Sporadic Creutzfeldt-Jakob Disease With Slow Progression:Report of One Case / 中国医学科学院学报

Sporadic Creutzfeldt-Jakob disease(sCJD)is a prion-caused degenerative disease of the central nervous system,with the typical clinical manifestation of rapidly progressive dementia.The course of disease is less than 1 year in most patients and more than 2 years in only 2% to 3% patients.We reported a case of sCJD with expressive language disorder and slow progression in this paper.By summarizing the clinical manifestations and the electroencephalograhpy,MRI,and pathological features,we aimed to enrich the knowledge about the sCJD with slow progression.

Case report of Chagas disease reactivation: new diagnosis tool by direct microscopic observation of biopsy specimen and its preservation fluid

Abstract Chagas Disease is caused by Trypanosoma cruzi. This infection is endemic in the Americas region. Neurological Chagas reactivation is diagnosed through the visualization of the parasite in the cerebrospinal fluid, blood, or tissue samples. Herein, we report the visualization of trypomastigotes by direct microscopic observation of a brain biopsy specimen and its preservation fluid (PF) in a paitient infected with VIH and T. cruzi. This easy and simple diagnostic method coupled with quantitative polymerase chain reaction can be used in all tissue biopsies and PF of T. cruzi seropositive patients, suspected of Chagas disease reactivation.

Stereotactic biopsy for lesions in brainstem and deep brain: a single-center experience of 72 cases

Stereotactic biopsies for lesions in the brainstem and deep brain are rare. This study aimed to summarize our 6-year experience in the accurate diagnosis of lesions in the brain stem and deep brain and to discuss the technical note and strategies. From December 2011 to January 2018, 72 cases of intracranial lesions in the brainstem or deep in the lobes undergoing stereotactic biopsy were retrospectively reviewed. An individualized puncture path was designed based on the lesion's location and the image characteristics. The most common biopsy targets were deep in the lobes (43 cases, 59.7%), including frontal lobe (33 cases, 45.8%), temporal lobe (4 cases, 5.6%), parietal lobe (3 cases, 4.2%), and occipital lobe (3 cases, 4.2 %). There were 12 cases (16.7%) of the brainstem, including 8 cases (11.1%) of midbrain, and 4 cases (5.6%) of pons or brachium pontis. Other targets included internal capsule (2 cases, 2.8%), thalamus (3 cases, 4.2%), and basal ganglion (12 cases, 16.7%). As for complications, one patient developed acute intracerebral hemorrhage in the biopsy area at 2 h post-operation, and one patient had delayed intracerebral hemorrhage at 7 days post-operation. The remaining patients recovered well after surgery. There was no surgery-related death. The CT-MRI-guided stereotactic biopsy of lesions in the brainstem or deep in the brain has the advantages of high safety, accurate diagnosis, and low incidence of complications. It plays a crucial role in the diagnosis of atypical, microscopic, diffuse, multiple, and refractory lesions.

Encefalomielitis aguda diseminada por Mycoplasma pneumoniae en un niño previamente sano / Acute disseminated encephalomyelitis due to Mycoplasma pneumoniae in a previously healthy boy

Se presenta el caso de un niño de 5 años sin antecedentes de enfermedad, que se internó en terapia intensiva por convulsiones tónico-clónicas focalizadas en la cara y en el hemicuerpo derecho, con documentación de temperatura axilar de 37,4°C. Se descartó la presencia de gérmenes comunes y la etiología viral a través de estudios de muestras de líquido cefalorraquídeo (LCR). Se sospechó la presencia de Mycoplasma pneumoniae por comprobarse inmunofluorescencia positiva en suero para anticuerpos de clase IgM. El diagnóstico se confirmó mediante la detección del ADN de dicho patógeno sobre la biopsia cerebral efectuada por el método de la reacción en cadena de la polimerasa (PCR) y una histología compatible con encefalomielitis aguda diseminada. El paciente recibió tratamiento con claritromicina y su evolución fue favorable. Al menos dentro de nuestros conocimientos, este es el primer caso en el que se detectó ADN de M. pneumoniae en una biopsia cerebral por el método de PCR.

