Combined Hypertrophic Pachymeningitis and Cerebral Venous Thrombosis in a Case of Granulomatosis with Polyangiitis.

We report a rare case of hypertrophic pachymeningitis (HP) and cerebral venous thrombosis associated with proteinase-3-antineutrophil cytoplasmic antibody (PR3-ANCA)-positive granulomatosis with polyangiitis (GPA). A 58-year-old male developed left headache after exudative otitis media. The laboratory data were positive for PR3-ANCA. Brain magnetic resonance imaging revealed bilateral paranasal sinusitis, left frontal lobe edema, and a thick dura mater with abnormal enhancement in the frontotemporal lobe. Magnetic resonance venography detected stenosis of the superior sagittal sinus. The patient was successfully treated with glucocorticoid, cyclophosphamide, and apixaban. Contrast neuroimaging should be performed for patients who present with unexplained headache, especially with middle ear and paranasal inflammation. These symptoms should be considered as GPA-related HP and cerebral venous thrombosis.

Clinical Benefits of Antioxidative Supplement Twendee X for Mild Cognitive Impairment: A Multicenter, Randomized, Double-Blind, and Placebo-Controlled Prospective Interventional Study.

Oxidative stress is part of the entire pathological process that underlies the development of Alzheimer's disease (AD), including the mild cognitive impairment (MCI) stage. Twendee X (TwX) is a supplement containing a strong antioxidative mix of eight antioxidants, which has been shown to have a clinical and therapeutic benefit in AD model mice. Here, we conducted a multicenter, randomized, double-blind, and placebo-controlled prospective interventional study to evaluate the efficacy of TwX in mitigating MCI. The primary outcomes were differences in Mini-Mental State Examination (MMSE) and Hasegawa Dementia Scale-revised (HDS-R) scores between baseline and six months for placebo and TwX groups. Seventy-eight subjects with MCI were randomized into placebo (n = 37) and TwX (n = 41) groups. MMSE scores at six months differed significantly between the TwX and placebo groups (p = 0.018), and HDS-R scores for the TwX group exhibited a significant improvement at six months relative to baseline (p = 0.025). The TwX group did not show any change in affective or activities of daily living scores at six months. The present study indicates that strong antioxidative supplement TwX is clinical beneficial for cognitive function in subjects with MCI.

Association of CYP2C19 Polymorphisms With Clopidogrel Reactivity and Clinical Outcomes in Chronic Ischemic Stroke.

BACKGROUND: CYP2C19variants are associated with the antiplatelet effects of clopidogrel against recurrent cardiovascular events. However, it remains unknown whether the elapsed time from stroke onset affects the relationship between the genetic variants and such events. To address this, we conducted a prospective cohort study to determine the effect ofCYP2C19variants on clinical outcomes in the chronic phase.Methods and Results:In total, 518 Japanese non-acute stroke patients treated with clopidogrel were registered at 14 institutions. Patients were classified into 3 clopidogrel-metabolizing groups according toCYP2C19genotype: extensive metabolizer (EM:*1/*1), intermediate metabolizer (IM:*1/*2or*1/*3), and poor metabolizer (PM:*2/*2,*2/*3, or*3/*3). Antiplatelet effects of clopidogrel were assessed by adenosine diphosphate (ADP)-induced platelet aggregation and vasodilator-stimulated phosphoprotein (VASP) phosphorylation. The endpoint was composite cerebrocardiovascular events (CVEs). In 501 successfully followed-up patients, the median time from index stroke to enrollment was 181 days. There were 28 cardiovascular and 2 major bleeding events. There were no significant differences in the rates of cardiovascular events among the groups. CONCLUSIONS: Despite associations betweenCYP2C19variants and on-clopidogrel platelet reactivity, there was no significant difference in rates of CVEs in the chronic stroke phase among the 3 clopidogrel-metabolizing groups ofCYP2C19variants.

Autologous Bone Marrow Transplantation for Polymyositis Combined with Myasthenia Gravis and Aplastic Anemia: A Case Report.

