, a therapy may be found by elucidation of the function of TFG. Recently its pathomechanism has been proposed to be associated with abnormalities in protein transfer from the endoplasmic reticulum. Such pathomechanisms might involve a similar process in amyotrophic lateral sclerosis; thus, its pathomechanisms and treatment strategy might make it a good model for neurodegenerative disorders. It is of great value to clarify the natural history of HMSN-P, in oder to judge the treatment effect. By evaluating 97 patients (79 out of 97 were examined and all confirmed with p.Pro 285 Leu mutation) in this study, it was confirmed that this disease follows a uniform course in the earlier stages, and there are individual differences in the onset between 20 and 30 years. Such uniformity might be due to the proposed single gene abnormality. At advanced stages, there are larger individual differences in the progression, but the reasons for these are unknown. Longer survival might be achieved with a better care for respiratory failure and dysphagia if such cares were undertaken at appropriate times.

[Complimentary use of needle electromyography and ultrasonography of tongue is effective for early detection of abnormality in 20 patients with amyotrophic lateral sclerosis].

A group of 20 consecutive patients with amyotrophic lateral sclerosis (ALS) were evaluated using electromyography (EMG) and ultrasonography (US) of the tongue. Their records were reviewed retrospectively for the rates at which abnormalities were detected by these two modalities as well as their clinical features. Visual inspection detected abnormalities in 9 of 20 patients, EMG in 12, and US in 6. However, EMG detected active denervation earlier than did US in 7 of the 12 EMG-diagnosed patients, and US detected fasciculation earlier than did EMG in 1 of the 6 US-diagnosed patients. Thus, we cannot replace EMG completely with US. Indeed, we currently use both methods complementarily at our hospital.

The Sequential Ultrasonographic, Electrophysiological and MRI Findings in a Patient with the Pharyngeal-cervical-brachial Variant of Guillain-Barré Syndrome from the Acute Phase to the Chronic Phase.

Acute progressive weakness in bulbar, neck and limbs is included in several differential diagnoses, including the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome (GBS). Patients with the PCB variant of GBS are reported to have localized diagnostic cervical spinal nerve abnormalities that can be examined by nerve ultrasonography (NUS) and magnetic resonance neurography (MRN). We herein report the case of a 77-year-old man with the PCB variant of GBS. Although the nerve conduction study (NCS) findings were indirect indicators for an early diagnosis, the combination of NCS and NUS was a useful complementary measure that facilitated an early diagnosis. MRN did not show any apparent diagnostic abnormalities. After early treatment, the patient was discharged and returned home.

[Clinical manifestations of 5 patients with idiopathic paroxysmal kinesigenic choreoathetosis].

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare disorder characterized by recurrent and brief attacks of choreoathetoid and/or dystonic movements in trunk and limbs triggered by initiation of voluntary movement. Of 5 patients with idiopathic PKC in our hospital, four were men and one was with family history. Age of onset ranged from 8 to 15 years old. They were consistent with previous reports in the characteristics of involuntary movements, normal neurological findings, normal laboratory data, no abnormal findings of standard imaging studies, and good restraining effects on attacks with carbamazepine. Individual body parts where attacks often involved were different among 5 patients. Although previous reports which said the prognosis and outcome of PKC were good, neuropsychological examinations in our study revealed that 2 patients out of 5 had certain cortical dysfunction, one patient was with progressive deterioration, and the other was with underlying mild abnormalities. Detailed and serial neuropsychological examinations might be necessary for some PKC patients.

Perceived age of facial features is a significant diagnosis criterion for age-related carotid atherosclerosis in Japanese subjects: J-SHIPP study.

AIM: Vascular aging is known to be a major determinant of life expectancy. Recently, perceived age was reported to be a better predictor for mortality than chronological age. Based on these findings, we investigated whether or not perceived age was related to atherosclerosis in a general population. METHODS: The participants were 273 individuals aged ≥ 50 years who participated in the Skin-doc in Anti-Aging Doc program. Facial photos were taken under a shadowless lamp from three directions (antero-posterior, and 60° right and left oblique projection) using a high-resolution digital camera. Perceived age was assessed either by 19 professional nurses in the geriatric ward or using facial identification program software. Carotid intima-media thickness (IMT), radial augmentation index (AI) and brachial-ankle pulse wave velocity (baPWV) were measured as indices for atherosclerosis. RESULTS: The perceived age difference (expressed as the difference between perceived age and chronological age), when estimated either by nurses or software, was significantly and negatively associated with chronological age. Subjects who were evaluated by nurses to be younger than their chronological age had significantly lower carotid IMT after adjustment for chronological age. Conversely, carotid IMT was an independent and negative determinant of looking young, as perceived by nurses. Similar observations were also made between perceived age using facial identification software and carotid IMT. Radial AI and baPWV were not associated with perceived age. CONCLUSION: These findings show that carotid atherosclerosis is related to perceived age. This association might underlie previous findings showing that perceived age predicts life expectancy.

Post-streptococcal chorea in an adult with bilateral striatal encephalitis.

We present distinctive MRI findings in an adult female patient with possible Sydenham's chorea. T2-weighted MRI showed bilaterally symmetric, diffusely homogenous, and clearly demarcated hyperintensities selectively involving the entire striatum with swelling of the bilateral caudate heads. The MRI features may reflect the pathogenetic mechanisms of Sydenham's chorea associated with a specific autoimmune response to the basal ganglia.

[An elderly fludrocortison-responsive woman with hyponatoremia].

An 89-years-old woman had anorexia for at least 1 month, and had been given symptomatic treatment at a nearby hospital. She was admitted to our hospital on August 22, 2003, for thorough examination and appropriate treatment for lack of spontaneity and appetite loss. On admission, laboratory data revealed hyponatremia (125 mEq/L) and hypoaldosteronism (0.7 ng/mL). Since hyponatremia did not improve by intravenous drip with saline, we identified the major cause of her complaint as hypoaldosteronism. She was treated with fludrocortisone (0.05 mg/day) and her condition improved immediately. Although she was discharged at that time, her condition shortly deteriorated. She was referred to our hospital on February 10, 2004 for medical treatment. On admission, inadequate oral intake, lack of spontaneity and weakness in her lower legs were noted. The plasma Na concentration was 127 mEq/L. Nasogastric tube feeding was started to prevent aspiration pneumonia because of her dysphagia. Fludrocortisone was given (0.2 mg/day), and she was able to swallow food without nasal feeding tube during the second month of therapy. Laboratory data including plasma natrium concentration were normal. Also she could perform bed-to-wheelchair transfer independently. This is a rare case of a critically ill elderly patient with hyponatremia caused by hypoaldosteronism possibly due to mineral corticoid-responsive hyponatremia of the elderly.