Symptomatology and Neuropathology of patients presenting with focal cortical signs.

Here, we describe two patients who presented with focal cortical signs and underwent neuropathological examination. Case 1 was a 73-year-old woman with progressive speech disorder and abnormal behavior. She showed agraphia of the frontal lobe type, featured by the omission of kana letters when writing, other than pyramidal tract signs, pseudobulbar palsy, and frontal lobe dementia. Neuropathological examination, including TAR DNA-binding protein 43 (TDP-43) immunohistochemistry, revealed bilateral frontal and anterior temporal lobe lesions accentuated in the precentral gyrus and posterior part of the middle frontal gyrus. Both upper and lower motor neurons showed pathological changes compatible with amyotrophic lateral sclerosis. Case 2 was a 62-year-old man with progressive speech disorder and hand clumsiness. He had a motor speech disorder, compatible with apraxia of speech, and limb apraxia of the limb-kinetic and ideomotor type. Neuropathological examination revealed degeneration in the left frontal lobe, including the precentral gyrus, anterior temporal, and parietal lobe cortices. Moreover, numerous argyrophilic neuronal intracytoplasmic inclusions (Pick body) and ballooned neurons were observed in these lesions and the limbic system. The pathological diagnosis was Pick disease involving the peri-Rolandic area and parietal lobe. In these two cases, the distribution of neuropathological changes in the cerebral cortices correlated with the clinical symptoms observed.

Stopwatch training improves cognitive functions in patients with Parkinson's disease.

Parkinson's disease (PD) impairs various cognitive functions, including time perception. Dysfunctional time perception in PD is poorly understood, and no study has investigated the rehabilitation of time perception in patients with PD. We aimed to induce the recovery of time perception in PD patients and investigated the potential relationship between recovery and cognitive functions/domains other than time perception. Sixty patients with PD (27 females) and 20 healthy controls (10 females) were recruited. The participants underwent a feedback training protocol for 4 weeks to improve the accuracy of subjective spatial distance or time duration using a ruler or stopwatch, respectively. They participated in three tests at weekly intervals, each comprising 10 types of cognitive tasks and assessments. After duration feedback training for 1 month, performance on the Go/No-go task, Stroop task, and impulsivity assessment improved in patients with PD, while no effect was observed after distance feedback training. Additionally, the effect of training on duration production correlated with extended reaction time and improved accuracy in the Go/No-go and Stroop tasks. These findings suggest that time perception is functionally linked to inhibitory systems. If the feedback training protocol can modulate and maintain time perception, it may improve various cognitive/psychiatric functions in patients with PD. It may also be useful in the treatment of diseases other than PD that cause dysfunctions in temporal processing.

A case of prosopometamorphopsia caused by infarction of the splenium of the corpus callosum and major forceps.

An adult female complained of enlargement of right eyes in other people. Diffusion-weighted imaging detected an abnormal high-intensity area in the region from the splenium of the corpus callosum to the major forceps on the right side. The patient reported that right eyes appeared larger in size, which suggested prosopometamorphopsia. Adichotic listening test identified left-ear deficit. Acombination of prosopometamorphopsia and left-ear deficit was not identified in the reported patients. Prosopometamorphopsia in most of the reported patients included the eye as did that in our patient. This result suggested the importance of information on the eye in recognizing faces.

Interhemispheric cortical connections and time perception: A case study with agenesis of the corpus callosum.

In daily life, we sometimes select temporal cues of one sort while suppressing others. This study investigated the mechanism of suppression by examining a split-brain patient's perception of target intervals while ignoring distractor intervals. A patient with agenesis of corpus callosum and five age- and sex-matched control subjects participated in reproduction of target intervals while ignoring distractors displayed in the visual field either ipsilateral or contralateral to target. In the patient, the distractor interfered with reproduction performance more strongly when contralateral rather than ipsilateral. Our results suggest that the corpus callosum plays an inhibitory role in interhemispheric interference and that temporal interval information can be transferred via subcortical structures when there are no direct interhemispheric pathways.

Wandering behavior of a severely demented patient with frontotemporal dementia.

Although wandering is a common behavioral problem in patients with dementia, information about wandering in patients with severe dementia is scarce. This study analyzed data about the wandering behavior of a 77-year-old woman with severe frontotemporal dementia. Due to severe atrophy of the anterior cortex, she was totally unable to act spontaneously. Nevertheless, she walked with her husband every day. Her walking pattern varied on a daily basis but it appeared to be random. However, a mathematical analysis showed that her walking pattern was not random. Our case suggests that even patients with severe atrophy in the anterior cortex may be able to orient themselves within their neighborhood if their posterior cortex is preserved.

Autobiographical age awareness disturbance syndrome in autoimmune limbic encephalitis: two case reports.

