Transient aphasia and persistent amnesia after surgery for internal carotid artery – posterior communicating artery aneurysm

We report a case of transient aphasia and persistent amnesia after clipping of a ruptured cerebral aneurysm to treat a subarachnoid hemorrhage. Postoperatively, aphasia was identified and magnetic resonance imaging (MRI) showed an abnormal intensity area in the left anterior thalamus. Single photon emission computed tomography (SPECT) revealed a wider area of low perfusion surrounding the left thalamus and left frontotemporal lobe than that shown by the MRI. His aphasia resolved over the subsequent 12-week period. He was left with an isolated disturbance of memory; in the absence of any dementia, aphasia or disturbance of consciousness, his condition was classified as one of amnesia. SPECT 14 weeks after admission revealed an area of low perfusion limited to the left thalamus. These findings suggest that the persistence of amnesia in this case was caused by the infarction of the mammillothalamic tract, and the recovery from aphasia may have resulted from the disappearance of surrounding edema.     

Cerebral embolism associated with Becker muscular dystrophy-related dilated cardiomyopathy]

A 65-year-old man with previous history of congestive heart failure and genetically proven Becker muscular dystrophy (BMD) was suddenly suffered from aphasia and right hemiplegia. Physical examination showed severe motor aphasia, right hemiplegia, and signs of left heart failure. An echocardiogram before the onset of aphasia showed markedly dilated left ventricle and decreased ventricular contraction. Intracardiac thrombus was not detected. Although his electrocardiogram on admission showed sinus rhythm, atrial fibrillation was noted at the time of neurological deterioration. MRI of the brain revealed acute infarction in the territory of the left middle cerebral artery and the left anterior inferior cerebellar artery. MR angiography showed vascular occlusion at the left M2 segment. Cerebral embolism due to atrial fibrillation associated with BMD-related DCM was diagnosed. While an administration of anti-coagulant, diuretics, and dopamine relieved his respiratory distress and right hemiplegia, severe motor aphasia persisted. Cerebral embolism may be a notable complication in patients with BMD presenting with late-life expression of skeletal muscular weakness and antecedent cardiac involvement.     

A case of unilateral ischemic stroke with ipsilateral limb-kinetic apraxia]

A 74-year-old man developed aphasia, weakness of the right upper extremity and left limb-kinetic apraxia. Brain MRI showed an infarct in the area supplied by the left middle cerebral artery. Cerebral angiography revealed high grade stenosis with plaques of bilateral proximal internal carotid arteries. The carotid endarterectomy of the left carotid artery was performed three months later. After this operation his left limb-kinetic apraxia improved. We considered transhemispheric diaschisis, callosal apraxia or diagonistic dyspraxia as a possible cause of this rare symptom.     

A case of slowly progressive aphasia without generalized dementia in a Japanese patient

A Japanese patient with a 4-year history of slowly progressive aphasia without generalized dementia is described. From 1985 on, this 61-yr-old right-handed dentist showed insidiously progressive deterioration in his speech and auditory comprehension, but has no memory disturbance, disorientation of space, time or persons, acalculia or other impairments in his behavior. His personality changes are unremarkable. He still treats patients at his clinic. General physical and neurological examinations were normal. CT and MRI (1.5T) scans showed widening of the left sylvian fissure and lateral ventricle without any vascular lesions. A PET scan demonstrated focal hypometabolism restricted to the left temporal lobe. The clinical course and picture of our patient corresponds, well to those of slowly progressive aphasia without generalized dementia, described by Mesulam.     

An autopsied case of corticobasal degeneration with onset of nonfluent aphasia revealing symmetrical cerebral involvement