This is the first report about a patient with concomitant polymyositis (PM), myasthenia gravis (MG), and aplastic anemia (AA). A 54-year-old male developed myalgia and muscle weakness, which gradually progressed over 2 months. He was persistently affected by MG and AA. Brachium magnetic resonance imaging showed increased signal intensity in the left triceps and deltoid muscles on short tau inversion recovery images. A muscle biopsy examination revealed perifascicular atrophication and inflammatory myopathy. We diagnosed the patient with PM combined with MG and AA. He was successfully treated with an autologous bone marrow transplantation (BMT). The present case suggests that BMT is a therapeutic option for PM, MG, and AA.

Blood biomarkers associated with neurological deterioration in patients with acute penetrating artery territory infarction: A multicenter prospective observational study.

Background and purpose Neurological deterioration in acute penetrating artery territory infarction is unpredictable and associated with unfavorable clinical outcomes. The aim of this prospective study was to clarify the cause of neurological worsening and predict clinical outcomes using blood biomarkers. Methods Eight Japanese stroke centers participated. Blood samples were obtained within 24 h (the first sampling) and on day 7 in hospital (the second sampling) in patients with penetrating artery territory infarction, arriving within two days of stroke onset. Symptomatic worsening was defined as a minimum increase of one point on the National Institutes of Health Stroke Scale. Poor outcome was defined as a modified Rankin Scale score of ≥3 at 90 days after ictus. Results Of the 89 patients, 25 (28%) had symptomatic worsening, and 25 (28%) had a poor outcome. Although tumor necrosis factor-alpha, high-sensitivity C-reactive protein levels were significantly increased in both groups at the second sampling, soluble lectin-like oxidized low-density lipoprotein receptor-1, CD40 ligand, and pro-adrenomedullin levels were significantly increased and ADAMTS13 activity was decreased in symptomatic worsening patients ( p < 0.05 for all). After multivariate adjustment, a low number of CD34+ cells at the first sampling was an independent predictor of poor outcome (odds ratio, 0.20; 95% confidence interval, 0.04-0.74, p = 0.011, per 1 cell/µl increase). Conclusions Blood biomarkers associated with atherosclerotic processes seem to be an indication for symptomatic worsening, and the number of CD34+ cells may help to predict three-month functional outcome in patients with penetrating artery territory infarction.

Successful Delayed Aortic Surgery for a Patient with Ischemic Stroke Secondary to Aortic Dissection.

The diagnosis of aortic dissection (AD) is sometimes difficult within the limited time window of recombinant tissue plasminogen activator (tPA) for ischemic stroke (IS). A 60-year-old man developed sudden left hemiparesis due to IS. During tPA infusion, his blood pressure dropped and consciousness declined. After transfer to our hospital, carotid duplex ultrasonography led to a diagnosis of AD. Emergency surgery was postponed because of the risk of hemorrhagic transformation. The patient successfully underwent aortic surgery on day 5 and was discharged with a remarkable improvement in his symptoms. Delayed surgery may avoid hemorrhagic transformation in patients with AD-induced IS who have received tPA.

Comparisons of l-cysteine and d-cysteine toxicity in 4-week repeated-dose toxicity studies of rats receiving daily oral administration.

Two 4-week repeated-dose toxicity studies were conducted to evaluate the potential toxicity of l-cysteine and d-cysteine. In one study, three groups of 6 male rats were each administered l-cysteine once daily by gavage at doses of 500, 1,000, or 2,000 mg/kg/day for 28 consecutive days. The control group was administered a 0.5% methylcellulose vehicle solution. The other study followed a similar protocol except that the experimental groups received d-cysteine. Toxicological observations showed that the l-cysteine-treated groups exhibited renal injuries such as basophilic tubules with eosinophilic material in the lumen, and there were increased numbers of basophilic tubules in all treated groups. In 1,000 or 2,000 mg/kg/day-treated groups, salivation and necropsy findings indicative of focal erosion in the stomach mucosa were found. Increases in reticulocyte counts were observed in the 2,000 mg/kg/day-treated group. Toxicological findings obtained for the d-cysteine-treated groups included anemia and renal injuries such as basophilic tubules with eosinophilic material in the lumen, increased numbers of basophilic tubules, and crystal deposition in the medulla in the 2,000 mg/kg/day-treated group. Additional findings included sperm granuloma in the epididymis, necropsy findings suggestive of focal erosion in the stomach mucosa, and salivation in the 1,000 or 2,000 mg/kg/day-treated groups. One rat in the 2,000 mg/kg/day-treated group died due to renal failure. In conclusion, the no-observed-adverse-effect levels (NOAELs) were estimated to be less than 500 mg/kg/day for l-cysteine and 500 mg/kg/day for d-cysteine under our study conditions. The toxicological profiles were similar for l-cysteine and d-cysteine; however, there were slight differences in the dose responses. The mechanisms underlying these differences remain to be determined.