BACKGROUND: Autobiographical memory is a form of episodic memory characterized by a sense of time and consciousness that enables an individual to subjectively re-experience his or her past. As part of this mental re-enactment, the past is recognized relative to the present. Dysfunction of this memory system may lead to confusion regarding the present perception of time. CASE PRESENTATION: Two Japanese women (42 and 55 years old) temporarily believed they were living in their past during a course of autoimmune limbic encephalitis. Their autobiographical memories and behaviour reflected their self-estimated age, and they could not recall memories experienced beyond that age. More surprisingly, their subjective age estimations and autobiographical memories were transiently corrected when they were made aware of their true age. Disorientation, anterograde amnesia, and retrograde amnesia were common additional symptoms. Neuroimaging suggested disturbances in medial temporal and orbitofrontal brain regions in both cases. CONCLUSIONS: This syndrome is characterized by three elements: 1) failure to subjectively recognize the present; 2) inability to suppress irrelevant past memories; and 3) transient restitution of awareness of the present through realization of the individual's true age. We defined this syndrome as 'autobiographical age awareness disturbance', and focused our investigation on the role of age self-awareness. If recall of relevant and suppression of irrelevant past memories are both necessary to subjectively recognize the present relative to the past, dysfunction of medial temporal and orbitofrontal brain regions is predicted to lead to abnormal subjective placement in time. However, the subjective experience of age tends to be an important informational component for retrieving remote autobiographical memories. This suggests that correct age awareness is essential for the proper recognition of the remote past in relation to the present. This is the first report to focus on the relationship between subjective temporal orientation and age self-awareness. While the role of age awareness in this process is still unclear, investigating autobiographical age awareness disturbance as a part of subjective temporal awareness dysfunction can be useful in understanding the processes underlying human time recognition.

The emergence of artistic ability following traumatic brain injury.

In this study, the case of a patient who developed artistic ability following a traumatic brain injury is reported. The subject was a 49-year-old male who suffered brain injury at the age of 44 due to an accidental fall. At age 48, he began drawing with great enthusiasm and quickly developed a personal style with his own biomorphic iconography. At first, his drawing was restricted to realistic reproductions of photographs of buildings, but his style of drawing changed and became more personal and expressionistic over the following 6 months.

Revisiting Clinical Utility of Chest Radiography and Electrocardiogram to Determine Ischemic Stroke Subtypes: Special Reference on Vascular Pedicle Width and Maximal P-Wave Duration.

BACKGROUNDS: It is often difficult to diagnose stroke subtypes at admission, particularly in sinus rhythm cases. Vascular pedicle width (VPW) on chest X-ray (CXR) and maximal P-wave duration (P-max) on electrocardiogram (ECG) are again realized as useful parameters reflecting intravascular volume and atrial conduction status, respectively. We investigated the utility of VPW and P-max as a tool for differentiating ischemic stroke subtypes. METHODS: We studied 343 acute stroke patients showing sinus rhythm on admission. Dividing the patients into cardioembolic (CE) stroke (n = 57) and non-CE (n = 286) groups, we compared clinical backgrounds including VPW on CXR, and P-max in lead II and premature atrial contraction (PAC) on 12-leads ECG. Then, we investigated the independent factors for CE. RESULTS: Independent factors associated with CE were VPW (≥59.3 mm) (p < 0.001; odds ratio (OR), 10.12; 95% confidence interval (CI), 4.13-24.8), P-max in lead II (≥120 ms) (p < 0.001; OR, 8.61; 95% CI, 3.96-18.7), PAC (p = 0.002; OR, 7.35; 95% CI, 2.14-25.3) and D-dimer level (≥1.11 µg/ml) (p = 0.016; OR, 2.57; 95% CI, 1.20-5.51). CONCLUSIONS: VPW, P-max, PAC and D-dimer are useful parameters for diagnosing CE stroke in patients with sinus rhythm at admission.

Influence of the Tohoku-Pacific Ocean earthquake and its aftershocks on the response to prophylactic therapy with lomerizine in patients with migraine in Tokyo: a retrospective study.

BACKGROUND: On March 11, 2011, the Tohoku-Pacific Ocean earthquake (magnitude 9.0) struck the eastern part of Japan. Despite being far from the epicenter of the catastrophic earthquake, the effects were strongly felt in Tokyo, and the aftershocks continued for several months. There are no reports regarding the influence of earthquakes on migraine medication. The aim of our study was to determine the impact of earthquakes on prophylactic therapy with lomerizine in patients with migraine in Tokyo. METHODS: The study included patients with migraine who were admitted to outpatient clinics in Tokyo between January 2010 and July 2010 or between January 2011 and July 2011 and who were prescribed lomerizine prophylactically for headache by specialists. We investigated clinical factors from the medical records for 26 of these patients. RESULTS: The study population included 10 patients in 2010 and 16 patients in 2011. The frequency of headaches was reduced to under 5 days/month during February in both the groups. Compared to 2010, the frequency of headaches significantly increased in 2011 in March, April and May. CONCLUSION: Patients with migraine were sensitive to exposure to the earthquake and their headaches worsened despite successful prophylactic treatment with lomerizine before the Tohoku-Pacific Ocean earthquake.

