PURPOSE: To investigate the neuro-ophthalmic diagnosis and clinical manifestations of intracranial aneurysm. METHODS: A retrospective survey of 33 patients who were diagnosed with intracranial aneurysm and underwent neuro-ophthalmic examination from April 2008 to December 2016. Frequency of the first diagnosis of intracranial aneurysm in ophthalmology, neuro-ophthalmic diagnosis, location of intracranial aneurysm, examination of intracranial aneurysm rupture, and neurologic prognosis of Terson's syndrome patients were analyzed by image examination, neurosurgery, and ophthalmology chart review. RESULTS: Of the 33 patients, most patients (n = 31, 94%) were diagnosed with intracranial aneurysm at the neurosurgical department and only 2 patients were diagnosed initially at the ophthalmology department. Causes and association were: Terson's syndrome (n = 10, 30%), third cranial nerve palsy (n = 10, 30%), internclear ophthalmoplegia (n = 4, 12%), visual field defect (n = 3, 9%), optic atrophy (n = 3, 9%), sixth cranial nerve palsy (n = 2, 6%), and nystagmus (n = 1, 3%). The location of intracranial aneurysms were: anterior communicating artery (n = 13, 39%), medial communicating artery (n = 12, 36%), and posterior communicating artery (n = 5, 15%). Ten of 33 patients had Terson's syndrome, and 6 patients (60%) with Terson's syndrome had apermanent neurological disorder such as agnosia, gait disorder and conduct disorder. CONCLUSIONS: Third cranial nerve palsy was the most common neuro-ophthalmic disease in patients presenting with intracranial aneurysm. The neuro-ophthalmic prognoses for those diseases were relatively good, but, if Terson's syndrome was present, neurological disorders (agnosia, gait disorder, conduct disorder) were more likely to remain after treatment.
Abducens Nerve Diseases ; Agnosia ; Arteries ; Conduct Disorder ; Diagnosis ; Gait ; Humans ; Intracranial Aneurysm* ; Nervous System Diseases ; Neurosurgery ; Oculomotor Nerve ; Ophthalmology ; Ophthalmoplegia ; Optic Atrophy ; Paralysis ; Prognosis ; Retrospective Studies ; Rupture ; Visual Fields

Kluver-Bucy syndrome is defined as a rare neurobehavioral disorder with hyperphagia, hyperorality, hypersexuality, and visual agnosia. This syndrome is usually resulting from bilateral lesions of the anterior temporal lobe including amygdala. Although it could occur after various diseases, stroke is uncommon etiology. We report a patient with Kluver-Bucy syndrome due to bilateral posterior cerebral artery territory infarction.
Agnosia ; Amygdala ; Cerebral Infarction ; Humans ; Hyperphagia ; Infarction ; Infarction, Posterior Cerebral Artery* ; Kluver-Bucy Syndrome* ; Posterior Cerebral Artery ; Stroke ; Temporal Lobe

Gerstmann's syndrome, assigned to a lesion of the dominant parietal lobe, is a neurological disorder characterized by acalculia, agraphia, right-left disorientation and finger agnosia. Some studies report that these symptoms are also shown in other brain lesions. We report two patients who presented with this tetrad of symptoms in initial assessment. Their Brain MRI images both showed lesion of left frontal lobe. Over time, these symptoms became better but some still remained in last assessment. Accordingly, we suggest that a left frontal lesion cause Gerstmann's syndrome.
Agnosia ; Agraphia ; Brain ; Dyscalculia ; Frontal Lobe* ; Gerstmann Syndrome* ; Humans ; Magnetic Resonance Imaging ; Nervous System Diseases ; Parietal Lobe