Relationship between gustatory function and average number of taste buds per fungiform papilla measured by confocal laser scanning microscopy in humans.

The aim of this study was to elucidate the relationship between the gustatory function and average number of taste buds per fungiform papilla (FP) in humans. Systemically healthy volunteers (n = 211), pre-operative patients with chronic otitis media (n = 79), and postoperative patients, with or without a chorda tympani nerve (CTN) severed during middle ear surgery (n = 63), were included. Confocal laser scanning microscopy was employed to observe fungiform taste buds because it allows many FP to be observed non-invasively in a short period of time. Taste buds in an average of 10 FP in the midlateral region of the tongue were counted. In total, 3,849 FP were observed in 353 subjects. The gustatory function was measured by electrogustometry (EGM). An inverse relationship was found between the gustatory function and average number of fungiform taste buds per papilla. The healthy volunteers showed a lower EGM threshold (better gustatory function) and had more taste buds than did the patients with otitis media, and the patients with otitis media showed a lower EGM threshold and had more taste buds than did postoperative patients, reflecting the severity of damage to the CTN. It was concluded that the confocal laser scanning microscope is a very useful tool for using to observe a large number of taste buds non-invasively.

Gustatory Dysfunction and Decreased Number of Fungiform Taste Buds in Patients With Chronic Otitis Media With Cholesteatoma.

OBJECTIVE: To compare the number of fungiform taste buds among patients with chronic otitis media (COM), those with pars flaccida retraction type cholesteatoma, and those with pars tensa retraction type cholesteatoma in combination with gustatory function. METHODS: Thirty-seven patients with COM, 22 patients with pars flaccida retraction type cholesteatoma, and 17 patients with pars tensa retraction type cholesteatoma were included. An average of 10 fungiform papillae (FP) per patient in the midlateral region of the tongue were observed by confocal laser scanning microscopy in vivo, and the average number of taste buds were counted. Just before the observation of FP, electrogustometry (EGM) was performed to evaluate gustatory function. RESULTS: A significant decrease of the average number of fungiform taste buds and significant elevation of EGM thresholds were clarified in the pars tensa retraction type cholesteatoma group but not in the COM or pars flaccida type cholesteatoma group. CONCLUSION: It was suggested that some neurotoxic cytokines produced by cholesteatoma tissue might affect the CTN morphology, resulting in a decreased number of fungiform taste buds and elevation of EGM threshold in patients with pars tensa retraction type cholesteatoma.

Long-term Follow-up Results of Regeneration Process of Fungiform Taste Buds After Severing the Chorda Tympani Nerve During Middle Ear Surgery.

OBJECTIVE: To elucidate the regeneration process of fungiform taste buds after severing the chorda tympani nerve (CTN) by confocal laser scanning microscopy in vivo. METHODS: In 7 consecutive patients whose CTN was severed during tympanoplasty, an average of 10 fungiform papillae in the midlateral region of the tongue were periodically observed, and the number of taste buds was counted until 12 to 24 months after surgery. Gustatory function was assessed by EGM. RESULTS: EGM thresholds showed no response within 1 month after surgery in any patient. All taste buds had disappeared until 13 to 71 days after surgery. Regenerated taste buds were first detected 5 to 8 months after surgery in 5 of the 7 patients. EGM thresholds recovered to their preoperative values in 2 patients. In these 2 patients, the number of regenerated taste buds gradually increased in combination with a recovered taste function. However, a time lag existed between taste bud regeneration and taste function recovery. EGM thresholds did not recover in the other 3 patients with regenerated taste buds, suggesting that these taste buds were immature without gustatory function. CONCLUSION: The long-term regeneration process of fungiform taste buds could be clarified using confocal laser scanning microscopy.

