Fluctuations in Upper and Lower Body Movement during Walking in Normal Pressure Hydrocephalus and Parkinson's Disease Assessed by Motion Capture with a Smartphone Application, TDPT-GT.

We aimed to capture the fluctuations in the dynamics of body positions and find the characteristics of them in patients with idiopathic normal pressure hydrocephalus (iNPH) and Parkinson's disease (PD). With the motion-capture application (TDPT-GT) generating 30 Hz coordinates at 27 points on the body, walking in a circle 1 m in diameter was recorded for 23 of iNPH, 23 of PD, and 92 controls. For 128 frames of calculated distances from the navel to the other points, after the Fourier transforms, the slopes (the representatives of fractality) were obtained from the graph plotting the power spectral density against the frequency in log-log coordinates. Differences in the average slopes were tested by one-way ANOVA and multiple comparisons between every two groups. A decrease in the absolute slope value indicates a departure from the 1/f noise characteristic observed in healthy variations. Significant differences in the patient groups and controls were found in all body positions, where patients always showed smaller absolute values. Our system could measure the whole body's movement and temporal variations during walking. The impaired fluctuations of body movement in the upper and lower body may contribute to gait and balance disorders in patients.

Echolalia in patients with primary progressive aphasia.

OBJECTIVE: This study aimed to examine echolalia and its related symptoms and brain lesions in primary progressive aphasia (PPA). METHODS: Forty-five patients with PPA were included: 19 nonfluent/agrammatic variant PPA (nfvPPA), 5 semantic variant PPA, 7 logopenic variant PPA, and 14 unclassified PPA patients. We detected echolalia in unstructured conversations. An evaluation of language function and the presence of parkinsonism, grasp reflex, imitation behaviour, and disinhibition were assessed. We also measured regional cerebral blood flow (rCBF) using single-photon emission computed tomography. RESULTS: Echolalia was observed in 12 nfvPPA and 2 unclassified PPA patients. All patients showed mitigated echolalia. We compared nfvPPA patients with echolalia (echolalia group) to those without echolalia (non-echolalia group). The median age of the echolalia group was significantly lower than that of the non-echolalia group, and the echolalia group showed a significantly worse auditory comprehension performance than the non-echolalia group. In contrast, the performance of repetition tasks was not different between the two groups. The prevalence of imitation behaviour in the echolalia group was significantly higher than that in the non-echolalia group. The rCBFs in the bilateral pre-supplementary motor area and bilateral middle cingulate cortex in the echolalia group were significantly lower than those in the non-echolalia group. CONCLUSIONS: These findings suggest that echolalia is characteristic of nfvPPA patients with impaired comprehension. Reduced inhibition of the medial frontal cortex with release activity of the anterior perisylvian area account for the emergence of echolalia.

Reduced default mode network connectivity relative to white matter integrity is associated with poor cognitive outcomes in patients with idiopathic normal pressure hydrocephalus.

BACKGROUND: The aim of this study was to investigate whether default mode network (DMN) connectivity and brain white matter integrity at baseline were associated with severe cognitive impairments at baseline and poor cognitive outcomes after shunt placement in patients with idiopathic normal pressure hydrocephalus (iNPH). METHODS: Twenty consecutive patients with iNPH whose symptoms were followed for 6 months after shunt placement and 10 healthy controls (HCs) were enrolled. DMN connectivity and brain white matter integrity at baseline in the patients with iNPH and HCs were detected by using resting-state functional magnetic resonance imaging (MRI) with independent component analysis and diffusion tensor imaging, respectively, and these MRI indexes were compared between the patients with iNPH and HCs. Performance on neuropsychological tests for memory and executive function and on the gait test was assessed in the patients with iNPH at baseline and 6 months after shunt placement. We divided the patients with iNPH into the relatively preserved and reduced DMN connectivity groups using the MRI indexes for DMN connectivity and brain white matter integrity, and the clinical measures were compared between the relatively preserved and reduced DMN connectivity groups. RESULTS: Mean DMN connectivity in the iNPH group was significantly lower than that in the HC group and was significantly positively correlated with Rey auditory verbal learning test (RAVLT) immediate recall scores and frontal assessment battery (FAB) scores. Mean fractional anisotropy of the whole-brain white matter skeleton in the iNPH group was significantly lower than that in the HC group. The reduced DMN connectivity group showed significantly worse performance on the RAVLT at baseline and significantly worse improvement in the RAVLT immediate recall and recognition scores and the FAB scores than the preserved DMN connectivity group. Moreover, the RAVLT recognition score highly discriminated patients with relatively preserved DMN connectivity from those with relatively reduced DMN connectivity. CONCLUSIONS: Our findings indicated that iNPH patients with reduced DMN connectivity relative to the severity of brain white matter disruption have severe memory deficits at baseline and poorer cognitive outcomes after shunt placement. However, further larger-scale studies are needed to confirm these findings.

