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1.
World Neurosurg ; 136: 444-453, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32204296

RESUMO

BACKGROUND: Cervicomedullary compression in young children has been described in the context of Chiari type 1 malformation, with symptoms associated with the extent of tonsillar herniation below McRae line. Historically, Chiari type 1 malformation has been defined by tonsillar herniation of at least 5 mm. However, in certain populations, including very young children, Chiari symptoms may be present without this finding. A new Chiari classification is thus necessary. METHODS: Cases involving patients up to 5 years of age evaluated for possible posterior fossa decompression were retrospectively reviewed. Preoperative symptoms, magnetic resonance imaging findings, surgical management, and short- and long-term outcome and follow-up were recorded. Tonsillar descent and presence of ventral herniation (VH) were recorded. We define VH as the tonsils crossing a line that bisects the caudal medulla at the level of the foramen magnum, thus creating a novel entity, Chiari type 0.5 malformation. Patients with ventrally herniated tonsils were compared with patients exhibiting more typical Chiari morphology. RESULTS: Of 41 cases retrospectively reviewed, 20 met criteria for VH. These differed from cases without VH because of the predominance of medullary symptoms. In the VH cohort, 11 patients underwent surgical decompression with symptom resolution; 9 were initially managed conservatively, but 3 subsequently required surgery. CONCLUSIONS: We define a novel Chiari entity, Chiari type 0.5 malformation, characterized by ventral tonsillar wrapping around the medulla in young children in the absence of classic Chiari type 1 malformation imaging findings. These patients are more likely to present with medullary symptoms than patients without VH. They are also more likely to require surgical decompression and respond favorably to intervention.


Assuntos
Malformação de Arnold-Chiari/diagnóstico , Malformação de Arnold-Chiari/patologia , Tonsila Palatina/patologia , Malformação de Arnold-Chiari/cirurgia , Pré-Escolar , Tratamento Conservador , Fossa Craniana Posterior/cirurgia , Descompressão Cirúrgica/métodos , Encefalocele/diagnóstico por imagem , Encefalocele/cirurgia , Feminino , Seguimentos , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Bulbo/patologia , Bulbo/cirurgia , Tonsila Palatina/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
2.
BMC Neurol ; 19(1): 341, 2019 Dec 27.
Artigo em Inglês | MEDLINE | ID: mdl-31881866

RESUMO

BACKGROUND: To evaluate the efficacy of microvascular decompression (MVD) in reducing hypertension (HTN) in hypertensive patients with trigeminal neuralgia (TN). METHODS: The clinical data of 58 cases of neurogenic HTN with TN treated in our hospital were retrospectively reviewed. Preoperative MR revealed abnormal blood pressure in the left rostral ventrolateral medulla (RVLM) and the posterior cranial nerve root entry zone (REZ). The patients were divided into control group: only trigeminal nerve was treated with MVD; experimental group: trigeminal nerve, RVLM and REZ were treated with MVD at the same time. The patients were followed up for 6 months to 1 year to observe the changes of blood pressure. RESULTS: There was no significant difference in gender, age, course of TN, course of HTN, grade of HTN and preoperative blood pressure between the two groups. After operation, the effective rate of HTN improvement with MVD was 32.1% in the control group. There was no significant difference in the preoperative and post operative blood pressure. (P△SBP = 0.131; P△BDP = 0.078). In the experimental group, the effective rate was 83.3%. The postoperative blood pressure was significantly lower than preoperative values. (P△SBP < 0.001; P△DBP < 0.001). CONCLUSIONS: MVD is an effective treatment for neurogenic HTN. However, the criteria for selecting hypertensive patients who need MVD to control their HTN still needs to be further determined. Possible indications may include: left trigeminal neuralgia, neurogenic HTN; abnormal blood pressure compression in the left RVLM and REZ areas on MR; and blood pressure in these patients can not be effectively controlled by drugs.