Herein we describe a patient with established corticobasal degeneration with onset of nonfluent aphasia and showing symmetrical cerebral involvement. A 64-year-old man with a speech disorder for 2 years visited our hospital. He had nonfluent aphasia (reduced spontaneous speech, loss of intonation, anomia, repetition disorder, and difficulty in speaking short sentences). He also showed right-sided motor neglect, hypertonus of the left lower limb, a mask-like facial expression, and difficulty in closing his eyes. He was restless and walked around even during examination, suggesting frontotemporal dementia (FTD). Single-photon emission computed tomography (SPECT) revealed symmetrical reduction of cerebral blood flow in the bilateral fronto-temporo-parietal lobes. His neurological condition deteriorated gradually and a year later he could not speak comprehensive sentences. Magnetic resonance imaging (MRI) of the head at age 70 showed symmetrical atrophy of the bilateral fronto-temporal lobes. He died of respiratory failure after clinical problems lasting ten years. On pathological examination, the fixed brain weighed 1,010 g and showed bilateral symmetrical atrophy of the frontal lobes. Histopathological examination revealed neuronal loss and gliosis in the frontal lobes, especially in the frontal convexity, superior frontal gyrus and precentral gyrus. Gallyas-Braak silver staining showed astrocytic plaques, argyrophilic threads and coiled bodies mainly in the frontal lobes. The substantia nigra showed severe neuronal loss on both sides and presence of free melanin. Pathological diagnosis was corticobasal degeneration (CBD). We believe that the patient had nonfluent aphasia and FTD reflected in bilateral degeneration of the frontal lobes. Some cases of CBD may present with symmetrical degeneration of the brain, even though left-hemisphere symptoms such as aphasia reveal themselves at an early stage.     

Cerebral embolism following trivial trauma in children--report of three cases]

Three cases of cerebral embolism secondary to trivial trauma are reported. Case 1: A 12-year-old male suffered a severe headache followed by a generalized convulsion after he turned his head when he was flying a kite. A neurological examination on admission demonstrated right hemiparesis and aphasia. A CT revealed a low density in the left putamen, temporal lobe and frontal lobe. Left carotid angiography (CAG) showed irregular narrowing of the internal carotid with an embolic occlusion and narrowing of the middle cerebral artery with the intraluminal presence of emboli both in the anterior and middle cerebral arteries. He is now doing well but has right hemiparesis. Case 2: This 6-year-old female could not grasp chopsticks and had neck pain 10 minutes after being pulled up by the right arm by her father. Neurological examination demonstrated a right hemiparesis and aphasia. A CT scan and magnetic resonance imaging (MRI) of the head showed an infarcted area in the left caudate head, anterior limb of the internal capsule and putamen. Left CAG revealed an obstruction of the trunk of the middle cerebral artery. She has slight weakness in her right extremities. Case 3: This 11-year-old female noted a weakness in her left lower limb soon after her hair was pulled backward. On admission, a neurological examination failed to demonstrate any abnormality. CT showed an ill defined low density lesion in the right putamen. MRI revealed a high intensity lesion in a T2 weighted image. Right CAG showed an irregularity of the arterial wall in the cavernous portion of the right internal carotid artery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Wernicke's aphasia after putaminal hemorrhage: Unusual clinical and SPECT findings

We present a case of a man with a putaminal hemorrhage who presented in the acute stage with a 'classic' Wernicke's aphasia. CT and MRI scans showed a large left basal ganglia hemorrhage involving the posteriorputamen. A SPECT scan performed acutely demonstrated decreased perfusion in the left temporal cortex, frontal cortex and white matter and to the left basal ganglia from the hemorrhage itself. Within five days his language abilities improved dramatically, with complete resolution of his aphasia over the course of six weeks. Repeat SPECT scans continued to show the structural changes to the left putamen and left hemisphere hypoperfusion, suggesting that the resolution of the language symptoms did not correlate with the structural and perfusion changes. Various theories advanced in the literature regarding the pathophysiological mechanisms causing aphasia due to subcortical lesions cannot completely explain the findings of our case. These hypotheses are reviewed and discussed.     

Nonconvulsive status epilepticus presenting as a subacute progressive aphasia.

We report a 62-year-old man with non-convulsive status epilepticus (NCSE) presenting as a progressive aphasia that developed insidiously over 5 weeks. On video-EEG monitoring, aggravation of the aphasia coincided with occurrence of seizure activities arising from the left fronto-temporal area. Brain MRI was noncontributory but a fluorodeoxyglucose-PET scan revealed a hypometabolism in the left anterior temporal area. Following anticonvulsant treatment, aphasia recovered gradually over several weeks despite prompt resolution of epileptic discharges on EEG. Our patient's findings, gradual onset of isolated aphasia with gradual resolution after initiation of treatment, may differ from previously reported cases with aphasic status epilepticus because their aphasia showed abrupt onset and rapid resolution with anticonvulsant medication.     

