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Neurologic Complications of Transplantation.

Neurologic disturbances including encephalopathy, seizures, and focal deficits complicate the course 10-30% of patients undergoing organ or … ng opportunistic infections in the months after transplantation. Other neurologic syndromes such as akinetic mutism and osmotic demyelination may also occur. While much of this neurologic dysfunction may be reversible if related to metabolic factors or drug toxicity (and the etiology is recognized and reversed), cases of multifocal cerebral infarction, hemorrhage …

Atypical epidermoid cyst with repetitive hemorrhages in the supracallosal region: case report.

A 63-year-old man presented with an unusual supracallosal epidermoid cyst with repetitive hemorrhages that initially manifested as severe he … the diagnosis of epidermoid cyst. Surgery was successfully performed without complications such as akinetic mutism, motor weakness, or chemical meningitis. The present case suggests that epidermoid cyst should be considered in the differential diagnosis of suspected cavernous malformation in any location.

Creutzfeldt-Jakob disease with E200K PRNP mutation: a case report and revision of the literature.

Creutzfeldt-Jakob disease (CJD) is typically characterized by rapidly progressive dementia and myoclonus, and it is caused by a conformation … ure. Later on, she developed also myoclonus, ataxia, spastic tetraplegia, and at last dementia with akinetic mutism. Usually, signs of degeneration of the pyramidal tracts occur in a small number of patients as the disease advances. Our report supports the variability of the clinical expression of the E200K genetic CJD. Further studies are needed to understand th …

[Bilateral anterior infarct and hydrocephalus aresorptivus. Complications of a ruptured aneurysm of the anterior communicating artery].

A 52-year old man suffered from a subarachnoid hemorrhage, out of a ruptured aneurysm of the anterior communicating artery. Due to the subarachnoid hemorrhage a hydrocephalus developed presenting the symptoms of akinesia and mutism. Arterial spasms of the anterior cerebral arteries led to bilateral ischemic infarction allowed by a distal anterior syndrome and signs of general brain damage. The combination of these complications of the ruptured aneurysm caused a complex syndrome which in the beginning resembled an apallic syndrome.

Dengue-Associated Acute Necrotizing Encephalopathy: A Case Report and Review of the Literature.

Dengue virus infection is increasingly recognized as a cause of neurological complications, though severe parenchymal involvement such as ac … ite prompt initiation of high-dose intravenous methylprednisolone, the patient developed persistent akinetic mutism, highlighting the poor prognosis in adult-onset cases. This case underscores the dissociation between severe radiological findings and relatively unremarkable CSF, emphasizes the diagnostic utility of MRI, and illustrates the therapeutic challenges …

[A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom].

A 79-year-old woman was admitted to our hospital, due to acute onset of left hemiparesis and disturbance of consciousness. Although her symptoms improved temporarily, she developed gait disturbance and cognitive deterioration 2 months after the onset. After that, she presented with myoclonus and startle response, followed by akinetic mutism within 8 months after the onset. Serial EEGs revealed no periodic synchronous discharge. Serial diffusion-weighted MRIs showed that high intensity lesions, which initially limited to the right cerebral cortex, gradually spread to the bilateral cerebral cort…

[Brain SPECT using Tc-99m-bicisate (ECD) in rapidly progressive dementia syndrome].

We present a 61-year-old male patient with progressive dementia. A brain SPECT with Tc-99m-bicisate was performed for confirmation of clinically suspected Alzheimer-dementia. At the time of the SPECT-investigation marked apraxia and aphasia besides severe dementia were present. Electrophysiological as well as anatomical neuroimaging findings showed non-diagnostic alterations. SPECT revealed distinct perfusion defects, which made Alzheimer Dementia unlikely. The further course of the patient was determined by rapidly progressive deterioration with development of akinetic mutism. Thereafter, inc…

[A case of abulia without memory disturbance due to infarction of the bilateral genua of the internal capsules].

A 68-year-old right handed man with treated hypertension presented with acute-onset somnolence without hemiparesis, dysarthria or sensory disturbance. Although he became laconic and his verbal responses were only in short terms, his replies were accurate and judgmental. A cranial MRI in axial, coronal, and sagittal section revealed small hemorrhagic infarcts essentially limited to the bilateral capsular genua without involvement of the inferior thalamic peduncles. A 123I-IMP single photon emission CT disclosed remarkable hypoperfusion in the bilateral frontal cortex. After a week of somnolence

L-3,4-dihydroxyphenylalanine (levodopa) lowers central nervous system S-adenosylmethionine concentrations in humans.

To determine whether levodopa reduces the levels of S-adenosylmethionine in the human central nervous system, cerebrospinal fluid (CSF) concentrations of S-adenosylmethionine, methionine, 3-methoxytyrosine, levodopa and 5-methyltetrahydrofolate were measured in six children with dopamine deficiency before and after treatment. In four, the lack of dopamine was secondary to a reduction in concentration of levodopa and these were treated with levodopa together with a peripheral dopa-decarboxylase inhibitor. In the other two, levodopa in the central nervous system naturally accumulated due to a co

Стероидно-реагирующий энцефалит коронавирусной болезни 2019 года.