Estimation of the Presence of Small Dense Lipoprotein Cholesterol in Acute Ischemic Stroke.

Small dense low-density lipoprotein (sdLDL) is an established risk factor in ischemic heart disease. However, its clinical significance in acute ischemic stroke (AIS) is uncertain. This study evaluates the prognostic value of the presence of sdLDL in patients with AIS by determining whether it contributes to clinical outcome or not. We studied 530 consecutive patients admitted within the first 48 hours after onset of ischemic stroke and 50 corresponding controls. Serum lipid parameters were measured on admission by standard laboratory methods. The percentage of AIS patients with sdLDL was significantly higher than the one of matched controls with sdLDL. Concerning comparisons between AIS patients with or without sdLDL, the percentages of males and patients with histories of smoking, hypertension, and cardiovascular disease were significantly higher in AIS patients with sdLDL. Concerning the grade of severity, modified Rankin Scale (mRS) on discharge was significantly higher in AIS patients with sdLDL. On logistic regression analysis, age (OR=2.29, P<0.001), male gender (OR=0.49, P<0.01), history of atrial fibrillation (OR=3.46, P<0.001), and the presence of sdLDL (OR=1.59, P<0.05) were significantly associated with poor prognosis (mRS on discharge >3). Our study showed that the presence of sdLDL might be independently associated with a poor prognosis after AIS.

Subsequent vascular events after ischemic stroke: the Japan Statin Treatment Against Recurrent Stroke-Longitudinal.

BACKGROUND: We undertook a multicenter cohort observational study to investigate the frequency and type of subsequent vascular events after an ischemic stroke and to compare the rates of vascular events between patients with and without hyperlipidemia. METHODS: This nationwide study was conducted in 19 hospitals participating in the Japan Standard Stroke Registry Study. We enrolled ischemic stroke patients, including those with a transient ischemic attack, who had not experienced any vascular events before enrollment after their ischemic stroke events. Each subject was observed prospectively from September 1, 2003, to October 1, 2005, or until a primary end point or death. Primary end points included subsequent fatal or nonfatal vascular events: stroke, angina pectoris, acute myocardial infarction, aortic aneurysm, or arteriosclerosis obliterans. RESULTS: A total of 449 patients (mean age, 67.6 years; 64.8% men) were enrolled in this study. Of the 41 vascular events observed during follow-up, 40 were stroke. The median observation period was 568 days. We found that patients with hyperlipidemia had a significantly higher rate of vascular events compared with those without hyperlipidemia according to the Kaplan-Meier method and the log-rank test (P = .013). Hyperlipidemia significantly increased the risk of vascular events (hazard ratio, 2.169 [1.125-4.312]; P = .021) according to the Cox proportional hazard model after adjusting for confounding factors (age, sex, days from ischemic stroke until enrollment, smoking habits, and daily drinking habits). CONCLUSIONS: This study demonstrated that stroke was the most common subsequent vascular event after ischemic stroke; the study also indicated that hyperlipidemia could be a risk factor for subsequent vascular events after ischemic stroke.

Intramuscular leukemic relapse: clinical signs and imaging findings. A multicentric analysis.