We present here the case of a previously healthy 5 year-old boy hospitalized in an intensive care unit due to tonic-clonic seizures focused on the face and right side of the body, and axillary temperature of 37.4 °C. Common bacterial and viral etiology was ruled out through studies of cerebrospinal fluid (CSF) samples. Mycoplasma pneumoniae was suspected by a positive immunofluorescence serum test for IgM class antibodies. Finally, with a brain biopsy, M. pneumoniae was confirmed by polymerase chain reaction (PCR) and acute disseminated encephalomyelitis by pathological anatomy. The patient was treated with clarithromycin and had an uneventful evolution. At least to our knowledge, this is the first case in which M. pneumoniae DNA was detected by PCR in a brain biopsy.

Stereotactic biopsies of brain lesions / Biópsia estereotáxica de lesões encefálicas

OBJECTIVE: In the majority of cases, the correct treatment of brain lesions is possible only when the histopathological diagnosis is made. Several deep-seated lesions near eloquent areas are not safely approached by the classical neurosurgical procedures. These patients can get benefit by a minimally invasive procedure. METHOD: We present a series of 176 consecutive patients submitted to stereotactic biopsies due to a great variety of brain lesions. RESULTS: Histological diagnosis found in this series: glioma in 40.1 percent of the patients, other neoplasms in 12.2 percent and infectious or inflammatory diseases in 29.1 percent. The result was inconclusive in 5.2 percent of the procedures. One patient died (0.6 percent) and two (1.2 percent) presented operative complications. The criteria, advantages and risks of the stereotactic biopsies are discussed. CONCLUSION: The efficacy of the method is adequate and morbid-mortality rates were low.

OBJETIVO: O diagnóstico anatomopatológico das lesões encefálicas é muitas vezes necessário para a instituição do tratamento adequado. Entretanto, muitas lesões localizadas profundamente no encéfalo ou em centros nervosos de grande importância funcional não podem ser acessadas sem riscos, com a aplicação dos procedimentos neurocirúrgicos habituais. MÉTODO: Apresentamos uma série de 176 doentes submetidos a biópsias estereotáxicas de lesões encefálicas. RESULTADOS: Em 40,1 por cento dos casos, o diagnóstico foi de glioma, em 12,2 por cento de outras neoplasias e em 29,1 por cento, de doenças infecciosas ou inflamatórias. O resultado foi inconclusivo em 5,2 por cento dos doentes. Um (0,6 por cento) doente faleceu e dois (1,2 por cento) apresentaram graves complicações operatórias. Os critérios de seleção, as vantagens e os riscos da biópsia estereotáxica são discutidos. CONCLUSÃO: A eficácia do método é boa e a morbimortalidade das biópsias estereotáxicas é baixa.

Is Brain Biopsy Rational in Shunt Operation of Normal Pressure Hydrocephalus in Elderly?

Early detection of AD in its initial or preclinical stage has become increasingly important. We report a 73-year-old woman who was confirmed to have AD by a cortical biopsy performed concomitantly during a shunt operation for hydrocephalus. The triads of hydrocephalus improved after the operation, as did follow-up neuropsychological and FDG-PET findings. However, 2.5 years after the operation, the patient developed cognitive declines with abnormal behaviors, which was considered to be an evolved form of AD from the preclinical stage.

Biopsia cerebral a mano alzada guiada por tomografía / Cerebral biopsy by a handfree procedure

Se presenta la experiencia del Servicio de Neurocirugía del Hospital Universitario San Vicente de Paúl con un procedimiento alternativo para realizar una biopsia cerebral, el cual se lleva a cabo a mano alzada y guiado por tomografía sin utilizar un marco estereotáxico. Se resalta su utilidad en los casos de lesiones supratentoriales y con tamaño mayor de 3 centímetros, en centros donde no se disponga de los métodos estereotáxicos tradicionales.

Scalp Defect, Injuries of Skull and Brain Parenchyme Caused by High Voltage Electrical Burn: A Case Report

The authors describe a case of accidental eletrical injury from high voltage direct current in a young worker, who was struck by the electric shock in the left occipito-parietal region. This case is interesting due to the development of left ooccipito-parietal parenchymal brain lesion with transient anomic aphasia and visual disturbances. We believe that is first report on parenchymal brain injury due to high voltage electrical burn confirmed pathologically in Korea. The patient had full thickness scalp defect, skull necrosis, and parenchymal brain injury. We performed one stage brain and dural biopsy with split rib graft cranioplasty after removal of devitalized skull and scalp flap surgery. The clinical presentation, radiological, pathological finding, and outcome are reviewed.