The mechanisms and functional anatomy underlying the early stages of speech perception are still not well understood. Auditory agnosia is a deficit of auditory object processing defined as a disability to recognize spoken languages and/or nonverbal environmental sounds and music despite adequate hearing while spontaneous speech, reading and writing are preserved. Usually, either the bilateral or unilateral temporal lobe, especially the transverse gyral lesions, are responsible for auditory agnosia. Subcortical lesions without cortical damage rarely causes auditory agnosia. We present a 73-year-old right-handed male with generalized auditory agnosia caused by a unilateral subcortical lesion. He was not able to repeat or dictate but to perform fluent and comprehensible speech. He could understand and read written words and phrases. His auditory brainstem evoked potential and audiometry were intact. This case suggested that the subcortical lesion involving unilateral acoustic radiation could cause generalized auditory agnosia.
Acoustics ; Agnosia ; Audiometry ; Brain ; Brain Stem ; Evoked Potentials ; Hearing ; Humans ; Male ; Music ; Speech Perception ; Temporal Lobe ; Writing

Kluver-Bucy Syndrome (KBS) is consisting of hyperorality, emotional blunting, hypersexuality, altered dietary habits, visual and auditory agnosia. It has been reported in variable neurological diseases. However, only a few cases reported in epilepsy. We report a patient with unilateral temporal lobe epilepsy who presents transient hyperorality during seizure. A 46-year-old man has complex partial seizures which were abdominal aura followed by hyperorality and hand automatisms. Hyperorality was characterized by putting patient's hand or seizure button into his mouth. Brain MRI demonstrated right hippocampal sclerosis. The interictal and ictal SPECT suggested right temporal lobe dysfunction, and PET showed bitemporal hypometabolism. Rhythmic ictal activities were arising from right temporal region when patient presented hyperorality. We speculate that transient hyperorality in this patient could be a symptom of KBS. This case suggests that transient KBS can be occurred in a unilateral temporal lobe epilepsy when ictal discharges cause bitemporal dysfunction during temporal lobe seizure.
Agnosia ; Brain ; Epilepsy ; Epilepsy, Temporal Lobe ; Food Habits ; Hand ; Humans ; Kluver-Bucy Syndrome ; Middle Aged ; Mouth ; Sclerosis ; Seizures ; Temporal Lobe ; Tomography, Emission-Computed, Single-Photon

Frontotemporal lobar degeneration (FTLD) is a progressive dementia with prominent neuropsychiatric features, aphasia or both. FTLD predominantly affects the frontal and anterior part of temporal cortex. FTLD is classified into frontotemporal dementia (FTD), progressive nonfluent aphasia (PA), and semantic dementia (SD). FTLD is estimated to account for 20% of cases of degenerative dementia with presenile onset. This disease typically has onset in the mid- or early fifties. FTD is characterized by behavioral change and executive dysfunction, PA features a progressive nonfluent aphasia. SD is characterized by a progressive semantic aphasia and associative agnosia. Structural imaging shows atrophy of the frontal lobe and the anterior portion of the temporal lobe, bilaterally symmetric or asymmetric. Pathologically, FTLD can be classified into tau-positive pathology, tau-negative, ubiquitin positive pathology, dementia lacking distinctive histology. At present, there are no specific pharmacological therapies approved for use in any of the FTLD syndrome.
Agnosia ; Aphasia ; Atrophy ; Dementia ; Frontal Lobe ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration* ; Pathology ; Primary Progressive Nonfluent Aphasia ; Temporal Lobe ; Ubiquitin

Crossed aphasia refers to language disturbance induced by unilateral right hemisphere (non-language dominant) injury in right-handed people who had no previous history of brain damage. Crossed aphasia occurs in less than 2 percent who developed a aphasia. We report a case of a 49-year-old right handed man with language disturbance after right middle cerebral infarction. He showed nonfluent crossed aphasia with Gerstman syndrome such as right-left disorientation, finger agnosia, acalculia and agraphia, but not with apraxia and neglect. At 7 weeks after onset, language function indicated improvement in spontaneous speech and at 19 weeks after onset, improvement in spontaneous speech, comprehension, repetition, naming and reading.
Agnosia ; Agraphia ; Aphasia* ; Apraxias ; Brain ; Cerebral Infarction ; Comprehension ; Dyscalculia ; Hand ; Humans ; Infarction, Middle Cerebral Artery* ; Middle Aged