Assuntos
Hipertensão/etiologia , Hipertensão/cirurgia , Bulbo/cirurgia , Cirurgia de Descompressão Microvascular/métodos , Doenças Vasculares/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Bulbo/patologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Neuralgia do Trigêmeo/etiologia , Neuralgia do Trigêmeo/cirurgia , Doenças Vasculares/complicações
3.
Muscle Nerve ; 60(6): 762-768, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31498901

RESUMO

INTRODUCTION: Equine recurrent laryngeal neuropathy (RLN) is a naturally occurring model of length-dependent axonopathy characterized by asymmetrical degeneration of recurrent laryngeal nerve axons (RLn). Distal RLn degeneration is marked, but it is unclear whether degeneration extends to include cell bodies (consistent with a neuronopathy). METHODS: With examiners blinded to RLN severity, brainstem location, and side, we examined correlations between RLN severity (assessed using left distal RLn myelinated axon count) and histopathological features (including chromatolysis and glial responses) in the nucleus ambiguus cell bodies, and myelinated axon count of the right distal RLn of 16 horses. RESULTS: RLN severity was not associated with RLn cell body number (P > .05), or degeneration. A positive correlation between the left and right distal RLn myelinated axon counts was identified (R2 = 0.57, P < .05). DISCUSSION: We confirm that RLN, a length-dependent distal axonopathy, occurs in the absence of detectable neuronopathy.


Assuntos
Corpo Celular/patologia , Bulbo/patologia , Fibras Nervosas Mielinizadas/patologia , Neurônios/patologia , Nervo Laríngeo Recorrente/patologia , Paralisia das Pregas Vocais/patologia , Animais , Atrofia , Contagem de Células , Cavalos , Nervo Laríngeo Recorrente/fisiopatologia , Paralisia das Pregas Vocais/fisiopatologia
5.
World Neurosurg ; 129: 200-201, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31426252

RESUMO

Only few case reports of intradural extramedullary-located cavernous angiomas are available. This report describes an extra-axial hemorrhagic lesion located on the lateral surface of the medulla oblongata at the foramen magnum. Follow-up magnetic resonance imaing clearly demonstrated growth of the lesion, probably due to repetitive intralesional hemorrhaging. Initially, a right vertebral artery aneurysm or an arteriovenous malformation was suspected. Digital subtraction angiography excluded these differential diagnoses, although doubt remained concerning the possibility of a thrombosed aneurysm. Preoperatively, the lesion did not had the typical macroscopical aspect of a cavernoma and appeared rather as an expansive encapsulated multicystic vascular lesion. Nevertheless, pathologic analysis confirmed the diagnosis of a cavernous hemangioma. Complete microsurgical resection was obtained without neurologic impairment.


Assuntos
Neoplasias do Tronco Encefálico/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Bulbo/cirurgia , Neoplasias do Tronco Encefálico/patologia , Feminino , Forame Magno/patologia , Forame Magno/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/patologia , Humanos , Bulbo/patologia , Pessoa de Meia-Idade
6.
Artigo em Alemão | MEDLINE | ID: mdl-31434127

RESUMO

We describe the magnetic resonance imaging (MRI) examination in a dog with confirmed suid herpesvirus 1 (SHV-1) infection and compare the findings to the results of the histopathologic examination. A 5-year-old female German Hunting Terrier used for hunting displayed severe pruritus and fever 7 days after contact with a wild boar. Two days after the onset of the first disease symptoms, the dog was presented with seizures and hyperthermia. MRI examination revealed hyperintense alterations in the occipital, temporal and parietal lobe areas. In the contrast sequences, contrast enhancement of the medulla oblongata as well as of the pachy- and leptomeninges within the occipital lobe and the cerebellum could be detected. The bitch was euthanized because of the acute deterioration of its condition. Histopathologically, multifocal mild to moderate mixed cellular vasculitis and satellitosis were found in the brain stem and pons, where SHV-1 antigen was detectable immunohistochemically in neurons and glial cells. In molecular-biological studies of the trigeminal ganglion and the medulla oblongata, SHV-1-specific DNA was detected. The MRI lesions of our patient displayed marked differences to the changes described in the literature for central European tick-borne meningoencephalomyelitis or the paralytic course of rabies. By contrast, it appears that similarities to the lesions described in canine distemper and the encephalitic form of rabies did exist.


