Reversible hippocampal lesions detected on magnetic resonance imaging in two cases of transient selective amnesia for simple machine operation.

We report two extremely rare cases involving the development of transient selective retrograde amnesia for simple machine operation lasting for several hours. A 61-year-old male taxi driver suddenly became unable to operate a taximeter, and a 66-year-old female janitor suddenly became unable to use a fax machine. They could precisely recount their episodes to others both during and after the attacks, and their memories during their attacks corresponded to the memory of the witness and the medical records of the doctor, respectively. Therefore, it appears that these individuals remained alert and did not develop anterograde amnesia during their attacks. On day 4, they underwent high-resolution magnetic resonance imaging (MRI), and diffusion-weighted MRI with 2-mm section thickness revealed small high-intensity signal lesions in the left hippocampal cornu ammonis area 1 (CA1) region. However, these lesions disappeared during the chronic phase. This is the first report describing lesions detected by MRI in patients with transient selective amnesia without anterograde amnesia. Reversible damage to the hippocampal CA1 region may cause transient selective amnesia by impairing the retrieval of relevant memories.

Frontotemporal lobar degeneration with writing disturbance mainly consisting of omission of kana letters.

A right-handed woman developed pseudobulbar palsy and a particular writing disturbance mainly composed of omission of kana letters (OKL) at the age of 79, followed by gradual progression of generalized motor disturbance and mutism. She died at the age of 88. Postmortem examination revealed frontotemporal lobar degeneration. The precentral cortex and premotor area were the most severely degenerated among the affected frontal, parietal, and temporal lobes. The omission of kana letters has been recently reported as a characteristic feature of writing disturbance in Japanese amyotrophic lateral sclerosis (ALS). Our case indicates that OKL is not specific to ALS, and that the prefrontal and precentral cortices, common lesions between our case and ALS, are responsible for OKL. This case also shows that OKL can be caused by a pathomechanism independent from other types of writing error. The neurolinguistic analysis of our case suggests the disturbance of the moraic frame of words in the transcription process of morae into kana letters or kana-letter cards.

Primary progressive apraxia of speech (AOS) in a patient with Pick's disease with Pick bodies: a neuropsychological and anatomical study and review of literatures.

A 56-year-old right-handed man suffered from progressive apraxia of speech (AOS), characterized by agrammatism and buccofacial apraxia. He also became mute at the later stages of the disease progression. At autopsy, the left precentral gyrus, pars opercularis, and hippocampus showed severe atrophy. Pick bodies and Pick cells were observed. In this report, we also review previous case reports of AOS. Pick's disease is among the most commonly associated of the major diseases. Brain lesions associated with AOS may be found in regions such as the precentral gyrus and the pars opercularis in the left hemisphere.

[Case Report: Broca's Aphasia in a Left-Handed Patient with a Right Brain Infarction and Early Stage Aphonia].

We report a case of Broca's aphasia in a left-handed patient with a right brain infarction. The patient's speech is consistent with a particular type of aphemia, that is, without vocalization except for a few phonemes or words. The patient presented with aphonia in an early stage. The lack of speech could be due to the impairment of the phonological-speech process or speech initialization. This type of aphemia has been reported to involve the right inferior precentral gyrus or right middle and inferior frontal gyri. Our patient had both lesions. The symptom and the lesion of this type of aphemia could differ from those of another type of aphemia corresponding to apraxia of speech, and the speech of Broca's aphasia could have multiple mechanisms. Our case shows Alexander's anomalous type with atypical lateralization and distribution of the lesion. Verbal intrahemispheric dissociation apraxia was suspected in our patient. The coexistence of aphasia, anosodiaphoria of hemiplegia is a dual symptom in which bilateral hemispheric functions exist in a unilateral hemisphere. (Received 1 December, 2021; Accepted 1 February, 2022; Published 1 April, 2022).

[Progressive supranuclear palsy-Richardson syndrome with visual attention disturbance (Holmes and Horrax) and ataxie optique (Garcin): a case report].