Improvement in callosal disconnection syndrome with recovery of callosal connectivity.

Recent advancements in radiological techniques have enabled the observation of the topographic distribution of the human corpus callosum. However, its functional connectivity remains to be elucidated. The symptoms of callosal disconnection syndrome (CDS) can potentially reveal the functional connections between the cerebral hemispheres. Herein, we report a patient with CDS, whose callosal lesion was restricted to the posterior midbody, isthmus, and an anterior part of the dorsal splenium. A 53-year-old right-handed woman demonstrated CDS following cerebral infarction associated with subarachnoid hemorrhage. She exhibited CDS including ideomotor apraxia, and tactile anomia with the left hand, cross-replication of hand postures, cross-localization of the fingers, and constructional impairment with the right hand. Six months after onset, the left-handed ideomotor apraxia on imitation improved, but that to command did not, which indicated the difference in the nature of the transcallosal connections between ideomotor apraxia on imitation and ideomotor apraxia to command. Longitudinal CDS observation and corpus callosum tractography will prove useful in expanding our understanding of the nature of the organization of interhemispheric information transference.

[Preoperative Predictors of Outcomes of Cerebrospinal Fluid Shunt Surgery in Patients with Idiopathic Normal Pressure Hydrocephalus].

The disproportionately enlarged subarachnoid space hydrocephalus (DESH) findings on cranial images are useful to predict cerebrospinal fluid (CSF) shunt responsiveness to some extent in patients with idiopathic normal-pressure hydrocephalus (iNPH). However, recent studies show that patients with iNPH often have concomitant Alzheimer's or Lewy body disease regardless of DESH findings, which may be associated with poor outcomes of CSF shunt surgery. Moreover, long-term outcomes after CSF shunt surgery in patients with iNPH, which is one of the most important issues to be addressed for effective treatment of iNPH, remain unknown.

A decade with anomic primary progressive aphasia.

Some patients with primary progressive aphasia (PPA) demonstrate only anomia. The lack of longitudinal observations of anomic PPA precluded us from determining whether progressive anomic aphasia was simply an early stage of semantic or logopenic variants, or a relatively independent variant. Herein, we report the 10-year clinical course of a patient with PPA who presented with pure anomic aphasia for 9 years. He is a right-handed man with anomia, who noticed word-finding difficulty at age 73. He was admitted to the hospital at age 77. On admission, the patient showed pure anomic aphasia with preserved other language function. Episodic memory and visuospatial function were preserved. Magnetic resonance imaging (MRI) revealed left temporal lobe atrophy. At 82 years of age, the patient presented with pure anomic aphasia. At 83 years old, he showed mild impairment in word comprehension and semantic memory, in addition to anomia. MRI demonstrated further atrophy in the bilateral anterior temporal lobes, predominantly on the left side. This case suggests the possibility of slowly progressive, late-onset anomic PPA, which could be differentiated from the early stage of semantic or logopenic variants.

The grasp reflex in patients with idiopathic normal pressure hydrocephalus.