Assuntos
Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Doenças do Cão/diagnóstico por imagem , Pseudorraiva/diagnóstico por imagem , Animais , Antígenos Virais/isolamento & purificação , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/patologia , Cerebelo/diagnóstico por imagem , Cerebelo/patologia , Cérebro/diagnóstico por imagem , Cérebro/patologia , Doenças do Cão/patologia , Doenças do Cão/virologia , Cães , Evolução Fatal , Feminino , Febre/veterinária , Herpesvirus Suídeo 1/imunologia , Herpesvirus Suídeo 1/isolamento & purificação , Imuno-Histoquímica/veterinária , Imagem por Ressonância Magnética/veterinária , Bulbo/diagnóstico por imagem , Bulbo/patologia , Meninges/diagnóstico por imagem , Meninges/patologia , Pseudorraiva/patologia , Pseudorraiva/virologia , Convulsões/veterinária , Sus scrofa
7.
World Neurosurg ; 132: 69-74, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31470167

RESUMO

BACKGROUND: Immunoglobulin (Ig)G4-related disease (IgG4-RD) was defined only recently and can be found in many organs. As intracranial lesions, hypophysitis and pachymeningitis are well known, whereas intracranial pseudotumor is unusual. This case involved multiple intracranial pseudotumors without extracranial lesions, mimicking multiple meningioma. CASE DESCRIPTION: A 72-year-old woman was referred to our hospital with an incidental mass lesion at the craniocervical junction on magnetic resonance imaging (MRI). MRI showed diffuse enhanced extra-axial nodules around the medulla and middle cranial fossa. Surgery was performed for the gradually enlarging tumor. Intraoperative findings showed hard nodules around the vertebral artery. We performed subtotal resection. Neuropathological findings showed diffuse lymphoplasmacytic infiltration with lymphoid follicles. Immunohistochemical studies for IgG4 and IgG showed the histological criteria for IgG4-RD were met. Given the high IgG4 serum level, we diagnosed IgG4-RD. Steroid was administered initially but was then tapered, and nodules have since remained small. CONCLUSIONS: This case demonstrates IgG4-RD mimicking meningioma with multiple masses but without extracranial lesions. This case and previous descriptions suggest the possibility of pseudotumor due to IgG4-RD in the presence of multiple extra-axial lesions at the dura and supplying artery, hard nodules with thickening of the artery, and frozen section findings of lymphoplasmacytes surrounded by rich collagen fibers. In such cases, the pseudotumor due to IgG4-RD should be resected, and total resection might not be warranted.


Assuntos
Doença Relacionada a Imunoglobulina G4/patologia , Bulbo/patologia , Idoso , Fossa Craniana Média/diagnóstico por imagem , Fossa Craniana Média/patologia , Diagnóstico Diferencial , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico por imagem , Doença Relacionada a Imunoglobulina G4/cirurgia , Bulbo/diagnóstico por imagem , Bulbo/cirurgia , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico
8.
Anal Cell Pathol (Amst) ; 2019: 8243813, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31281769