[Background] Supranuclear extraorbital muscle palsy is the core feature of progressive supranuclear palsy (PSP), and ordinarily presents as spontaneous vertical gaze constriction. However, higher visual function associated with visuospatial cognition in PSP patients was not previously considered. [Case presentation] We present a 72-year old right-handed man with PSP- Richardson syndrome (PSP-RS) and abnormal higher visual function. His symptoms began 2 years previously and included the use of small steps while walking, forgetfulness, and postural instability. Neurological examination revealed supranuclear vertical gaze limitation, akinesia, and lead-pipe rigidity without laterality. Neuro-ophthalmological examination showed abnormal ocular movement consistent with PSP, and no visual abnormality was observed. General cognitive functions, including attention and prominent visuospatial orientation and visual attention disturbances, were assessed using neuropsychological tests and concomitant spatial agraphia and impaired configuration using figure copying. Although he presented with mildly decreased and monotonous speech with palilalia, he showed no apparent aphasia, apraxia, visual object agnosia, or Bálint's 'optische Ataxie' i.e. visual ataxia under fixation. Brain MRI revealed atrophy of the mesencephalic tegmentum, bilateral frontal lobe, and bilateral hippocampus. N-isopropyl-p-(iodine-123)-iodoamphetamine single photon emission computed tomography revealed decreased cerebral blood flow in the bilateral frontal lobe, lateral temporal lobe, and basal ganglia. Dopamine transporter single photon emission CT revealed uptake attenuation in the bilateral striatum. 123I-metaiodobenzyl-guanidine myocardium scintigraphy results were normal. [Discussion] The patient's symptoms indicated classical PSP-RS accompanied with a combination of disturbances in spatial orientation and visual attention as noted by Holmes and Horrax and 'ataxie optique' by Garcin. Thus, as observed in this patient, many clinically diagnosed PSP patients with undiagnosed higher visual dysfunction, masked by limited eye movement may exist. These symptoms may further our understanding about posterior cortical atrophy and tauopathy including not only PSP but also corticobasal syndrome and Alzheimer disease.

[Transcortical sensory aphasia due to extensive infarction of left cerebral hemisphere].

We report a case of transcortical sensory aphasia occurred after extensive infarction of left cerebral hemisphere. A 68-year-old, right-handed man with atrial fibrillation suddenly developed cerebral embolism of left middle cerebral artery. He was treated conservatively, and the right hemiplegia, aphasia, apraxia in a slight degree and right hemispatial neglect in a slight degree consequently existed. MRI showed a large cortical and subcortical infarct lesion including the left Broca's area, central region, perisylvian area with Wernicke's area and temporal lobe. In contrast, neuropsychological evaluation using the Western Aphasia Battery (WAB) demonstrated transcortical sensory aphasia, e.g., fluency 8, auditory comprehension 1. repetition 10 and object naming 2.4. In addition to preserved repetition, both linguistic prosody and affective prosody were well preserved. Most cases with transcortical sensory aphasia are known to occur with the lesion including temporo-parieto-occipital junction of dominant hemisphere. Our patient and a few other reported cases of transcortical sensory aphasia had a lesion in perisylvian area including Wernicke's area. Therefore, it is possible that their minor hemisphere worked selectively for repetition. Furthermore, we suggest that this patient presented dissociative aphasia that all the process of repetition and the function of linguistic and emotional prosody were represented in the right hemisphere and the other functions including comprehension of word meanings were existed in the left hemisphere. We believe that our case of transcortical sensory aphasia with dissociative aphasia gives a suggestion about the mechanism and localization of repetition and prosody in the whole system of language.

[An autopsy case of corticobasal degeneration with notable early onset apraxia: a case report and literature review focused on apraxia].

We report the autopsy case of a 74-year-old woman. Onset of gait disturbance and left-side dominant bilateral motor disturbance in the patient led to bilateral progressive apraxia. This was associated with a decline in motor imagery, right-side dominant atrophy of the central sulcus region, and a decrease in cerebral blood flow during illness. She died of respiratory failure that had progressively worsened over a 9-year period. Pathologically, she exhibited right-side dominant cerebral atrophy; neuronal loss, gliosis, and astrocytic plaques were mainly present in the frontal lobe. She was subsequently diagnosed with corticobasal degeneration (CBD). The premotor and primary motor areas revealed marked degeneration; in addition, severe myelin pallor was observed in these regions, and it was suggested that such pathological features were responsible for the apraxia. We believe the present case is valuable since very few reports have provided a detailed description of clinicopathological apraxia in association with CBD.

Elderly-onset neuromyelitis optica which developed after the diagnosis of prostate adenocarcinoma and relapsed after a 23-valent pneumococcal polysaccharide vaccination.