OBJECTIVE: To investigate the prevalence and intensity of grasp reflexes and to examine changes in these reflexes after shunt surgery in patients with idiopathic normal pressure hydrocephalus (iNPH). METHODS: We enrolled 147 patients with probable iNPH. A standard procedure was used to determine the presence of grasp reflexes, and the intensity of these reflexes was assessed using a four-category classification. Clinical rating scales and their correlation with grasp reflexes were also evaluated. Grasp reflexes were reassessed in 72 patients 1 year after surgery. RESULTS: We found that approximately 50.3% of patients with iNPH exhibited a positive grasp reflex. Among these patients, 69% exhibited bilateral positivity, while the remaining patients showed unilateral positivity. Furthermore, the intensity of the grasp reflex was significantly correlated with the severity of gait and with cognitive, urinary, motor, and behavioural symptoms. Surgical interventions led to a reduction (41.7%) or maintenance (30.6%) of the reflex intensity in 72.3% of iNPH patients. The changes in reflex intensity showed significant positive correlations with changes in the number of steps of the Timed Up and Go test and Trail Making Test-A scores but not with changes in total scores on the iNPH Grading Scale. CONCLUSION: This retrospective study identified grasp reflexes as a highly prevalent phenomenon in patients with iNPH. These reflexes can assist in evaluating the severity of various symptoms, including cognitive, gait, urinary, motor and emotional symptoms.

Cognitive dysfunction associated with anti-glutamic acid decarboxylase autoimmunity: a case-control study.

BACKGROUND: Glutamic acid decarboxylase (GAD) is the rate-limiting enzyme in the synthesis of γ-aminobutyric acid (GABA). Anti-GAD antibodies (GADA) are associated with the progression of stiff person syndrome and other neurological diseases, as well as the immune-mediated (type 1) diabetes. GABA is one of the most widely distributed neurotransmitters, but the non-motor symptoms of GADA-positive patients are not well understood. Diabetes is increasingly recognized as a risk factor for dementia; however, the relationship between diabetes and dementia is controversial.The objective of this study was to assess cognitive function in patients with GADA-positive diabetes using subjects with GADA-negative type 2 diabetes as controls. METHODS: Twenty-one patients with GADA-positive diabetes (mean age 52.5 ± 12.3 years, mean duration 7.7 ± 6.6 years) and 19 control subjects with GADA-negative type 2 diabetes (mean age 53.4 ± 8.9 years, mean duration 12.5 ± 6.7) were included in the study. The subjects underwent extensive neuropsychological testing and brain MRI. RESULTS: The neuropsychological test scores were lower in the GADA-positive group than the control group (GADA-negative). Twelve subjects (57%) in the GADA group and 4 subjects (21%) in the control group had low performances (p = 0.027). No statistically significant differences were found between the GADA and control groups regarding demographics, diabetic severity cardiovascular risks, cerebral T2 hyperintensities, white matter volume and gray matter volume. CONCLUSIONS: Our study showed that GADA-positive diabetic patients have an increased risk of cognitive decline compared to patients with type 2 diabetes of comparable diabetic severity. It also showed that GADA may be associated with isolated cognitive decline in the absence of other neurological complications.

Severe olfactory dysfunction is a prodromal symptom of dementia associated with Parkinson's disease: a 3 year longitudinal study.

Dementia is one of the most debilitating symptoms of Parkinson's disease. A recent longitudinal study suggests that up to 80% of patients with Parkinson's disease will eventually develop dementia. Despite its clinical importance, the development of dementia is still difficult to predict at early stages. We previously identified olfactory dysfunction as one of the most important indicators of cortical hypometabolism in Parkinson's disease. In this study, we investigated the possible associations between olfactory dysfunction and the risk of developing dementia within a 3-year observation period. Forty-four patients with Parkinson's disease without dementia underwent the odour stick identification test for Japanese, memory and visuoperceptual assessments, (18)F-fluorodeoxyglucose positron emission tomography scans and magnetic resonance imaging scans at baseline and 3 years later. A subgroup of patients with Parkinson's disease who exhibited severe hyposmia at baseline showed more pronounced cognitive decline at the follow-up survey. By the end of the study, 10 of 44 patients with Parkinson's disease had developed dementia, all of whom had severe hyposmia at baseline. The multivariate logistic analysis identified severe hyposmia and visuoperceptual impairment as independent risk factors for subsequent dementia within 3 years. The patients with severe hyposmia had an 18.7-fold increase in their risk of dementia for each 1 SD (2.8) decrease in the score of odour stick identification test for Japanese. We also found an association between severe hyposmia and a characteristic distribution of cerebral metabolic decline, which was identical to that of dementia associated with Parkinson's disease. Furthermore, volumetric magnetic resonance imaging analyses demonstrated close relationships between olfactory dysfunction and the atrophy of focal brain structures, including the amygdala and other limbic structures. Together, our findings suggest that brain regions related to olfactory function are closely associated with cognitive decline and that severe hyposmia is a prominent clinical feature that predicts the subsequent development of Parkinson's disease dementia.

