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

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

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

Posterior cortical atrophy(PCA) is a presenile dementia that presents primarily with signs and symptoms of cortical visual dysfunction, while memory is relatively preserved until the late stage of the disease. We report a patient with PCA, confirmed by brain magnetic resonance imaging (MRI) and F18-fluorodeoxyglucose positron emission tomography(FDG PET). A 58-year-old right-handed woman presented initially with visual dimness and difficulty finding things around her. She had partial Balint's syndrome, partial Gerstmann syndrome, and idiomotor apraxia. She also had a mild memory disturbance, but preserved insight of her disease. Neuropsychological evaluation showed decreased parietal and left temporal functions bilaterally. Brain MRI and F18-FDG PET revealed typical bilateral occipitoparietal atrophy and hypometabolism, which were slightly worse on the right side. Cholinesterase inhibitor administration for 6 months improved the memory impairment slightly, but not the cortical visual dysfunction. This is a typical case of PCA, confirmed by neurologic signs and imaging findings.
Alzheimer Disease ; Apraxias ; Atrophy ; Brain ; Cholinesterases ; Dementia ; Electrons ; Female ; Gerstmann Syndrome ; Glycogen Storage Disease Type VI ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Neurologic Manifestations ; Passive Cutaneous Anaphylaxis

BACKGROUND: It remains unclear whether the four signs of Gerstmann syndrome are a cluster because the neuronal nets responsible for these symptoms are closer together, or because they shares a common networks. If the latter is correct, then with degenerative disorders such as Alzheimer's disease, each sign associated with Gerstmann syndrome should correlate with the other three signs more closely than they correlate with other cognitive dysfunctions. METHODS: Cluster and correlation analyses for various cognitive deficits including signs of Gerstmann syndrome were done among sixty-nine patients with probable Alzheimer's disease. RESULTS: The four signs of Gerstmann syndrome did not cluster together. With the exception of calculation and writing, other signs including right-left orientation and finger naming placed in other groups and did not significantly correlate each other. CONCLUSIONS: A detailed statistical analysis of the tetrad showed that Gerstmann syndrome was not attributable to a common neuronal network, and the phenomenological association of the four signs may be related to the anatomical proximity of the different networks mediating these functions.
Alzheimer Disease* ; Fingers ; Gerstmann Syndrome* ; Humans ; Negotiating ; Neurons ; Writing

Symptomatic cerebral amyloid angiopathy (CAA) is characterized by lobar cerebral hemorrhage, transient neurological symptoms, and dementia with leukoencephalopathy in the absence of hypertension or coagulopathy. Contrary to its common appearance in English literature, pathologically confirmed CAA has rarely been reported in Korea. A 77-year-old normotensive, nondemented woman was admitted for a case of incidentally found intracerebral lobar hemorrhage at the left occipito-parietal area. On neurological examination, she displayed symptoms of a drowsy mentality and Gerstmann syndrome. Light-microscopically, medium to large cortical and meningeal vessels appeared to be thickened with amorphous glassy materials showing apple-green birefringence under polarized light on Congo red stain, which is consistent with CAA. The amyloid deposits were detected in the medium-sized vessels of the leptomeninges and cerebral cortex, but not in the capillaries and small arterioles in the white matter. Electron microscopy revealed amyloid fibrils and basal lamina duplication, indicating damaged vessels. Careful cortical biopsy during evacuating hematoma for histopathologic and ultrastructural examination is essential for diagnosis of CAA in case of clinical probability because the ignored sampling procedure and sampling error of patchy, segmental distribution of affected vessels might be ascribed to rare incidence of CAA in Korea.
Aged ; Amyloid ; Arterioles ; Basement Membrane ; Biopsy ; Birefringence ; Capillaries ; Cerebral Amyloid Angiopathy* ; Cerebral Cortex ; Cerebral Hemorrhage ; Congo Red ; Dementia ; Diagnosis ; Female ; Gerstmann Syndrome ; Hematoma ; Hemorrhage ; Humans ; Hypertension ; Incidence ; Korea ; Leukoencephalopathies ; Microscopy, Electron ; Neurologic Examination ; Plaque, Amyloid ; Selection Bias

OBJECTIVETo analyze clinical features of patients with Gerstmann syndrome (GS).

METHODSWe retrospectively analysed the clinical manifestations of 7 patients (6 men and 1 woman) with GS secondary to cerebral vascular diseases and reviewed the literatures.

RESULTSThe age ranged from 51 to 70 years with a mean of 70 years. They all had sudden onset and the tetrad of GS-finger agnosia, left-right disorientation, agraphia and acalculia, 3 patients accompanied by incomplete aphasia, 3 by anomic aphasia, 2 by alexia and 1 by constructional apraxia. Cranial computed tomographic scan showed low-density focus of the left parietal lobe in 6 cases and high-density focus of the left parietal lobe in 1 case.

CONCLUSIONGS has the high value in localization and the lesion is mainly localized to angular gyrus of the dominant hemisphere.

Aged ; Aged, 80 and over ; Cerebral Infarction ; complications ; Female ; Gerstmann Syndrome ; diagnosis ; diagnostic imaging ; etiology ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed