Impaired laryngeal voice production in a patient with foreign accent syndrome.

We report a Japanese-speaking monolingual woman who developed foreign accent syndrome (FAS) following an infarction in the precentral and premotor cortices (Brodmann Area 6) at and around the inferior frontal sulcus. Her speech sounded Chinese or Korean to our bilingual coauthor who speaks Chinese and Japanese. Quantitative acoustic analyses of words and sentences showed that pitch (fundamental frequency variation) and intensity variances appeared lowered and fully voiced glottal pulses were reduced. These findings suggest laryngeal dysfunction that contributes to the unusual speech production in a case of FAS. This may be caused by damage to a restricted area of the motor and premotor cortices that controls laryngeal function.

Anomia with Amnesia Caused by Hemorrhage in the Left Anterior and Medial Thalamus: Thalamic Anomia Particularly for Artificial Objects.

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.

Pure alexia for kana. Characterization of alexia with lesions of the inferior occipital cortex.

OBJECTIVE: To characterize reading impairments caused by lesions in the posterior occipital cortices. METHODS: We gave six patients with these lesions reading and writing tests and located a critical site for alexia using MRI and SPECT. RESULTS: The patients read three-character kana (Japanese syllabograms) nonwords, and five-character kana nonwords significantly or at a near significant level more poorly and slowly than normal subjects, whereas they read kanji (Japanese morphograms) almost correctly but more slowly. Letter-by-letter reading with a single-kana character identification impairment (in five patients), a word-length effect, kinesthetic facilitation, a lexicality effect, and minor to mild agraphia for kanji (in three patients) were observed. These deficits were characteristic of pure alexia. Alexia disappeared within a few months except in one patient who had extensive hypoperfusion in the left occipital lobe. A shared lesion was located in the left posterior fusiform/inferior occipital gyri (Area 18/19) on MRI, and there was blood flow reduction around this area on SPECT. This area coincided with the activation site for kana word covert reading in our previous study. CONCLUSIONS: These results suggest that pure alexia particularly for kana, or more generally pure alexia for letters, is caused by a lesion in the posterior inferior occipital cortex, characterized primarily by impaired kana character or letter identification, with relatively preserved kanji or word recognition.

Agraphia for kanji resulting from a left posterior middle temporal gyrus lesion.

OBJECTIVE: To clarify whether agraphia or alexia occurs in lesions of the left posterior middle temporal gyrus. METHODS: We assessed the reading and writing abilities of two patients with this lesion using kanji (Japanese morphograms) and kana (Japanese syllabograms). RESULTS: Patient 1 first presented with pure alexia more impaired for kana after an infarction in the left middle and inferior occipital gyri and right basal occipital cortex, and after a second infarction in the left posterior middle temporal gyrus adjoining the first lesion he showed alexia with agraphia for kanji and worsened alexia for kana; kanji alexia recovered over the following six to 10 months. Patient 2 presented with alexia with agraphia for kanji following a hemorrhage in the left posterior middle and inferior temporal gyri, which resolved to agraphia for kanji at two months after onset. Kana nonword reading was also slightly impaired, but became normal by six months post-onset. In both patients, kanji agraphia was mostly due to impaired character recall. CONCLUSION: The present patients demonstrate that damage to the left posterior middle temporal gyrus alone can cause agraphia for kanji. If the adjacent mid fusiform/inferior temporal gyri (Area 37) are spared, the kanji alexia is transient.

Parietal dysgraphia: characterization of abnormal writing stroke sequences, character formation and character recall.

OBJECTIVE: To characterize various dysgraphic symptoms in parietal agraphia. METHOD: We examined the writing impairments of four dysgraphia patients from parietal lobe lesions using a special writing test with 100 character kanji (Japanese morphograms) and their kana (Japanese phonetic writing) transcriptions, and related the test performance to a lesion site. RESULTS: Patients 1 and 2 had postcentral gyrus lesions and showed character distortion and tactile agnosia, with patient 1 also having limb apraxia. Patients 3 and 4 had superior parietal lobule lesions and features characteristic of apraxic agraphia (grapheme deformity and a writing stroke sequence disorder) and character imagery deficits (impaired character recall). Agraphia with impaired character recall and abnormal grapheme formation were more pronounced in patient 4, in whom the lesion extended to the inferior parietal, superior occipital and precuneus gyri. CONCLUSION: The present findings and a review of the literature suggest that: (i) a postcentral gyrus lesion can yield graphemic distortion (somesthetic dysgraphia), (ii) abnormal grapheme formation and impaired character recall are associated with lesions surrounding the intraparietal sulcus, the symptom being more severe with the involvement of the inferior parietal, superior occipital and precuneus gyri, (iii) disordered writing stroke sequences are caused by a damaged anterior intraparietal area.