Alzheimer's Disease: Report of two cases

Alzheimer's disease (AD) is the most common etiology of dementia, that has not been previously reported in Korea. We have experienced two cases of Alzheimer's disease, one occurred in a 53-year-old man with dementia and the other occurred in a 36-year-old woman with a family history of early onset dementia. A neocortical biopsy was done to rule out the cause of dementia and showed diffusely scattered numerous senile plaques and neurofibrillary tangles in cortex of both cases. Interestingly, GFAP, an immunohistochemical stain showed strong positivity in neuritic plaques and the surrounding fibrillary gathering.

Alzheimer's Disease: Report of two cases

Alzheimer's disease (AD) is the most common etiology of dementia, that has not been previously reported in Korea. We have experienced two cases of Alzheimer's disease, one occurred in a 53-year-old man with dementia and the other occurred in a 36-year-old woman with a family history of early onset dementia. A neocortical biopsy was done to rule out the cause of dementia and showed diffusely scattered numerous senile plaques and neurofibrillary tangles in cortex of both cases. Interestingly, GFAP, an immunohistochemical stain showed strong positivity in neuritic plaques and the surrounding fibrillary gathering.

Clinical,imaging and pathological features of primary angiitis of central nervous system / 临床神经病学杂志

Objective To study the clinical, imaging and pathological features of primary angiitis of central nervous system (PACNS). Methods The clinical data of 8 patients with PACNS (4 suspected and 4 definite) were retrospectively analysed.Results There were 5 males and 3 females in 8 patients with PACNS. Average age was 32 years old and average disease course was 11 months. The initial symptoms were headache in 5, extremital weakness in 2, and speech disorder in 1 patients. The major clinical manifestations were headache in all patients, extremital weakness and numbness in 5, speech disorder in 1, and seizure disorder in 2 patients. 7 patients were with slow-progressive disease courses, and 1 patients with a relapsing-remitting one. MRI showed abnormality in all patients. 6 cases had unilateral lesions and 2 had bilateral lesions. MRI showed ill-defined, irregular or atypical wedge-shaped foci in cortical and subcortical areas in 7 patients and strip-shaped foci with surrounding finger-like cerebral edema in the left frontal and parietal lobe and gyrus cinguli in 1 patient. All lesions enhanced with different degree on contrast-enhanced scan. Enhanced lesions along sulcus and gyrus were seen in 7 patients and mass-like irregular linear enhancement lesion in 1 patient. Diffusion-weighted imaging(DWI) showed hyper-signal and apparent diffusion coefficient(ADC) showed hypo-signal in 5 patients. MRA showed stiffness and lack of smooth in one or more cerebral arteries with multiple segmental narrowing and expanding, and rarity of arterial distal branches in 8 patients. Digital subtraction angiography (DSA) also showed multiple segmental narrowing and expanding in cerebral arteries, and rarity of arterial distal branches in 4 patients. Brain biopsy in 4 cases revealed angiitis with lymphocytic infiltration. 5 patients received treatment with single corticoid, and 3 with cyclophosphamide. All cases had good outcome. Conclusions The clinical manifestations of PACNS are various. The changes of imaging are obvious, MRI, MRA and DSA are helpful in the diagnosis of PACNS. The definite diagnosis relies on brain biopsy.

CT Guided Stereotaxic Biopsy for the Differential Diagnosis of Brain Lesions

Some intracranial masses are usually treated following a presumptive diagnosis based on the clinical picture and neurological studies but in some instances a proven histological diagnosis might alter the method of treatment as well as affect the prognosis. Open surgical biopsy however might cause serious neurological deficit in many of these patients however a stereotaxic approach might avoid such problems. For the correct three dimensional target localization on CT scan we developed nearly artifact free simplified CT interface frame that accurately interface between the CT scanner and the stereotasic instrument makes it possible to take biopsies at exact and with low risk. In 10 patients CT interfaced stereotaxic biopsy of brain lesions were performed. 100% accurate histological diagnosis was obtained and helped considerably in planning further therapy. The localization error was less than 0.04mm and no significant complications occured in these patients.