We report a case of elderly-onset neuromyelitis optica (NMO) positive for the anti-aquaporin-4 (AQP-4) antibody; symptoms developed after the diagnosis of prostate adenocarcinoma and relapsed after a 23-valent pneumococcal polysaccharide vaccination. We suggest that activation of CD4-positive T cells and secretion of interferon-gamma induced by adenocarcinoma and complement activation induced by vaccination are responsible for the onset and relapse of NMO, even if a patient is positive for the anti-AQP-4 antibody. This case supports the previous experimental finding that the anti-AQP-4 antibody does not cause NMO-like lesions when injected alone, but does so after the induction of T cell-mediated experimental autoimmune encephalomyelitis or when co-injected with human complement.

[Development of pure alexia due to a lesion in the left fusiform gyrus in a patient with hypertrophic pachymeningitis].

We present the case of a patient with pure alexia due to a restricted lesion in the left fusiform gyrus. A 59-year-old right-handed female, with a 7-year history of rheumatoid hypertrophic pachymeningitis suddenly developed reading and writing difficulties. Neuropsychological examinations revealed the presence of alexia for both Japanese kanji (morphograms) and kana (phonograms); mild agraphia predominantly for kanji; and word-finding difficulty. Brain magnetic resonance imaging (MRI) revealed a high signal intensity lesion in the left fusiform gyrus on fluid attenuated inversion recovery (FLAIR) images in addition to marked thickness of the left cerebellar tentorium on contrast-enhanced T1-weighted images. The abnormal intensity lesion in the left fusiform gyrus was recognized as representing a cerebral edematous change due to venous insufficiency associated with dural thickness. After high-dose methyl-prednisolone therapy, there was a rapid improvement in the reading and writing abilities of the patient, and 5 days later all the symptoms had disappeared. Three months later, a repeat MRI showed that the abnormal intensity lesion in the left fusiform gyrus had disappeared completely. The present case suggests that damage to the left fusiform gyrus alone can cause pure alexia and mild agraphia. Furthermore, this case raises the possibility that the fusiform gyrus is a part of the writing center.

Rheumatoid leptomeningitis: rare complication of rheumatoid arthritis.

Rheumatoid leptomeningitis is a rare complication of rheumatoid arthritis (RA). We describe a woman with rheumatoid leptomeningitis presenting with acute-onset behavioral changes and consciousness disturbance in the early stage of RA. On fluid-attenuated inversion recovery images or diffusion-weighted images, high-signal-intensity lesions in the subarachnoid spaces of the right frontal lobe were observed. Biopsies of brain tissues and the dura mater located in the right frontal lobe were obtained. On the basis of the findings of histopathological analysis, a diagnosis of necrotizing granulomas involving the leptomeninges consistent with rheumatoid leptomeningitis was made. An early diagnosis of rheumatoid leptomeningitis and immediate initiation of treatment may prevent neurological sequelae.

Imaging of corticobasal degeneration syndrome.

INTRODUCTION: Diagnosing corticobasal degeneration is often difficult on the basis of clinical symptoms and radiological images. We aimed to clarify the imaging findings of corticobasal degeneration syndrome (CBDS). METHODS: Included in the study were 16 patients (8 men, 8 women, 46-75 years old) with clinically diagnosed CBDS. We evaluated the patients' symptoms and signs, and MR and single-photon emission CT (SPECT) imaging findings. RESULTS: All the patients had cerebral atrophy. Asymmetric cerebral atrophy was observed in 13 patients (81%) predominantly contralateral to the side clinically more affected. Atrophy in the cerebral peduncle was observed in seven patients. FLAIR images showed hyperintensity in the subcortical white matter in the frontoparietal lobes in the clinically more affected side in 14 patients, and in the rolandic region in 13 patients. Asymmetric hypoperfusion in the frontoparietal lobes on SPECT images was observed in all of the patients, and in the basal ganglia in 11 patients. CONCLUSION: CBDS might be unique in showing hyperintensity in the subcortical white matter in the rolandic region on FLAIR images with asymmetric atrophy predominantly contralateral to the side clinically more severely affected. Asymmetric atrophy in the cerebral peduncle without signal abnormalities was also characteristic of CBDS. Atrophy in the midbrain tegmentum was also seen in patients with CBDS.