Genetic prion diseases account for about 10-15% of all cases of human prion disease and are caused by mutations in the prion protein gene. Gerstmann-Sträussler-Scheinker (GSS) disease is a rare genetic prion disease, which is characterized by slowly progressive cerebellar ataxia and the occurrence of cognitive decline in the later stage. P102L is the most common mutation in GSS. We report a patient with a P102L mutation that initially manifested as rapidly progressive dementia without cerebellar symptoms.
Cerebellar Ataxia ; Creutzfeldt-Jakob Syndrome ; Dementia ; Gerstmann-Straussler-Scheinker Disease ; Humans ; Prion Diseases ; Prions

No abstract available.
Arteriovenous Malformations* ; Gerstmann Syndrome* ; Hemorrhage* ; Rabeprazole*

BACKGROUND: Alzheimer's disease is a chronic neurodegenerative condition, mostly affecting the medial temporal lobe and associated neocortical structures. In this report, we present a rare clinical manifestation of this disease. CASE REPORT: A 61-year-old female with word finding difficulty and memory disturbances was diagnosed with Alzheimer's disease. Two years later, she complained of right homonymous hemianopia without optic ataxia, ocular apraxia, and simultagnosia. No findings other than parenchymal disease were apparent in magnetic resonance imaging and laboratory tests. CONCLUSIONS: In this case, in a patient initially diagnosed with Alzheimer's dementia with progressive disease, we found only homonymous hemianopia, without signs of Balint's syndrome or Gerstmann's syndrome. After careful investigation showing that Alzheimer's dementia with visual symptom was not associated with parenchymal disease, we concluded a case of atypical variant of Alzheimer's disease.
Alzheimer Disease ; Apraxias ; Ataxia ; Dementia* ; Female ; Gerstmann Syndrome ; Hemianopsia* ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Temporal Lobe

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

Giant serpentine aneurysms are uncommon types of aneurysmal disease and have angiographically authentic features. We report a case of a 44-year-old male with headache and seizure. He presented a giant serpentine aneurysm arising from the middle cerebral artery (MCA). It was a large intracranial aneurysm thrombosed as a mass-like lesion while it maintained its outflow drainage into the distal MCA branches. The balloon occlusion test (BOT) was performed to test the tolerance of temporary collateral circulation. Following routine cerebral angiography, we performed an endovascular embolization on the proximal artery of MCA. He was discharged from the hospital with alert mental status and mild Gerstmann syndrome. The short-term follow-up imaging studies showed the decreased mass effect, and the patient presented an improved Gerstmann syndrome. After a careful evaluation of BOT, an endovascular embolization can be one of the powerful therapeutic instruments for giant serpentine aneurysm.
Adult ; Aneurysm* ; Arteries ; Balloon Occlusion ; Cerebral Angiography ; Collateral Circulation ; Drainage ; Follow-Up Studies ; Gerstmann Syndrome ; Headache ; Humans ; Intracranial Aneurysm ; Male ; Middle Cerebral Artery* ; Seizures

BACKGROUND: Posterior cortical atrophy (PCA) is characterized by slowly progressive early onset dementia with cortical visual dysfunction and disproportionate atrophy of the posterior cortex. CASE REPORT: A 55-year-old right-handed woman developed visuo-spatial impairments that progressed rapidly into cortical blindness over the following 3 months. Neuro-psychological evaluation revealed Gerstmann syndrome and severe constructional impairments with all components of Balint syndrome. However, her memory, insight, and judgment were preserved. Her brain MRI was normal. However, 18F fluorodeoxyglucose positron emission tomography revealed a marked hypometabolism in the bilateral parieto-occipital region. CONCLUSIONS: Although rapid progression of visuo-spatial dysfunction without memory impairment occurred, we considered PCA as well.
Atrophy* ; Blindness, Cortical ; Brain ; Dementia ; Female ; Fluorodeoxyglucose F18 ; Gerstmann Syndrome ; Humans ; Judgment ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Passive Cutaneous Anaphylaxis ; Positron-Emission Tomography

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