OBJECTIVE: Leukemia is a group of malignant diseases involving peripheral blood and bone marrow. Extramedullary tumor manifestation in leukemia can also occur. They more often involve lymph nodes, skin, and bones. Intramuscular leukemic relapse (ILR) is very unusual. The aim of this analysis was to summarize the reported data regarding clinical signs and radiological features of ILR. MATERIAL AND METHODS: The PubMed database was searched for publications related to ILR. After an analysis of all identified articles, 20 publications matched the inclusion criteria. The authors of the 20 publications were contacted and provided imaging of their cases for review. The following were recorded: age, gender, primary diagnosis, clinical signs, pattern, localization and size of the intramuscular leukemic relapse. Images of 16 patients were provided [8 computer tomographic (CT) images and 15 magnetic resonance images, MRI]. Furthermore, one patient with ILR was identified in our institutional database. Therefore, images of 17 patients were available for further analysis. RESULTS: Overall, 32 cases with ILR were included in the analysis. In most cases acute myeloid leukemia was diagnosed. Most ILRs were localized in the extremities (44 %) and in the extraocular muscles (44 %). Clinically, ILR manifested as local pain, swelling and muscle weakness. Radiologically, ILR presented most frequently with diffuse muscle infiltration. On postcontrast CT/MRI, most lesions demonstrated homogeneous enhancement. ILRs were hypo-/isointense on T1w and hyperintense on T2w images. CONCLUSION: ILR manifests commonly as focal pain, swelling and muscle weakness. ILR predominantly involved the extraocular musculature and the extremities. Radiologically, diffuse muscle infiltration was the most common imaging finding.

Degeneration process of fungiform taste buds after severing the human chorda tympani nerve--observation by confocal laser scanning microscopy.

OBJECTIVE: To elucidate the degeneration process of fungiform taste buds after severing the chorda tympani nerve (CTN) by confocal laser scanning microscopy in vivo. STUDY DESIGN: Prospective study. SETTING: University hospital. PATIENTS: Seven consecutive patients whose CTN was severed during tympanoplasty for middle ear cholesteatoma. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Preoperative and postoperative gustatory functions were assessed by electrogustometry (EGM). An average of 10 fungiform papillae (FP) in the midlateral region of the tongue were periodically observed, and the number of taste buds was counted using a confocal laser microscope. Among them, 2 to 3 reference FPs were selected based on the typical form of the FP or characteristic arrangements of taste pores. Observation was performed before surgery, 1 or 2 days after surgery, 2 or 3 times a week until 2 weeks after surgery, once a week between 2 and 4 weeks, and every 2 to 4 weeks thereafter until all taste buds had disappeared. RESULTS: EGM thresholds showed no response within 1 month after surgery in all patients. The initial change in the degeneration process was the disappearance of taste pores. The surface of taste buds became covered with epithelium. Finally, taste buds themselves atrofied and disappeared. The time course of degeneration differed depending upon individuals, each FP, and each taste bud. By employing the generalized linear mixed model under the Poisson distribution, it was calculated that all taste buds would disappear at around 50 days after surgery. CONCLUSION: Confocal laser scanning microscopy was useful for clarifying the degeneration process of fungiform taste buds.

Observation of regenerated fungiform taste buds after severing the chorda tympani nerve using confocal laser scanning microscopy in vivo.

OBJECTIVE: To evaluate whether regenerated fungiform taste buds after severing the chorda tympani nerve can be detected by confocal laser scanning microscopy in vivo. STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Six patients with a normal gustatory function (Group 1), 9 patients with taste function recovery after severing the CTN (Group 2), and 5 patients without taste function recovery (Group 3) were included. In Groups 2 and 3, canal wall up (closed) tympanoplasty or canal wall down with canal reconstruction tympanoplasty was performed in all patients. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: The severed nerves were readapted or approximated on the temporalis muscle fascia used to reconstruct the eardrum during surgery. Preoperative and postoperative gustatory functions were assessed using electrogustometry. Twelve to 260 months after severing the CTN, the surface of the midlateral region of the tongue was observed with a confocal laser microscope. RESULTS: EGM thresholds showed no response 1 month after surgery in all patients of Groups 2 and 3. In Group 2, EGM thresholds showed recovery 1 to 2 years after surgery and before confocal microscopy (-1.3 ± 6.5 dB). There was a significant difference between Group 1 (-5.7 ± 2.0 dB; p < 0.01) and Group 2. In Group 3, EGM thresholds showed no response for more than 2 years. In the control group (Group 1), 0 to 16 taste buds were observed in each FP, and 55 (79.7%) of 69 FP contained at least 1 taste bud. The mean number of taste bud per papilla was 3.7 ± 3.6. In patients with a recovered taste function (Group 2), 0 to 8 taste buds were observed in each FP. In this group, 54 (56.2%) of 94 FP contained at least 1 taste bud. The mean number of taste bud per papilla was 2.0 ± 2.2 (p < 0.01). In Group 3, without recovery, the FP was atrophied, and no taste bud was observed. CONCLUSION: Regenerated fungiform taste bud could be observed in vivo using confocal laser scanning microscopy, indicating that regenerated taste bud can be detected without biopsy.