Optic aphasia is a rare syndrome in which patients are unable to name visually presented objects but have no difficulty in naming those objects on tactile or verbal presentation. We report a 79-year-old man who exhibited anomic aphasia after a left posterior cerebral artery territory infarction. His naming ability was intact on tactile and verbal semantic presentation. The results of the systematic assessment of visual processing of objects and letters indicated that he had optic aphasia with mixed features of visual associative agnosia. Interestingly, although he had difficulty reading Hanja (an ideogram), he could point to Hanja letters on verbal description of their meaning, suggesting that the processes of recognizing objects and Hanja share a common mechanism.
Aged ; Agnosia ; Anomia ; Aphasia* ; Dyslexia ; Humans ; Infarction ; Posterior Cerebral Artery ; Semantics

OBJECTIVETo investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS).

METHODSThe clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up.

RESULTSThe age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia. All patients had epileptic seizures. Partial motor seizures during sleep occurred in 8 patients, and other seizure type including atypical absence seizure and generalized tonic-clonic seizure were also observed. Psychological and behavioral abnormalities occurred in 9 patients. There were no abnormalities of hearing and neuro-imaging tests in all patients, and family histories were negative. All the patients had EEG abnormalities. Focal spike and waves of temporal lobe were recorded in 9 patients. Electrical status epilepticus during sleep (ESES) was observed on Video-EEG (VEEG) monitoring in 4 patients. Anti-epileptic drugs (AEDs) showed favorable effects on epileptic seizures, but no effects on aphasia. All patients responded to corticosteroid, and got language improved. Eight patients were followed up for long-term outcome. All patients were seizure free, while the level of language development was abnormal in 5 patients. The VEEG follow-up was conducted in 6 patients. Continuous epileptic discharges in slow sleep recurred in 2 patients after the discontinuation of steroid therapy.

CONCLUSIONSLKS is one of the childhood epileptic encephalopathy, and acquired aphasia and epileptic seizures are two main clinical characteristics. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very common in children with LKS. Focal epileptic discharges were often located in temporal area, and usually generalized, and could be continuous during sleep. AEDs could control seizure but had no effects on aphasia. Early use of full dose corticosteroids could improve the language significantly. Long-term follow up showed that language impairments often remained, but the outcome in terms of EEG and epileptic seizure was good.

Adrenal Cortex Hormones ; therapeutic use ; Age of Onset ; Agnosia ; drug therapy ; physiopathology ; Anticonvulsants ; therapeutic use ; Auditory Perceptual Disorders ; drug therapy ; physiopathology ; Brain ; drug effects ; physiopathology ; Child ; Child, Preschool ; Electroencephalography ; Female ; Follow-Up Studies ; Humans ; Landau-Kleffner Syndrome ; drug therapy ; physiopathology ; Male ; Prognosis ; Retrospective Studies ; Seizures ; drug therapy ; physiopathology ; Time Factors

The case of a 66-year-old woman with coexisting idiopathic basal ganglia calcification(IBGC) and dementia was presented. The calcification was detected in bilateral basal ganglia, dentate nucleus, and thalamus by brain imaging. Serum calcium and phosphorus levels were normal. The underlying diseases of calcification of basal ganglia such as parathyroid dysfunction and other infectious, toxic, or metabolic illness were excluded. The patient had memory impairment and frontal executive dysfunction without aphasia, agnosia, apraxia, and visuospatial impairment in neuropsychological test. It suggested that the cognitive impairment might be due to the dysfunction of frontal-subcortical circuit.
Aged ; Agnosia ; Aphasia ; Apraxias ; Basal Ganglia* ; Calcium ; Cerebellar Nuclei ; Dementia* ; Female ; Humans ; Memory ; Neuroimaging ; Neuropsychological Tests ; Phosphorus ; Thalamus