Fusiform type alexia: pure alexia for words in contrast to posterior occipital type pure alexia for letters.

OBJECTIVE: To clarify the behavioral differences between patients with pure alexia from different lesions. METHODS: Two patients with pure alexia caused by damage to the fusiform or posterior occipital gyri were given reading and writing tests including kanji (Japanese morphograms) and kana (Japanese phonetic writing). RESULTS: Patient 1 (pure alexia from a fusiform gyrus lesion) had difficulty reading both kanji and kana, with kanji reading more impaired, and imageability and visual complexity effects (imageable or less complex words/characters were read better than nonimageable or more complex words/characters), whereas patient 2 (pure alexia from a posterior occipital gyri lesion) showed selective impairment of kana reading. CONCLUSION: Pure alexia for kanji (and kana; fusiform type) is characterized by impairments of both whole-word reading, as represented in kanji reading, and letter identification, and is different from pure alexia for kana (posterior occipital type) in which letter identification is primarily impaired. Thus, fusiform type pure alexia should be designated pure alexia for words, whereas posterior occipital type pure alexia should be designated pure alexia for letters.

Ubiquitin-positive frontotemporal lobar degeneration presenting with progressive Gogi (word-meaning) aphasia. A neuropsychological, radiological and pathological evaluation of a Japanese semantic dementia patient.

A patient with progressive anomia and alexia with agraphia for kanji (Japanese morphograms) is described. The patient showed a deficit in single-word comprehension and on-reading (a type of reading that conveys phonetic value) dominance in kanji reading, i.e. on-preceding (pronouncing first with on-reading, irrespective of its preferred reading) and kun-deletion (inability to recall and recognize kun-reading [another type of reading that conveys meaning]) when reading a single-character kanji. These features were due to loss of lexico-semantic information and thus the patient was regarded as having progressive Gogi (word-meaning) aphasia by Imura, a Japanese manifestation of semantic dementia. Macroscopically, neuropathological examination disclosed atrophy of the left frontotemporal lobe with accentuation in the anterior portion of the temporal lobe. Histologically, there was neuronal loss in the cerebral cortex, hippocampus, parahippocampal gyrus, amygdala, caudate nucleus, and putamen. Ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells. This case demonstrates that progressive Gogi aphasia is semiologically identical to semantic dementia, and our patient clinicopathologically resembled those of Rossor et al. [Rossor, M.N., Revesz, T., Lantos, P.L., Warrington, E.K. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. Brain 2000; 123: 267-76.] and Hodges et al. [Hodges, J.R., Davies, R.R., Xuereb, J.H., Casey, B., Broe, M., Bak, T.H., et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004; 56: 399-406.].

Anti-glutamate ∊2 receptor antibody-positive and anti-N-methyl-d-aspartate receptor antibody-negative lobar encephalitis presenting as global aphasia and swallowing apraxia.

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 ∊2, 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 (99m)Tc-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.

Delayed leukoencephalopathy after carbon monoxide poisoning presenting as subacute dementia.

We herein report the case of a 65-year-old woman who presented with the subacute onset of dementia and subsequently developed abnormal behavior and a gait disturbance. Her condition transiently improved; however, within one month, she became drowsy and poorly responsive, with limb chorea and urinary incontinence. Her history of frequently using charcoal led us to diagnose her with carbon monoxide (CO) poisoning. The findings of this case and a literature review suggest that subacute dementia due to CO poisoning recovers late, after a year or more, in patients above sixty years of age, and both hyperbaric oxygen and corticosteroid pulse therapy should be considered in such cases, even after one month.

Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake.

A 65-year-old woman developed progressive apraxic agraphia, characterized by poorly formed graphemes, a kanji (Japanese morphograms) recall impairment, relatively preserved oral spelling of kanji characters, and incorrect stroke sequences on writing accompanied by micrographia over a 3-year period. She also showed minor degrees of rigidity, limb-kinetic apraxia, and ideomotor apraxia of the left hand. Although asymmetric rigidity and limb-kinetic apraxia strongly suggested corticobasal degeneration, (11)C-Pittsburgh compound B positron emission tomography (PiB-PET) showed the predominantly right-sided accumulation of amyloid ß in the cortices and striatum. (18)F-fluoro-deoxy-glucose PET and single photon emission computed tomography with a (99m)Tc-ethylcysteinate dimer (ECD-SPECT) also revealed predominantly right-sided hypometabolism and hypoperfusion in the primary sensorimotor cortex, posterior cingulate gyrus, temporoparietal cortices, frontal cortices, thalamus, and basal ganglia, a pattern characteristic of both corticobasal degeneration and Alzheimer's disease. The findings suggest that progressive apraxic agraphia with micrographia presenting as corticobasal syndrome can show an Alzheimer's disease pathology. It is also suggested that ideomotor apraxia of the left hand can occur without a callosal lesion, and is caused by hypometabolism or hypoperfusion in the right frontal and parietal cortices, as revealed by PET and SPECT.