Bilingual aphasia due to spontaneous acute subdural haematoma from a ruptured intracranial infectious aneurysm

We report a case of spontaneous subdural haematoma due to ruptured intracranial infectious aneurysm, presenting with bilingual aphasia and illustrating differential language recovery. A 62-year-old right-handed bilingual gentleman, with a diagnosis of infective endocarditis, developed headache and became expressively aphasic in the English language. Three days later he was receptively and expressively aphasic in both English and Arabic. Cranial MRI scans showed a left-sided acute subdural haematoma with mass effect and midline shift. Contrast CT brain scans showed an enhancing speck adjacent to the clot and cerebral angiogram confirmed a distal middle cerebral artery aneurysm. He underwent image-guided craniotomy, evacuation of the subdural haematoma and excision of the aneurysm. Histopathological examination was consistent with an infectious intracranial aneurysm. Postoperatively his aphasia did not improve immediately. He had widened pulse pressure due to severe aortic regurgitation, confirmed on echocardiography. He underwent aortic valve replacement and mitral valve repair, following which his aphasia recovered gradually. Initially the recovery of his language was limited to Arabic. About a week later he recovered his English language as well. At 3-year follow-up he is doing well and has no neurological deficits. His aphasia has recovered completely. The present case is unique because of (a) presence of pure subdural haematoma, and (b) the differential susceptibility and recovery of native (L1) and acquired language (L2) in presence of a common pathology. The neurology of language in a bilingual is analysed and possible mechanisms discussed.     

The clinical application of the arcuate fasciculus for stroke patients with aphasia: a diffusion tensor tractography study

Little is known about the clinical usefulness of diffusion tensor tractography (DTT) for the arcuate fasciculus (AF) in stroke patients with aphasia. Using DTT, we attempted to investigate the clinical usefulness of the AF in patients with aphasia. Five stroke patients and 7 age- and sex-matched normal subjects were recruited for this study. We recruited stroke patients with language dysfunction who had lesions in the left corona radiata and basal ganglia level. DTT for the AF was reconstructed using DTI-studio software. Korean-Western Aphasia Battery (K-WAB) was used for measurement of language function. Patient 1, who showed mild dysarthria, revealed a normal left AF in terms of integrity and DTT parameters. In patient 2, with conduction aphasia, the left AF showed partial injury; however, the integrity of the left AF was spared. Patients 3 and 4, who had no brain lesions at Broca's area on conventional brain MRI, showed disruptions of the left AF over the stroke lesions after originating from Wernicke's area and they presented with Broca's aphasia. Patient 5 revealed global aphasia on K-WAB and the left AF was not reconstructed due to severe injury and Wallerian degeneration. We found that DTT for the AF could provide useful information on the presence or severity of injury of the AF, which could not be detected on conventional brain MRI in stoke patients. In addition, it could be helpful in classification of the aphasia type of stroke patients.     

Molecular neuroimaging in primary progressive aphasia with predominant agraphia

A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy. Fluorodeoxyglucose-F18 positron emission tomography showed hypometabolism in the lateral left frontal lobe, including Exner’s area. Beta-amyloid and tau-positron emission tomography scans were negative, indicating the etiology was not Alzheimer’s disease. The underlying neurodegenerative process is most likely related to TDP-43, although a 4-repeat tauopathy cannot be excluded. Following his clinical evolution, and ultimately identifying the underlying pathology from autopsy, will elucidate the etiology of this interesting clinical presentation.     

Landau-Kleffner syndrome: a case report

A healthy 5 year old boy developed aphasia, attention disorder and hyperkinesia preceded by transient formed visual hallucinations and emotional outburst, immediately after a stressful event of forced separation from his father. EEG showed generalized epileptiform activity. He was diagnosed as Landau-Kleffner syndrome (LKS). CT and MRI of the brain were normal. SPECT showed left mesial temporal hypoperfusion. He improved on antiepileptics and ACTH.     

Excellent recovery of aphasia in a patient with complete injury of the arcuate fasciculus in the dominant hemisphere

The arcuate fasciculus (AF) is the neural tract that connects Wernicke's area and Broca's area. The main role of the AF is speech repetition; therefore, injury to the AF typically causes conduction aphasia. We report on a patient who showed excellent recovery of aphasia despite complete injury of the AF due to a cerebral infarct. A 54-year-old, right-handed male presented with aphasia and right hemiparesis. Brain MRI showed an infarct in the left centrum semiovale and corona radiata. Diffusion tensor tractography for the AF was reconstructed using DTI-studio software. The Korean-Western Aphasia Battery (K-WAB) was used for measurement of language function. On K-WAB at 1 week after onset, his aphasia type was compatible with global aphasia (aphasia quotient: 12‰, fluency: 5‰, comprehension: 24‰, repetition: 15‰, and naming: 31‰). The patient underwent rehabilitative therapy, including language therapy and medication, which is known to facilitate recovery from aphasia, for a period of 24 months. His aphasia had improved to a nearly normal state at 30 months after onset; aphasia quotient: 93‰ (fluency: 91‰, comprehension: 92‰, repetition: 85‰, and naming: 96‰). The left AF showed a complete disruption on 27-month diffusion tensor tractography. Findings from this study suggest the possibility that aphasia might show good recovery, even in cases of severe injury of the AF.     

