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Key Clinical Message Apraxia of speech (AOS) due to a postcentral infarction differs from conventional precentral AOS with respect to phonemic errors (phoneme substitution) which are more common than phonetic errors (phoneme distortion) and preserved accent and intonation. Abstract Clinical features of apraxia of speech caused by lesions in the postcentral gyrus have not yet been elucidated. Here, we report a patient with this lesion and show how postcentral apraxia of speech differs from the hitherto known precentral apraxia of speech. A 54‐year‐old man developed Broca's aphasia with apraxia of speech that resolved into pure apraxia of speech within 3 weeks following infarction of the postcentral gyrus. The diagnosis of apraxia of speech was based on the patient's effortful, slow speech and inconsistent phonetic distortions with phonemic paraphasia. The Western Aphasia Battery was used to examine the patient's speech samples. Speech was recorded using a digital voice recorder and transcribed into a narrow transcription of the International Phonetic Alphabet. The error types were categorized phonologically. The results revealed that (a) phonemic errors (vowel and consonant substitutions, also known as phonemic paraphasia) were more common than phonetic errors (vowel and consonant distortions). Similar to conduction aphasia, phonemic errors were more pronounced in confrontation naming than in repetition, accompanied by self‐correction, and (b) word accent and sentence intonation were preserved, although the speech was slow. These two features are characteristic of postcentral apraxia of speech, which can be differentiated from conventional precentral apraxia of speech.

We herein report the case of a patient who showed pure anomia and amnesia caused by hemorrhage in the left thalamus, involving the anterior, ventral anterior, and mediodorsal nuclei. It was revealed that the anomia was characterized by impaired retrieval of object names, which was more pronounced in artificial objects, and abundant perseveration, whereas the amnesia was mild and limited to daily routine events, which was made clear from the results of an episodic memory scale. Detailed lesion localization and literature review revealed that a combination of pure anomia and amnesia can occur in a lesion involving the anterior, ventral anterior, or mediodorsal nucleus of the thalamus. The relative specificity to artificial objects can be explained by the locally damaged fiber connection to the putative category-specific lexical area in the temporal lobe.

Objective: The aim of this study was to investigate the impact of the coronavirus disease 2019 (COVID-19) pandemic on neurology in Japan, by analyzing data on the number of neurological patients at our hospital in Tokyo. Material and Methods: We counted the number of inpatients and outpatients per month; from January 2018 to September 2020. We defined the data from April 2020 to May 2020, as the first wave of the COVID-19 pandemic, and that from July 2020 to September 2020, as the second wave. The data from each wave were compared to those in the same period within the previous 2 years. We also analyzed other data; including, inpatients with stroke, outpatients with Parkinson’s disease, and outpatients with epilepsy. Results: In the first wave, the overall number of inpatients and outpatients greatly decreased; however, the number of inpatients with stroke increased. The ratio of outpatients with Parkinson’s disease, or outpatients with epilepsy to total outpatients also increased. In the second wave, the overall number of inpatients markedly increased, while that of outpatients slightly decreased. Conclusion: All Japanese general hospitals were greatly affected by the COVID-19 pandemic; especially in the first wave, even if the hospitals did not have in-hospital COVID-19 infection, or were not designated for COVID-19. Three factors; i.e. governmental, hospital, and patient factors, could affect the number of neurological patients during the COVID-19 pandemic. The numerical data reflecting the patients’ behavior might provide suggestions for addressing issues during other pandemics in the future.

Japanese kanji (morphograms) have two ways of reading: on-reading (Chinese-style pronunciation) and kun-reading (native Japanese pronunciation). It is known that some Japanese patients with semantic dementia read kanji with on-reading but not with kun-reading. To characterize further reading impairments of patients with semantic dementia, we analyzed data from a total of 9 patients who underwent reading and writing tests of kanji and kana (Japanese phonetic writing) and on-kun reading tests containing two-character kanji words with on-on reading, kun-kun reading, and specific (so-called Jukujikun or irregular kun) reading. The results showed that on-reading preceding (pronouncing first with on-reading) and kun-reading deletion (inability to recall kun-reading) were observed in nearly all patients. In the on-kun reading test, on-reading (57.6% correct), kun-reading (46.6% correct), and specific-reading (30.0% correct) were more preserved in this decreasing order (phonology-to-semantics gradient), although on-reading and kun-reading did not significantly differ in performance, according to a more rigorous analysis after adjusting for word frequency (and familiarity). Furthermore, on-substitution (changing to on-reading) errors in kun-reading words (27.0%) were more frequent than kun-substitution (changing to kun-reading) errors in on-reading words (4.0%). These results suggest that kun-reading is more predominantly disturbed than on-reading, probably because kun-reading and specific-reading are closely associated with the meaning of words.