Isolated thalamic agraphia with impaired grapheme formation and micrographia.

Two patients with isolated thalamic agraphia are described. Both showed kanji (Japanese morphograms) agraphia due to impaired character recall, grapheme deformity and micrographia (progressive reduction in character size during writing) after a lesion that involved the ventral lateral and ventroposterolateral nuclei. Single photon emission computed tomography with a (99m)Tc-ethylcysteinate dimer revealed hypoperfusion in the left precentral gyrus (Brodmann Area 6) and anterior supramarginal gyrus in both. Six months later, the extent of blood flow reduction decreased in the supramarginal gyrus in both patients and the precentral gyrus in patient 1. By this time, the writing impairment improved to nearly the normal range. Our study suggests that kanji agraphia (corresponding to lexical agraphia in Western countries) with poor grapheme formation and micrographia arises from a lesion in the ventral lateral and ventroposterolateral nuclei in the left thalamus. The accompaniment of poor grapheme formation and micrographia may reflect disruption of the cortico-subcortical motor circuit involving the putamen, thalamus, premotor cortex and sensorimotor cortex. It is also suggested that multiple cortical sites can be a target for secondary dysfunction that yields agraphia in a thalamic lesion, and that the recovery of reduced cortical blood flow does not always proceed in parallel with that of agraphia.

Alexia and agraphia with lesions of the angular and supramarginal gyri: evidence for the disruption of sequential processing.

OBJECTIVE: To determine the features of alexia or agraphia with a left angular or supramarginal gyrus lesion. METHODS: We assessed the reading and writing abilities of three patients using kanji (Japanese morphograms) and kana (Japanese syllabograms). RESULTS: Patient 1 showed kana alexia and kanji agraphia following a hemorrhage in the left angular gyrus and the adjacent lateral occipital gyri. Patient 2 presented with minimal pure agraphia for both kanji and kana after an infarction in the left angular gyrus involving part of the supramarginal gyrus. Patient 3 also showed moderate pure agraphia for both kanji and kana after an infarction in the left supramarginal and postcentral gyri. All three patients made transposition errors (changing of sequential order of kana characters) in reading. Patient 1 showed letter-by-letter reading and a word-length effect and made substitution errors (changing hiragana [one form of kana] characters in a word to katakana [another form of kana] characters and vice versa) in writing. CONCLUSION: Alexia occurs as "angular" alexia only when the lesion involves the adjacent lateral occipital gyri. Transposition errors suggest disrupted sequential phonological processing from the angular and lateral occipital gyri to the supramarginal gyrus. Substitution errors suggest impaired allographic conversion between hiragana and katakana attributable to a dysfunction in the angular/lateral occipital gyri.

Intramedullary spinal cord abscess treated with antibiotic therapy--case report and review.

A 58-year-old man presented with an intramedullary spinal cord abscess (ISCA) manifesting as posterior neck pain, gait disturbance, and urinary retention, and transverse myelopathy 1 week later. Magnetic resonance imaging showed the ISCA at the C7 to T1 levels. He was treated under a diagnosis of cryptogenic ISCA with high-dose ampicillin and third- or fourth-generation cephalosporins, which resulted in complete recovery after 2 months. Review of the literature between January 1998 and August 2007 identified 26 cases of ISCA, including our patient. We also identified two additional nonsurgically treated ISCA patients reported between 1977 and 2007. The most common presentation was motor deficits in all patients, followed by fever, pain, and bladder dysfunction. The mortality rate was 1 of 26 patients, and neurological sequelae were observed in 15 of the 25 surviving patients. There was no significant difference in the frequency of neurological sequelae between surgically and nonsurgically treated patients. Mean length of the abscess in the surgically treated group was significantly larger than that in the medically treated group (5.8 vs. 2.2 vertebral bodies). All three nonsurgically treated patients with neurological sequelae had anaerobic infections and received antibiotic therapy later and for shorter periods than those with complete neurological recovery. Antibiotic treatment is comparable to surgery plus antibiotic treatment. Early broad-spectrum high-dose ampicillin and third-generation cephalosporin, covering Gram-positive, Gram-negative, and anaerobic organisms, should be the first choice of management for patients with ISCA.