Two cases of cerebral embolism showing global aphasia without hemiparesis]

We report two cases of typical global aphasia without hemiparesis due to cerebral embolism. Case 1 was a 65-year-old right-handed man with a history of old myocardial infarction. No spontaneous speech was noted by his family. Neurological examination upon admission revealed confusional state, global aphasia, conjugate deviation to the left and slight drift of the outstretched right limbs. The right hemiparesis rapidly recovered after admission. CT scan performed on the second hospital day showed discrete low density areas in the left posterior frontal lobe and left temporo-parietal regions. The extent and severity of his global aphasia were unchanged. The second case was an 82-year-old right-handed man with a history of atrial fibrillation. He was admitted to our hospital one hour after he was found unable to speak. Neurological examination upon admission revealed global aphasia, conjugate deviation to the left and suspected right homonymous hemianopia by confrontation. There was no sign of hemiparesis. CT scan showed extensive low density area in the left temporo-parietal regions. In both cases, cerebral angiography failed to demonstrate any occlusion of intra- and extra-cranial blood vessels. IMP-SPECT showed a depression of cerebral blood flow in the left anterior and posterior watershed areas in case 1 and 2. In the literature, there have been 20 cases of global aphasia without hemiparesis including our two cases. In many cases, the initial symptom was inability or difficulty in speaking.(ABSTRACT TRUNCATED AT 250 WORDS)     

MELAS-like episodes in an adult case with cytochrome c oxidase deficiency]

A 30-year-old man was hospitalized with dysarthria and weakness of his right arm and leg. Three months previously, he had noticed numbness and weakness of his right shoulder, which spread to involve his left leg but which improved after 8 months. On admission, neurological examination revealed limb kinetic apraxia and constructive apraxia of the right hand, motor aphasia, dysarthria, and spastic quadriplegia. Sensory examination revealed hyperalgesia and dysesthesia in the right arm and left leg. Deep tendon reflexes were hyperactive in all four extremities. And he had bilateral Babinski signs. Laboratory examination revealed pH 7.38, PCO2 46.1 Torr, PO2 93.4 Torr, BE 1.7, and blood lactate, 9.0 mg/dl (normal 5-20 mg/dl). Cerebrospinal fluid lactate level was 20.0 mg/dl. pyruvate 1.34 mg/dl. and protein 83 mg/dl. Blood lactate and pyruvate values were markedly elevated after aerobic exercise. T2WI brain MRI showed scattered high signal lesions in the left precentral and postcentral gyrus, right paracentral lobes, both superior frontal gyri, and right superior temporal gyrus. Right biceps brachi biopsy showed almost complete cytochrome c oxidase (COX) deficiency. There were no ragged-red fibers. There was marked decrease of COX activity: 2.7 nmol/min/mg-mitochondrial protein (normal range: 33.0 +/- 16.1, n = 7) in the biopsied muscle. Open brain biopsy (after permission from the patient and his family) revealed gliosis and perivascular infiltration of lymphocytes and macrophages without vascular proliferation. There was no mitochondrial DNA mutations, deletion or duplication, including tRNA-Leu 3243, 8993, 3271, 9176, 3291, and tRNA-Lys 8344, 8356, and 8363. From these findings, a diagnosis of COX deficiency presenting as MELAS-like episodes was done. His mother also showed abnormality on aerobic exercise test, but she had no episode of stroke or neurological dysfunction. Six months later, his aphasia and apraxia of the right hand had resolved, and at discharge he was able to ambulate with a cane. Ten months later, he returned to his work. There has been no recurrence of neurologic symptoms over the next 3 years and 10 months. This patient appears to represent a rare case of adult onset COX deficiency presenting as MELAS-like episodes.     