Auditory phonological identification impairment in primary progressive aphasia.

OBJECTIVE: To examine the audiological characteristics and neuroanatomical regions associated with auditory phonological identification impairment in primary progressive aphasia (PPA). METHODS: Twenty-seven patients with PPA [13 non-fluent/agrammatic variant PPA (nfvPPA), three logopenic variant PPA (lvPPA), seven semantic variant PPA (svPPA), and four mixed type PPA] were included in the study. Neuropsychological, language, audiological, and neuroradiological examinations were also performed. Auditory function examinations consisted of a pure-tone threshold test, a phonological identification task, and temporal auditory acuity tests, such as click counting or fusion. As an evaluation value of phonological identification ability, we calculated the discrepancy scores, which were the smaller discrepancy (left or right ear) in phonological identification ability scores between measured and expected values from the pure-tone threshold. In the neuroradiological examination, we evaluated the regional cerebral blood flow using 123I-iodoamphetamine single-photon emission computed tomography. RESULTS: Eight of the 27 patients were allocated to the impaired phonological identification group, and four were considered to have significant impairment on further analysis. Two of these patients, one with lvPPA and one with mixed type of lvPPA and nfvPPA, showed apparent phonological identification deficits that could be observed in daily life. The discrepancy scores were not significantly related to the results of neuropsychological, language, or any other auditory examinations, except for the click counting score in the left ear. Voxel-based correlation analyses revealed that regional cerebral blood flow in the bilateral superior temporal gyrus and bilateral primary auditory cortex was significantly and positively correlated with phonological identification ability. CONCLUSIONS: Our results suggest that progressive dysfunction of the bilateral superior temporal gyrus and bilateral primary auditory cortex due to neurodegenerative diseases leads to phonological identification impairment in PPA syndrome.

Ventriculoperitoneal Shunt Failure 3-year after Shunt Surgery Caused by Migration of Detached Ventricular Catheter into the Cranium: A Case Study of Idiopathic Normal-pressure Hydrocephalus.

Idiopathic normal-pressure hydrocephalus (iNPH) is a neurological disorder that typically presents with gait disturbance, cognitive impairment, and urinary incontinence. Although most patients respond to cerebrospinal-fluid shunting, some do not react well because of shunt failure. A 77-year-old female with iNPH underwent ventriculoperitoneal shunt implantation, and her gait impairment, cognitive dysfunction, and urge urinary incontinence improved. However, 3 years after shunting (at the age of 80), her symptoms gradually recurred for 3 months and she did not respond to shunt valve adjustment. Imaging studies revealed that the ventricular catheter detached from the shunt valve and migrated into the cranium. With immediate revision of the ventriculoperitoneal shunt, her gait disturbance, cognitive dysfunction, and urinary incontinence improved. When a patient whose symptoms have been relieved by cerebrospinal-fluid shunting experiences an exacerbation, it is important to suspect shunt failure, even if many years have passed since the surgery. Identifying the position of the catheter is crucial to determine the cause of shunt failure. Prompt shunt surgery for iNPH can be beneficial, even in elderly patients.

Buccofacial apraxia in primary progressive aphasia.