[Epilepsy and dementia].

Japan is experiencing an increase in the number of dementia patients, and the incidence of epilepsy is high among the elderly. A survey, from 2007 to 2012, of 279 epilepsy patients admitted to our hospital's neurology department showed that newly-onset epilepsy occurred more frequently in elderly patients. Of 153 elderly patients with epilepsy, aged 65 years and older, 31(20.2%) had dementia as an underlying disease. Although the prevalence of epilepsy in dementia is varied, higher brain dysfunctions are known to occur as a result of epilepsy. Considering such symptoms as epilepsy with higher brain dysfunction (E-HBD) would enable clinical investigation without overlooking higher brain dysfunctions that have effective treatments.

[A Case of Bacterial Meningitis in Which Occurrence of Ataxie Optique Led to Detection of a Small Subdural Abscess and Pialitis].

We report the case of a 69-year-old man with bacterial meningitis who presented with ataxie optique in the peripheral part of the left visual field in both hands. A detailed neurological examination with contrast-enhanced brain MRI in the early stage of the clinical course identified a small subdural abscess and pialitis in the right parietal area. A favorable outcome was obtained with antibiotic therapy alone. In a case with higher brain dysfunction of unknown cause in the clinical course of bacterial meningitis, a detailed neurological examination may be helpful to identify the causative site. (Received September 25, 2023; Accepted October 31, 2023; Published March 1, 2024).

A more realistic approach, using dynamic stimuli, to test facial emotion recognition impairment in temporal lobe epilepsy.

To explore potentially impaired social functioning in patients with mesial temporal lobe epilepsy (MTLE), we evaluated facial emotion recognition (FER) using dynamic facial stimuli. We evaluated FER in 88 patients with MTLE, including 25 posttemporal lobectomy (PTL) patients, when they watched videos of actors expressing the six basic emotions of happiness, sadness, anger, fear, surprise, and disgust. Thirty-two healthy subjects were examined as controls. The relationships between task, performance, and neurophysiological and radiological variables potentially affecting the ability to recognize moving facial emotions were examined by multivariate analysis. Both the patients with MTLE and the PTL subset demonstrated significantly impaired FER compared with healthy controls. Of the six emotions, they showed impaired recognition of sadness, fear, and disgust. Facial emotion recognition was impaired in patients with chronic MTLE, particularly those with bilateral damage. Failure to recognize emotional expressions, particularly fear, disgust, and sadness, may contribute to difficulties in social functioning and relationship building.

Self-rated anosognosia score may be a sensitive and predictive indicator for progressive brain atrophy in amyotrophic lateral sclerosis: an X-ray computed tomographic study.

We investigated whether a self-rated anosognosia score can be an indicator for progression of brain atrophy in patients with amyotrophic lateral sclerosis (ALS). Scores for 16 patients were compared with the ventricular areas of the bilateral anterior and inferior horns measured on x-ray computed tomography. Longitudinal enlargement was expressed as a monthly increase in size: (ventricular size at the initial scan - ventricular size at the follow-up scan)/scan interval (months). The anosognosia scores ranged from -4 to 3 and 3-18 in patients with and without frontotemporal lobar degeneration (FTLD), respectively (p = 0.0011). Anosognosia scores were significantly correlated with sizes of anterior (r = 0.704, p = 0.0016) and inferior (r = 0.898, p < 0.0001) horns. In non-demented patients for whom follow-up CT scans were available (n = 7), the scores were significantly correlated with the longitudinal increase in inferior horn size (r = 0.754, p = 0.0496), but not with that of anterior horn size (r = -0.166, p = 0.7111). In conclusion, anosognosia in ALS is associated with greater anterior and inferior horn sizes, reflecting frontotemporal lobar atrophy. Moreover, mild anosognosia in ALS patients without FTLD may predict impending inferior horn enlargement, reflecting medial temporal atrophy.

Pseudobulbar dysarthria in the initial stage of motor neuron disease with dementia: a clinicopathological report of two autopsied cases.

We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.

Tryptophan hydroxylase 2 gene polymorphisms in Japanese patients with medication overuse headaches.

PURPOSE: We investigated whether tryptophan hydroxylase 2 (TPH2) gene polymorphisms were involved in the aggravation of migraines due to the overuse of medication. METHODS: Forty-seven migraine patients (6 males and 41 females; 36.4 10.3 years) and 22 MOH patients (1 male and 21 females; 39.6 9.9 years) who had migraines participated in this study. The genotypes for the TPH2 gene polymorphisms (rs4565946, rs4570625, and rs4341581) were analyzed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) methods. RESULTS: The rs4565946, rs4570625, and rs4341581 genotypes were similarly distributed between migraine patients and MOH patients. CONCLUSION: The results of this study showed no association between tryptophan TPH2 gene polymorphisms and the complication of MOH in patients with migraines.

[Allesthesia].

Allesthesia is a peculiar symptom in which sensory stimulation to one side of the body is perceived on the opposite side. This was first described by Obersteiner1) in patients with spinal cord lesions in 1881. Thereafter, it has occasionally been reported for brain lesions and was classified into higher cortical dysfunction as a right parietal lobe symptom. No detailed studies on this symptom have long been reported in association with lesions of either the brain or spinal cord, partly because of difficulties in its pathological evaluation. Being scarcely mentioned in recent books on neurology, allesthesia has virtually become a forgotten neural symptom. The author identified allesthesia in some patients with hypertensive intracerebral hemorrhage and three patients with spinal cord lesions, and studied its clinical signs and mechanism of pathogenesis2). The following sections discuss allesthesia in light of its definition, cases and responsible lesions, clinical signs, and mechanism of pathogenesis.

Neuropsychological and radiological assessments of two cases with apparent idiopathic rapid eye movement sleep behaviour disorder.

We examined two male patients (64 and 70 years old) with rapid eye movement sleep behaviour disorder (RBD) using cognitive tests, myocardial (123)I-metaiodobenzylguanidine (MIBG) scintigraphy, and dopamine positron emission tomography (PET) imaging. One patient had impaired social cognition and decision-making skills and reduced MIBG reuptake, and dopamine PET imaging indicated striatal terminal loss. The other patient had normal social cognition and reduced MIBG reuptake, but no abnormalities were detected by dopamine PET imaging. Both patients reported constipation. This study supports the hypothesis by Braak et al. [Mov Disord 2006;21:2042-2051] that impairment of the amygdala and striatum occurs at the same stage as α-synucleinopathy, and the findings suggest that autonomic dysfunctions such as reduced MIBG reuptake and constipation are the earliest symptoms of RBD. Detailed neuropsychological and radiological assessments of these 2 cases revealed that idiopathic RBD could develop in various clinical situations and include cognitive and autonomic changes. Further longitudinal studies with larger sample sizes will help understand the neuropsychology and pathophysiology of RBD.

Picture agnosia as a characteristic of posterior cortical atrophy.

BACKGROUND: Posterior cortical atrophy (PCA) is a degenerative disease characterized by progressive visual agnosia with posterior cerebral atrophy. We examine the role of the picture naming test and make a number of suggestions with regard to diagnosing PCA as atypical dementia. METHODS: We investigated 3 cases of early-stage PCA with 7 control cases of Alzheimer disease (AD). The patients and controls underwent a naming test with real objects and colored photographs of familiar objects. We then compared rates of correct answers. RESULTS: Patients with early-stage PCA showed significant inability to recognize photographs compared to real objects (F = 196.284, p = 0.0000) as measured by analysis of variants. This difficulty was also significant to AD controls (F = 58.717, p = 0.0000). CONCLUSION: Picture agnosia is a characteristic symptom of early-stage PCA, and the picture naming test is useful for the diagnosis of PCA as atypical dementia at an early stage.

The brainstem is at high risk for recurrent noncardioembolic cerebral infarction in association with diabetes mellitus: a hospital-based study.

The goals of the study were to investigate the importance of brainstem infarction (BSI) in recurrent noncardioembolic ischemic stroke and to examine the relevant clinical background. Data were retrospectively reviewed for 655 consecutive patients with acute noncardioembolic infarction who were admitted to our hospital from January 2004 to August 2010. The patients were divided into first-stroke (n = 592) and recurrent-stroke (n = 63) groups. Acute infarcted lesions were explored on MRI, and clinical background factors including age, sex, smoking, atrial fibrillation, coronary heart disease, hypertension, hyperlipidemia and diabetes mellitus (DM) were assessed. The frequency of BSI in the recurrent-stroke group was significantly higher than that in first-stroke patients (30.2 vs. 14.9%, p = 0.0033). No other clinical background factors differed between the two groups. Only the frequency of DM differed significantly among four subgroups formed based on stroke recurrence and BSI (p < 0.0001): DM was present in 63.2% of recurrent-stroke patients with BSI, 54.5% of first-stroke patients with BSI, 27.4% of first-stroke patients without BSI, and 20.5% of recurrent-stroke patients without BSI. We conclude that the brainstem is at high risk for recurrent cerebral infarction in patients with DM.

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

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