Prevalence of metabolic syndrome in stroke patients: a prospective multicenter study in Japan.

AIMS: The fact that Metabolic Syndrome (MetS) increases the risk of atherosclerosis has been epidemiologically studied and proven; however, a prospective study on the prevalence of MetS in stroke patients has never been conducted because of the difficulty in diagnosis under critical illness in the acute phase. Therefore, we conducted a prospective multicenter study to investigate the prevalence of MetS in stroke patients with modified diagnostic criteria for MetS. METHODS: Stroke patients admitted in the seven participating Emergency and Critical Care Centers within the two years from April 2007 were registered in this study as a prospective multicenter study. Inclusion criteria were 50 to 89 year-old stroke patients who presented within three days from the onset of symptoms. A total of 992 subjects were classified according to the stroke type and the prevalence of MetS and the associated risk factors were investigated. The participants in a medical checkup without any history of a stroke were enrolled as the control group, and compared between the two groups. RESULTS: The prevalence of MetS as well as hyperglycemia and dyslipidemia in the infarction group was significantly higher than that in the non-stroke group. While the hemorrhage group showed no significant difference in the prevalence of MetS, only hypertension was significantly high. According to a subtype analysis, there is a significant correlation between waist circumference increment of the stroke patients and the prevalence of the risk factors of hypertension, hyperglycemia and dyslipidemia. CONCLUSIONS: Different risk factors are significantly related to the type of stroke.

Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan).

OBJECTIVE: To characterize the phenotype of spinocerebellar ataxia type 36 (SCA36), a novel dominant disorder (nicknamed "Asidan") caused by a hexanucleotide GGCCTG repeat expansion in intron 1 of the NOP56 gene. METHODS: We investigated the clinical, genetic, and neuropathologic characteristics of 18 patients with SCA36. We performed histologic evaluation of a muscle biopsy specimen from 1 patient with SCA36, and neuropathologic evaluation of an autopsied brain from another patient with SCA36. RESULTS: The (GGCCTG)n expansion was found in 18 ataxic patients from 9 families. The age at onset of ataxia was 53.1 ± 3.4 years, with the most frequent symptoms being truncal ataxia (100% of patients), ataxic dysarthria (100%), limb ataxia (93%), and hyperreflexia (79%). Tongue fasciculation and subsequent atrophy were found in 71% of cases, particularly in those of long duration. Skeletal muscle fasciculation and atrophy of the limbs and trunk were found in 57% of cases. Lower motor involvement was confirmed by EMG and muscle biopsy. The neuropathologic study revealed significant cerebellar Purkinje cell degeneration with obvious loss of lower motor neurons. Immunohistochemical analysis showed that NOP56 was localized to the nuclei of various neurons. Cytoplasmic or intranuclear inclusion staining of NOP56, TDP-43, and ataxin-2 was not observed in the remaining neurons. CONCLUSIONS: This is the first description of the unique clinical features of SCA36, a relatively pure cerebellar ataxia with progressive motor neuron involvement. Thus, SCA36 is a disease that stands at the crossroads of SCA and motor neuron disease.

PSP as distinguished from CBD, MSA-P and PD by clinical and imaging differences at an early stage.