Surface anatomy scanning image by MRI in herpes simplex encephalitis]

We present surface anatomy scanning (SAS) image by weighted-summation technique using MRI images in herpes simplex encephalitis. Recently, SAS has been developed as a technique that visualizes brain surface structures. This patient was a 64-year-old male who suffered from aphasia and memory disturbance after herpes simplex encephalitis. He was transferred to our hospital for rehabilitation. Neuropsychological tests showed Wernicke's aphasia and memory impairment. SAS was performed for the purpose of analyzing these neuropsychological deficits, especially the Wernicke's aphasia. In SAS image, sulci were enhanced and gyri were easy to identify clearly. The affected structures disclosed low intensity areas in the entire left temporal lobe, including the superior temporal gyrus. It was clarified that the damage to the left superior temporal gyrus resulted in his Wernicke's aphasia. We think that SAS is very useful for cortical lesion analysis, not only in encephalitis but also in other disorders presenting higher brain dysfunctions.     

Sequential changes of brain CT and MRI after febrile status epilepticus in a 6-year-old girl

Brain CT or MRI occasionally shows transient or permanent changes in the brain after status epilepticus (SE). The mechanism for these changes has not been well elucidated. We performed repeated imaging studies on a patient with febrile SE characterized by right hemiconvulsion. CT showed transient mild edema on both hemispheres immediately after the cessation of SE. The edema improved the next day. But aphasia and right hemiparesis were observed. On day 17, CT revealed edema on left hemisphere and MRI showed a high signal intensity in cortex and subcortical white matter of the left hemisphere on T2-weighted images. Although right hemiparesis and aphasia were improved, severe atrophy of the left hemisphere was noted on CT and MRI. The results suggest that brain edema observed in several days after SE but not edema observed immediately after the cessation of SE is more pathological for the permanent brain damage. Possible mechanisms of the initial brain edema and the second edema preceded severe atrophy in left hemisphere were discussed.     

Alpha-interferon therapy in a case of probable progressive multifocal leukoencephalopathy.

Probable progressive multifocal leukoencephalopathy (PML) was diagnosed on the basis of clinical picture and magnetic resonance imaging in a 63-year-old man with a complete remission of a non-Hodgkin's lymphoma. After the introduction of intramuscular alpha-interferon therapy, his neurological state and MRI findings showed a clear improvement. Eighteen months after the onset of first symptoms the patient has significantly recovered from both aphasia and motor impairment, and shows only a mild attentional deficit.     

Callosal disconnection syndrome caused by left hemisphere infarction

A 49-year old right-handed taxi-driver experienced right upper limb weakness and global aphasia following internal carotid artery occlusion. Five months later, aphasia and hemiparesis had resolved but he complained of difficulties in his daily activities, termed "inner conflict". Specific testings disclosed typical features of a callosal syndrome: left unilateral ideomotor apraxia, left hand agraphia, left tactile dysnomia, right hand constructional difficulties, and left ear extinction on dichotic listening. Naming and recognition of tachistoscopically presented images or words was similar to that of classical split-brain studies. MRI showed a left-hemisphere periventricular increased signal involving the callosal outflow of the whole posterior half of the corpus callosum and partially destroying the splenium. Fibers from the anterior half of the body and the genu were spared. According to clinical and MRI findings in this case and other documented cases of callosal syndrome, localization of callosal transfer of various hemisphere functions is discussed. The relevance of callosal symptoms to the diagnosis of watershed infarcts is emphasized.     

Thalamic aphasia associated with mitochondrial encephalopathy,lactic acidosis,and stroke-like episodes: A case report

BackgroundMitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with aphasia is a rare disorder, with the associated aphasia reported as either Wernicke’s or Broca’s. Herein, we report a patient with MELAS complicated by thalamic aphasia.CaseA 15-year-old right-handed girl presented with headache, nausea, right homonymous hemianopsia, and aphasia. She could repeat words said by others, but had word-finding difficulty, paraphasia, and dysgraphia. Brain MRI revealed abnormal signals from the left occipital lobe to the temporal lobe and left thalamus, but Wernicke’s area and Broca’s area were not involved. Additionally, she had short stature, lactic acidosis, bilateral sensorineural hearing loss, and a maternal family history of diabetes and mild deafness. Based on clinical findings and the presence of a mitochondrial A3243G mutation, she was diagnosed with MELAS. With treatment, the brain MRI lesions disappeared and her symptoms improved. Her aphasia was classified as amnesic aphasia because she could repeat words, despite having word-finding difficulty, paraphasia, and dysgraphia. Based on MRI findings of a left thalamic lesion, we diagnosed her with thalamic aphasia.ConclusionThalamic aphasia may be caused by MELAS. Assessment of whether repetition is preserved is important for classifying aphasia.