Инфекция коронавируса 2019 года (COVID-19) способна нацелиться на центральную нервную систему, и несколько неврологических симптомов были описаны у пациентов с тяжелым респираторным расстройством. Здесь мы описали случай 60-летнего пациента с острым острым респираторным синдромом коронавируса 2 (SARS-COV-2), но только лёгкие респираторные аномалии, которые развили акинетический мутизм, вызванный энцефалитом. Магнитный резонансный снимок был отрицательным, в то время как электроэнцефалография показала общее замедление теты. Анализ цереброспинных жидкостей на этапе острой токсичности был отрицат

Совпадение в аннотации оригинала
Coronavirus disease 2019 (COVID-19) infection has the potential for targeting the central nervous system, and several neurological symptoms … drome coronavirus 2 (SARS-CoV-2) infection but only mild respiratory abnormalities who developed an akinetic mutism attributable to encephalitis. Magnetic resonance imaging was negative, whereas electroencephalography showed generalized theta slowing. Cerebrospinal fluid analyses during the acute stage were negative for SARS-CoV-2, positive for pleocytosis and hy …

Subacute Sclerosing Panencephalitis in a North African Cohort: Findings From the Post-Measles Epidemic Era.

Subacute sclerosing panencephalitis (SSPE) is a rare, fatal encephalitis caused by chronic measles virus infection. Its recent resurgence, p … lobulins G in cerebrospinal fluid and blood. Despite treatment, rapid clinical deterioration led to akinetic mutism and neurovegetative symptoms. This study alerts clinicians and radiologists to consider SSPE in children presenting with subacute cognitive decline, myoclonus and epilepsy with periodic complexes, associated with specific WM changes, particularly in …

[A case of Creutzfeldt-Jakob disease presenting with nonconvulsive status epilepticus in the early stages].

A 64-year-old Japanese woman presented with 1 week of recurrent convulsive seizures. At the time of admission, she was in a coma and did not … (NCSE) was suspected, and the patient was actively treated with anti-epileptic drugs. She developed akinetic mutism and generalized myoclonus 1 month after admission. Follow-up EEG studies disclosed periodic synchronous discharges. Abnormal prion protein in the cerebral fluid was detected using a real-time quaking-induced conversion assay. The clinical diagnosis …

A Chinese patient of P102L Gerstmann-Sträussler-Scheinker disease contains three other disease-associated mutations in SYNE1.

Gerstmann-Sträussler-Scheinker disease (GSS) with the P102L mutation in PRNP gene is characterized with progressive cerebellar dysfunction c … ng progressive dementia, myoclonus, pyramidal and extrapyramidal signs. She is still alive but with akinetic mutism 21 months after onset. Observation of intense signal changes in cortical regions (cortical ribboning) in diffusion weighted imaging (DWI) MRI scanning and positive protein 14-3-3 in cerebrospinal fluid (CSF) proposed the diagnosis of sporadic CJD. T …

Evaluating the causality of novel sequence variants in the prion protein gene by example.

The estimation of pathogenicity and penetrance of novel prion protein gene (PRNP) variants presents significant challenges, particularly in the absence of family history, which precludes the application of Mendelian segregation. Moreover, the ambiguities of prion disease pathophysiology renders conventional in silico predictions inconclusive. Here, we describe 2 patients with rapid cognitive decline progressing to akinetic mutism and death within 10 weeks of symptom onset, both of whom possessed the novel T201S variant in PRNP. Clinically, both satisfied diagnostic criteria for probable sporad…

[Distigmine bromide improves chronic intestinal pseudo-obstruction in a case of MELAS].

A 34-year-old man with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) showed chronic intestinal pseudo-obstruction (CIPO), which was improved by the administration of distigmine bromide. He exhibited generalized tonic clonic seizures at the age of 21, and mitochondrial DNA analysis showed the MELAS mutation. At the age of 34, he became akinetic mutism after nonconvulsive status epilepticus and needed enteral nutrition through a nasogasrtic tube. However, he developed abdominal distention and vomiting, and was diagnosed as CIPO, therefore tube feeding …

An unusual course of progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ T lymphocytopenia.

A case is reported of idiopathic CD4+T lymphocytopenia with progressive multifocal leukoencephalopathy and cervical lymph node tuberculosis. A 57 year old Japanese man presented with cervical lymphadenopathy and progressive neurological deficits, and six months later he developed akinetic mutism. He had a persistent severely depressed number of circulating CD4+T lymphocytes in the absence of human immunodeficiency virus infection. T1 weighted MRI showed a diffuse decreased signal intensity limited to the white matter without mass effect. A brain biopsy specimen had a morphology similar to that…

Three cases of Creutzfeldt-Jakob disease with prion protein gene codon180 mutation presenting with pathological laughing and crying.

Although there are no reports of pathological laughing and crying being observed in patients with Creutzfeldt-Jakob disease (CJD), the autho … ical laughing and crying co-occured with the startle reaction and stopped right before the onset of akinetic mutism, and the degree of both symptoms was almost parallel during this period. On the basis of MRI and autopsy findings, pathological laughing and crying was suspected of being induced by the widespread cerebral cortical involvement that is characteristic …

[The locked-in syndrome. A case report].

Based on five of our own cases and the literature on hand the etiological, clinical, diagnostic and intensive therapeutic aspects of the syndrome are represented. Special emphasis is placed on the possible role of the oral contraceptives in triggering this syndrome.