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome has previously been reported in only 2 patients with progressive supranuclear palsy (PSP). Herein, we report a third case of WEBINO syndrome with PSP. The patient was an 81-year-old man who had experienced gradually increasing gait disturbance and occasional falls since the age of 78 years. At 80 years of age, he presented with cognitive impairment, parkinsonism, and oculomotor abnormalities. The oculomotor abnormalities consisted of vertical gaze palsy and loss of eye convergence. Brain magnetic resonance imaging demonstrated marked atrophy of the midbrain. He was diagnosed with PSP. At the age of 81 years, he presented with alternating extropia in his forward gaze and adduction paresis and outward nystagmus of the abducted eye in his horizontal gaze, both of which were compatible with WEBINO syndrome. Previously, we reported the first case of PSP with WEBINO syndrome, and another group recently reported a second case. In light of the previous cases and the present case, WEBINO syndrome in PSP should not be considered extremely rare. Furthermore, WEBINO syndrome has not been reported in other neurodegenerative disorders, which suggests that it might be a useful and specific diagnostic finding in PSP.

We report a patient with phonological agraphia (selective impairment of kana [Japanese phonetic writing] nonwords) and acalculia (mental arithmetic difficulties) with impaired verbal short-term memory after a cerebral hemorrhage in the opercular part of the left precentral gyrus (Brodmann area 6) and the adjacent postcentral gyrus. The patient showed phonemic paragraphia in five-character kana nonword writing, minimal acalculia, and reduced digit and letter span. Mental arithmetic normalized after 8 months and agraphia recovered to the normal range at 1 year after onset, in parallel with an improvement of the auditory letter span score from 4 to 6 over a period of 14 months and in the digit span score from 6 to 7 over 24 months. These results suggest a close relationship between the recovery of agraphia and acalculia and the improvement of verbal short-term memory. The present case also suggests that the opercular part of the precentral gyrus constitutes the phonological route in writing that conveys phonological information of syllable sequences, and its damage causes phonological agraphia and acalculia with reduced verbal short-term memory.

Background: Little is known about the difference between anti-N-methyl-D-aspartate receptor (NMDAR) antibody-positive encephalitis and anti-glutamate receptor (GluR) antibody-positive encephalitis. Objectives: To characterize anti-GluR antibody-positive encephalitis. Methods: We report a 33-year-old man with nonparaneoplastic anti-GluR ε2, ζ1 and δ2 antibody-positive and anti-NMDAR antibody-negative encephalitis, using neuropsychological tests and imaging studies including magnetic resonance imaging and single photon emission computed tomography (SPECT) with a 99mTc-ethylcysteinate dimer. Results: The patient exhibited global aphasia and swallowing apraxia (inability to transfer food to the pharyngeal cavity without sialorrhea). He was treated with 3 courses of corticosteroid pulse therapy and had recovered markedly 3 weeks after onset. Magnetic resonance diffusion-weighted images revealed hyperintensity in the bilateral frontal and left parietal cortices. Seven months later, a small area of hyperintensity in the left supramarginal gyrus remained. SPECT revealed hypoperfusion in extensive regions of the bilateral frontal lobes and left supramarginal gyrus. Thirteen months later, blood flow reduction was restricted to diffuse areas in the frontal lobes. Conclusions: Frontal lobar encephalitis without medial temporal involvement, marked cognitive impairment with a relatively preserved level of consciousness, and a favorable response to corticosteroid therapy, with nearly reversible cortical damage, may characterize anti-GluR antibody-positive encephalitis.

Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis.

Objective: To clarify whether agraphia or alexia occurs in lesions of the left posterior middle temporal gyrus.

Objective: To characterize various dysgraphic symptoms in parietal agraphia.

Reading impairments of three alexia patients, two pure alexia and one alexia with agraphia, due to different lesions were examined quantitatively, using Kanji (Japanese morphogram) words, Kana (Japanese phonetic writing) words and Kana nonwords. Kana nonword reading was impaired in all three patients, suggesting that widespread areas in the affected occipital and occipitotemporal cortices were recruited in reading Kana characters (corresponding to European syllables). In addition, the findings in patient 1 (pure alexia for Kanji and Kana from a fusiform and lateral occipital gyri lesion) and patient 2 (pure alexia for Kana from a posterior occipital gyri lesion) suggested that pure alexia could be divided into two types, i.e. ventromedial type in which whole-word reading, together with letter identification, is primarily impaired because of a disconnection of word-form images from early visual analysis, and posterior type in which letter identification is cardinally impaired. Another type of alexia, alexia with agraphia for Kanji from a posterior inferior temporal cortex lesion (patient 3), results from deficient whole-word images of words per se, and thus should be designated “orthographic alexia with agraphia”. To account for these impairments, a weighted dual-route hypothesis for reading is suggested.