Encephalitis that primarily involves limbic system structures such as the hippocampus and parahippocampal gyrus has been described in early papers, most commonly characterized by a subacute progressive impairment of short-term memory, psychiatric features and seizures. While these findings might be caused by viral infections or systemic autoimmune disorders, many patients with limbic encephalitis have an immune-mediated etiology (paraneoplastic or not) characterized with serum or CSF antineuronal antibodies. This case reports about non-paraneoplastic autoantibody-negative limbic encephalitis in which there are no detection of antigens and no evidence of tumors.
Antibodies ; Encephalitis ; Hippocampus ; Humans ; Limbic Encephalitis* ; Limbic System ; Memory* ; Memory, Short-Term ; Parahippocampal Gyrus ; Seizures

Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. There are more than 35 different types of spinocerebellar ataxias, each caused by a different genetic mutation. Spinocerebellar ataxia type 2 (SCA2) is a subtype of type I autosomal dominant cerebellar ataxia (ADCA type I) characterized by truncal ataxia, dysarthria, slowed saccades and less commonly ophthalmoparesis and chorea. The age at onset varies from 3 to 79 years (mean 33). Usually, the first symptom of the disease is the gait ataxia, followed by the cerebellar dysarthria. Of late, other clinical manifestations of SCA2 are the cognitive dysfunctions, which include frontal executive impairment, verbal short-term memory deficits as well as reduction of attention and concentration. We report a 56-year old woman identified as spinocerebellar ataxia type 2 (SCA2) with only clinical feature of memory impairment.
Ataxia ; Cerebellar Ataxia ; Chorea ; Dysarthria ; Eye Movements ; Female ; Gait ; Gait Ataxia ; Hand ; Humans ; Memory* ; Memory, Short-Term ; Middle Aged ; Ophthalmoplegia ; Saccades ; Spinocerebellar Ataxias*

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

Dementia pugilistica (DP) or chronic traumatic encephalopathy (CTE) is a neurodegenerative disease or dementia that may affect amateur or professional boxers as well as athletes in other sports who suffer concussions. The condition is thought to affect around 15% to 20% of professional boxers and caused by repeated concussive or subconcussive blows. CTE was in the past referred to as dementia pugilistica, which reflected the prevailing notion that this condition was restricted to boxers. Recent research, however, has demonstrated neuropathological evidence of CTE in retired American football players, a professional wrestler, a professional hockey player and a soccer player, as well as in nonathletes. It is probable that many individuals are susceptible to CTE, including those who experience falls, motor vehicle accidents, assaults, epileptic seizures, or military combat, and that repeated mild closed head trauma of diverse origin is capable of instigating the neurodegenerative cascade leading to CTE. We report a 62-year old man suspicious of dementia pugilistica with clinical features of frontotemporal dementia and parkinsonism.
Athletes ; Brain Injury, Chronic ; Dementia ; Epilepsy ; Football ; Frontotemporal Dementia ; Head Injuries, Closed ; Hockey ; Humans ; Military Personnel ; Motor Vehicles ; Neurodegenerative Diseases ; Parkinsonian Disorders ; Soccer ; Sports

BACKGROUND: Neuromyelitis optica (NMO) is characterized by optic neuritis and longitudinally extensive transverse myelitis. Generally, the brain had been considered healthy in NMO patients, though recent studies have demonstrated that T2-weighted abnormalities may be observed in various brain regions. Logically, NMO brain lesions are localized at sites of high aquaporin-4 expression. CASE REPORT: A 68-year-old right-handed man with dysuria, weakness in the bilateral upper and lower limbs, and decreased sensation of the lower extremities, was diagnosed with neuromyelitis optica. The patient was gradually speaking less, was showing reduced interest in hobbies, and had undergone changes in character and behavior. An examination was performed using the Seoul Neuropsychological Screening Battery (SNSB), which revealed that the profile of frontal lobe dysfunctions was prominent as compared with other cognitive domains. The patient was treated with prednisolone and azathioprine for about 1 year without recurrence, and showed prognostic improvement according to further SNSB testing. CONCLUSIONS: Further studies are considered necessary in order to find the most effective medication regimen for improving cognitive functions in those accurately diagnosed with NMO, and to develop systematic treatment using even more diversified immune-related agents.
Aged ; Azathioprine ; Brain ; Dysuria ; Frontal Lobe* ; Hobbies ; Humans ; Logic ; Lower Extremity ; Mass Screening ; Myelitis, Transverse ; Neuromyelitis Optica* ; Optic Neuritis ; Prednisolone ; Recurrence ; Sensation ; Seoul

No abstract available.
Cerebrospinal Fluid* ; Constriction, Pathologic*

Creutzfeldt-Jakob disease (CJD) is a degenerative neurological disorder that is incurable and invariably fatal. It is characterized by rapidly progressive dementia presenting with memory loss, personality changes and hallucinations. The symptoms of CJD are caused by progressive death of neurons in the central nervous system, which is associated with build-up of the abnormal prion proteins forming amyloids. In human, CJD can be acquired genetically through a mutation of the gene encoding for the prion protein (PRNP). This occurs in only 5-10% of all CJD cases. We report a 64-year old woman with CJD carrying a V180I mutation that features late onset, rapid progression, no periodic sharp wave complexes on electroencephalography, and cortical signal change and edema in bilateral frontotemporoparietal lobes and basal ganglia on MRI.
Amyloid ; Basal Ganglia ; Central Nervous System ; Creutzfeldt-Jakob Syndrome ; Dementia ; Depression ; Edema ; Electroencephalography ; Female ; Hallucinations ; Humans ; Lifting ; Memory Disorders ; Nervous System Diseases ; Neurons ; Prions