Buccofacial apraxia (BFA) is associated with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) as well as with the severity of apraxia of speech (AOS), a core symptom of nfvPPA. However, an association with agrammatism has not been established. The aim of this study was to examine the association between BFA and agrammatism in nfvPPA and to determine differences in atrophic regions in primary progressive aphasia (PPA) with and without BFA. Seventy-four patients with PPA were recruited, including 34, 15, 10, and 15 patients with nfvPPA, semantic variant PPA, logopenic variant PPA, and unclassified PPA, respectively. All patients underwent language examination and BFA evaluations. Voxel-based morphometry (VBM) was performed to determine whether atrophy of a specific lesion correlated with the presence of BFA. BFA was observed in 20 and 3 patients with nfvPPA and unclassified PPA, respectively. In a comparison of patients with nfvPPA with and without BFA, the BFA group showed significantly worse spontaneous speech and writing in the Western Aphasia Battery. The agrammatism ratio or the ratio of agrammatic errors to the total number of particles was higher in the BFA group; however, the severity of prosodic and phonetic components of AOS did not differ between the two groups. VBM showed that the severity of BFA correlated with atrophy of the opercular and triangular areas of the inferior frontal gyrus to a part of the left middle frontal gyrus. BFA has a different anatomical basis from AOS in patients with nfvPPA and that BFA is characterized by more anterior degeneration compared to that of AOS.

Olfactory Dysfunction, an Often Neglected Symptom of Hydrocephalus: Experience from a Case of Late-Onset Idiopathic Aqueductal Stenosis.

Disturbance of smell is often accompanied with common neurodegenerative diseases such as Parkinson's and Alzheimer's diseases. In addition, patients with head trauma, intracranial tumors, and hydrocephalus can also develop olfactory dysfunction, and some of which can improve with treatment of the underlying disease. In clinical practice, few patients complain of smell disturbances, thus olfactory dysfunction is often overshadowed by visible motor symptoms. Herein, we report a case of late-onset idiopathic aqueductal stenosis, a rare form of adult-onset hydrocephalus in which olfactory dysfunction and gait disturbance was markedly improved after endoscopic ventriculostomy. This case report is expected to make more physicians aware that hydrocephalus can cause olfactory dysfunction and that it can be corrected postoperatively. Furthermore, in addition to motor and neuropsychological function, olfactory function test might be useful for functional assessment before and after surgical treatment of hydrocephalus.

Interhemispheric asymmetrical change in gray matter volume in patients with unilateral hippocampal sclerosis.

Objectives: To clarify the interhemispheric asymmetrical change in gray matter volume (GMV) in unilateral hippocampal sclerosis (HS), we compared changes in GMV relative to normal subjects between the HS and contralateral or non-HS sides. Material and Methods: Forty-five patients with unilateral HS and 30 healthy subjects were enrolled. We quantified changes in GMV in the patients with HS as compared to GMV in the normal subjects by introducing the Z-score (Z-GMV) in each region or region of interest in unilateral HS. Then, we assessed the asymmetrically decreased regions, that is, regions with significantly higher Z-GMV on the HS side than the contralateral or non-HS side. Z-GMV was calculated according to the two templates of 58 regions per hemisphere covering the whole brain by anatomical automatic labeling (AAL) and 78 regions per cerebral hemisphere using the Anatomy Toolbox. Results: Seven and four regions in AAL and 17 and 11 regions in Anatomy Toolbox were asymmetrically decreased in the Left Hand Side (LHS) and Right Hand Side (RHS), respectively. Hippocampus and Caudate in AAL, five subregions of the hippocampus (CA1-3, Dentate Gyrus and hippocampus-amygdala-transition-area and 4 extrahippocampal regions including two subregions in amygdala (CM: Centromedial, SF: Superficial), basal forebrain (BF) (Ch4), and thalamus (temporal) in anatomy toolbox were common among LHS and RHS concerning asymmetrically decreased regions. Conclusion: By introducing Z-GMV, we demonstrated the regions with asymmetrically decreased GMV in LHS and RHS, and found that the hippocampus and extrahippocampal regions, including the BF, were the common asymmetrically decreased regions among LHS and RHS.

Dyslexia and dysgraphia of primary progressive aphasia in Chinese: A systematic review.

Introduction: Currently, little is known about Chinese-speaking primary progressive aphasia (PPA) patients compared to patients who speak Indo-European languages. We examined the demographics and clinical manifestations, particularly reading and writing characteristics, of Chinese patients with PPA over the last two decades to establish a comprehensive profile and improve diagnosis and care. Methods: We reviewed the demographic features, clinical manifestations, and radiological features of Chinese-speaking PPA patients from 56 articles published since 1994. We then summarized the specific reading and writing errors of Chinese-speaking patients. Results: The average age of onset for Chinese-speaking patients was in their early 60's, and there were slightly more male patients than female patients. The core symptoms and images of Chinese-speaking patients were similar to those of patients who speak Indo-European languages. Reading and writing error patterns differed due to Chinese's distinct tone and orthography. The types of reading errors reported in Chinese-speaking patients with PPA included tonal errors, regularization errors, visually related errors, semantic errors, phonological errors, unrelated errors, and non-response. Among these errors, regularization errors were the most common in semantic variant PPA, and tonal errors were specific to Chinese. Writing errors mainly consisted of non-character errors (stroke, radical/component, visual, pictograph, dyskinetic errors, and spatial errors), phonologically plausible errors, orthographically similar errors, semantic errors, compound word errors, sequence errors, unrelated errors, and non-response. Conclusion: This paper provides the latest comprehensive demographic information and unique presentations on the reading and writing of Chinese-speaking patients with PPA. More detailed studies are needed to address the frequency of errors in reading and writing and their anatomical substrates.

Case Report: Semantic Variant Primary Progressive Aphasia With Impaired Verbal Word Discrimination.

Some patients with primary progressive aphasia (PPA) present with various types of hearing deficits. Research on the auditory function and speech sounds in PPA, including temporal, phonemic, and prosodic processing, revealed impairment in some of these auditory processes. Many patients with PPA who present with impaired word recognition subsequently developed non-fluent variant PPA. Herein, we present a patient with semantic variant PPA (svPPA) who demonstrated impaired verbal word discrimination. Audiological examinations revealed normal auditory brainstem responses and slightly impaired pure-tone perception. By contrast, verbal word discrimination and monosyllable identification were impaired, and temporal auditory acuity deteriorated. Analyses of brain magnetic resonance images revealed a significant decrease in the gray matter volume in bilateral superior temporal areas, predominantly on the left, compared with those of patients with typical svPPA, which appeared to be associated with impaired word recognition in our patient.

Mirror writing and cortical hypometabolism in Parkinson's disease.

Mirror writing (MW) is the production of individual letters, words, or word strings in the reverse direction. Parkinson's disease (PD) is a progressive neurodegenerative disorder, and high MW rates have been reported in patients with PD. Thus, the present study sought to identify the factors that cause MW in patients with PD. We examined the frequency of MW in patients with PD and investigated the area of the brain where such frequency inversely correlates with reduced regional cerebral metabolic rates of glucose (rCMRglc). We also examined whether this area satisfied the motor and visual monitoring hypotheses of MW that have been presented in previous studies. Thirty-six subjects with idiopathic PD and 23 healthy controls were included in the study. We asked the participants to write down words, numerals, and sentences from left to right using their dominant and non-dominant hands. Patients with PD underwent an 18F-fluorodeoxyglucose positron emission tomography scan to measure the rCMRglc. Neither the patients with PD nor the healthy subjects exhibited MW in the use of the right hand. In the use of the left hand, MW occurred in 15 of the 36 patients with PD, but in none of the healthy controls. The right intraparietal sulcus was identified as the area where rCMRglc was inversely correlated with the number of left-right reversed characters. Previous functional imaging studies have suggested that the right superior parietal cortex and intraparietal sulcus play an important role in recognizing left-right reversed letters. Therefore, dysfunction in the intraparietal sulcus may hinder the recognition of left-right reversed characters, resulting in MW. Consequently, our findings in patients with PD are consistent with the visual-monitoring hypothesis of MW.

Risk factors for unfavourable outcomes after shunt surgery in patients with idiopathic normal-pressure hydrocephalus.