OBJECTIVE: Because it is often difficult to precisely diagnose and distinguish progressive supranuclear palsy (PSP) from corticobasal degeneration (CBD), multiple system atrophy-parkinsonism (MSA-P) and Parkinson's disease (PD) at the onset of the disease, we compared the patients and clarified the features of these diseases. METHODS: We compared 77 PSP, 26 CBD, 26 MSA-P and 166 PD patients from clinical and imaging points of view including cerebral blood flow (CBF) in the frontal eye field. RESULTS: The clinical characteristics of PSP were supranuclear gaze disturbance, optokinetic nystagmus (OKN) impairment and falls at the first visit. On head MRI, midbrain tegmentum atrophy was much more frequently detected in PSP than in all of the other groups. Heart-to-mediastinum average count ratio (H/M) in iodine-123 meta-iodobenzyl guanidine ((123)I-MIBG) myocardial scintigraphy was not decreased in PSP, CBD, MSA-P and PD-Yahr 1 (-1), but patients of PD-2, 3, 4 and 5 showed a significant decrease compared with the PSP group. The CBF in the left frontal eye field of PD-3 group and that in right frontal eye field of PD-3 and PD-4 groups were lower than that of PSP group, although other groups showed a tendency without a significant decrease compared with PSP group. CONCLUSION: PSP is distinguishable from CBD, MSA-P and PD even at the early stage with extra-ocular movement (EOM) disturbance, falls, atrophy of the midbrain tegmentum, and H/M in (123)I-MIBG myocardial scintigraphy, and the reduction of CBF in area 8 could serve as a supplemental diagnostic method for distinguishing PSP from PD-3 or PD-4.

Length of nerve gap defects correlates with incidence of nerve regeneration but not with recovery of taste function in patients with severed chorda tympani nerve.

OBJECTIVE: To evaluate the relationship between the length of nerve gap defects, incidence of nerve regeneration, and recovery of gustatory function after severing the chorda tympani nerve (CTN). STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Eighty-eight consecutive patients whose CTNs were severed during primary surgery and who underwent secondary surgery were included. Proximal and distal stumps of severed nerves were readapted or approximated during surgery. INTERVENTION: Therapeutic. MAIN OUTCOME MEASURES: Before and after surgery, the taste function was periodically evaluated using electrogustometry. Nerve gaps were classified into 4 groups: readaptation (Group 1), 1 to 3 mm (Group 2), 4 to 6 mm (Group 3), and more than 7 mm (Group 4). RESULTS: Regenerated nerves in the tympanic segment were detected in 36 (41%) of the 88 patients during secondary surgery. The incidence of nerve regeneration was 100% (10/10) in Group 1, 45% (10/22) in Group 2, 47% (9/19) in Group 3, and 19% (7/37) in Group 4. There was a significant difference between the length of nerve gap defects and incidence of nerve regeneration (p < 0.001). In the 36 patients with a regenerated CTN, the incidence of gustatory function recovery was 60% (6/10) in Group 1, 50% (5/10) in Group 2, 56% (5/9) in Group 3, and 43% (3/7) in Group 4. There was no significant difference between the length of nerve gap defects and incidence of taste function recovery. CONCLUSION: Reconstruction of a severed CTN is very important for regeneration. However, the regenerated CTN in the tympanic segment does not always reinnervate the fungiform papillae.

Morphology of human fungiform papillae after severing chorda tympani nerve.

OBJECTIVES: We aimed to clarify the postoperative morphology of the fungiform papillae (FP) of the tongue in patients who recovered gustatory function after the chorda tympani nerve was severed during middle ear surgery. METHODS: Fifty-four patients with normal preoperative gustatory function measured by electrogustometry (EGM) were included. The proximal and distal stumps of the severed nerves were re-adapted or re-approximated during surgery to promote regeneration of the nerve. The EGM thresholds over 2 years after surgery were compared with preoperative values. At the same time, the morphological characteristics of the FP in the midlateral region of the tongue were recorded with a digital microscope. RESULTS: One month after surgery, EGM showed no response in any patients. At a time point of more than 2 years, the FP showed complete atrophy and no response to EGM on the surgical side in 21 of the 54 patients. In 16 patients who showed complete recovery of the EGM threshold (below 20 microA), the FP showed an almost normal appearance, and the mean number of FP was 77.5% (10 +/- 4.1 papillae per square centimeter) of that on the contralateral side (12.9 +/- 4.9 papillae per square centimeter; p > 0.05). CONCLUSIONS: The morphology of the FP was maintained in patients who recovered gustatory function after the chorda tympani nerve was severed. Because the results indicate regeneration of the taste buds, further observation is needed to detect regenerated taste buds in the FP.