A number of vascular risk factors (VRFs) have been reported to be associated with idiopathic normal-pressure hydrocephalus (iNPH), but it remains unclear whether these VRFs are related to patient outcomes after shunt surgery. Therefore, we investigated the risk factors for unfavourable outcomes after shunt surgery in iNPH patients using two samples from Tohoku University Hospital and from a multicentre prospective trial of lumboperitoneal (LP) shunt surgery for patients with iNPH (SINPHONI-2). We enrolled 158 iNPH patients. We compared the prevalence of VRFs and clinical measures between patients with favourable and unfavourable outcomes and identified predictors of unfavourable outcomes using multivariate logistic regression analyses. The presence of hypertension, longer disease duration, more severe urinary dysfunction, and a lower Evans' index were predictors of unfavourable outcomes after shunt surgery. In addition, hypertension and longer disease duration were also predictors in patients with independent walking, and a lower Evans' index was the only predictor in patients who needed assistance to walk or could not walk. Our findings indicate that hypertension is the only VRF related to unfavourable outcomes after shunt surgery in iNPH patients. Larger-scale studies are needed to elucidate the reason why hypertension can affect the irreversibility of symptoms after shunt placement.

Facial memory ability and self-awareness in patients with temporal lobe epilepsy after anterior temporal lobectomy.

Anterior temporal lobectomy (ATL) is the most common surgical treatment for drug-resistant temporal lobe epilepsy (TLE). Right ATL has been reported to reduce facial memory ability in patients with TLE, as indicated by poor performance on the Warrington Recognition Memory Test for Faces (RMF), which is commonly used to evaluate visual memory in these patients. However, little is known about whether patients with TLE exhibit difficulties in identifying faces in daily life after ATL. The aim of this study was to investigate facial memory ability and self-awareness of face identification difficulties in patients with TLE after ATL. Sixteen patients with TLE after right ATL, 14 patients with TLE after left ATL, and 29 healthy controls were enrolled in this study. We developed the multiview face recognition test (MFRT), which comprises a learning phase (one or three frontal face images without external facial feature information) and a recognition phase (frontal, oblique, or noise-masked face images). Facial memory abilities were examined in all participants using the MFRT and RMF, and self-awareness of difficulties in face identification was evaluated using the 20-item prosopagnosia index (PI20), which has been widely used to assess developmental prosopagnosia. The MFRT performance in patients with TLE after ATL was significantly worse than that in healthy controls regardless of the resected side, whereas the RMF scores in patients with TLE were significantly worse than those in healthy controls only after right ATL. The MFRT performance in patients with TLE after both left and right ATL was more influenced by working memory load than that in healthy controls. The PI20 scores revealed that patients with TLE after left ATL were aware of their difficulties in identifying faces. These findings suggest that patients with TLE not only after right ATL but also after left ATL might have difficulties in face identification.

Neural substrates underlying progressive micrographia in Parkinson's disease.

INTRODUCTION: The neural substrates associated with the development of micrographia remain unknown. We aimed to elucidate the neural substrates underlying micrographia in Parkinson's disease (PD) patients. METHODS: Forty PD patients and 20 healthy controls underwent handwriting tests that involved free writing and copying. We measured the size of each letter and the resting cerebral glucose metabolic rate of the PD patients and another group of age- and sex-matched 14 healthy controls (HCs), who had not participated in the writing tests, using resting-state 18F-fluorodeoxyglucose positron emission tomography. RESULTS: In the PD patients, the prevalence of consistent micrographia (CM) associated with free writing was 2.5% for both tasks. Alternatively, the prevalence of progressive micrographia (PM) was 15% for free writing and 17.5% for copying. In the PD patients, there was no significant difference in the letter sizes between these tasks, whereas the variability of the letter sizes for copying was significantly different from that for free writing. The means and decrements in letter sizes in either task were not significantly correlated with the severity of brady/hypokinesia in the PD patients. For free writing, the PD patients with PM showed glucose hypometabolism in the anterior part of the right middle cingulate cortex, including the rostral cingulate motor area, compared with those without PM. For copying, the PD patients with PM showed glucose hypometabolism in the right superior occipital gyrus, including V3A, compared with those without PM. CONCLUSIONS: These findings suggest that PM in free writing in PD patients is caused by the difficulty of monitoring whether the actual handwriting movements are desirable for maintaining letter size during self-paced handwriting. By contrast, PM in copying in PD patients is evoked by a lack of visual information about the personal handwriting and hand motions that are used as cues for maintaining letter sizes.