RESUMO

Background/Aims: The aim of this study is to examine the protective effect of the cholinergic anti-inflammatory pathway (CAP) in experimental esophagitis in rats. Methods: A total of 40 male Sprague-Dawley (SD) rats were randomly divided into five groups as follows: control group, sham + saline group, sham + acid group, operation + saline group, and operation + acid group. Two weeks after the dorsal motor nucleus of the vagus (DMV) destruction, hydrochloric acid with pepsin was perfused into the lower part of the esophagus for 90 min. The rats were sacrificed 60 min after perfusion. The esophagus was prepared for hematoxylin and eosin (HE) staining, and the degree of inflammation and NF-κB activation in the esophagus was measured. Inflammatory cytokines (TNF-α, IL-6, IL-1ß, and PGE2) in the esophagus were measured by ELISA. The brain was removed and processed for c-fos immunohistochemistry staining. The c-fos-positive neurons were counted and analyzed. Results: The TNF-α, IL-1ß, IL-6, and PGE2 concentrations in the esophageal tissue increased after acid perfusion. The microscopic esophagitis scores and the activation of NF-κB p65 in the esophagus were significantly higher in the operation + acid group than in the operation + saline group. c-fos-positive neurons significantly increased in rats receiving acid perfusion in the amygdala (AM), the paraventricular nucleus of the hypothalamus (PVN), the parabrachial nucleus (PBN), the nucleus of the solitary tract (NTS)/DMV, the nucleus ambiguous (NA), the reticular nucleus of the medulla (RNM), and the area postrema (AP). After DMV destruction, c-fos expression was reduced in the AM, PVN, PBN, NTS/DMV, NA, RNM, and AP, especially in the AM, PVN, NTS/DMV, RNM, and AP. Conclusions: The DMV is an important nucleus of the CAP. The DMV lesion can aggravate esophageal inflammation and injury from acid-induced acute esophagitis in a rat model. The CAP has a protective effect on the acute esophagitis rat model and could be a new therapy for reflux esophagitis (RE).


Assuntos
Esofagite Péptica/induzido quimicamente , Bulbo/patologia , Nervo Vago/patologia , Ácidos/administração & dosagem , Animais , Dinoprostona/metabolismo , Modelos Animais de Doenças , Esofagite Péptica/patologia , Esofagite Péptica/fisiopatologia , Imuno-Histoquímica , Inflamação/induzido quimicamente , Interleucina-1beta/metabolismo , Interleucina-6/metabolismo , Masculino , Bulbo/metabolismo , Neuroimunomodulação/efeitos dos fármacos , Perfusão , Proteínas Proto-Oncogênicas c-fos/metabolismo , Ratos , Ratos Sprague-Dawley , Fator de Transcrição RelA/metabolismo , Nervo Vago/metabolismo
9.
World Neurosurg ; 130: 400-404, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31326640

RESUMO

BACKGROUND: Intracranial solitary fibrous tumor (SFT) is a rare occurrence and involvement of the fourth ventricle rarely reported. Because of its rarity, some characteristics of intracranial SFT seem to still remain uncertain. CASE DESCRIPTION: This study describes a very rare case of intracranial SFT in a 55-year-old woman who presented with gait disturbance and numbness in bilateral upper limbs from 3 months before visiting the hospital. Head magnetic resonance imaging scan revealed a homogeneously enhancing mass lesion located primarily in the fourth ventricle extending into the spinal canal and left foramen of Luschka, with a maximum diameter of 60 mm. Notably, this tumor presented spontaneous partial regression during waiting planned surgery without therapy, including chemotherapy and radiotherapy. This patient underwent a midline suboccipital craniotomy and resection of the tumor. Interestingly, there was no attachment to the dura mater of the posterior cranial fossa and the lesion was only attached to the dorsal part of the medulla oblongata. CONCLUSIONS: Although the location of the SFT in the fourth ventricle is rare, SFT should be considered as 1 of the differential diagnosis of fourth ventricle tumors. In addition, this case indicates that SFT in the fourth ventricle may regress on occasion spontaneously without a precisely known cause for this spontaneous partial regression.


Assuntos
Neoplasias do Tronco Encefálico/patologia , Bulbo/patologia , Tumores Fibrosos Solitários/patologia , Neoplasias do Tronco Encefálico/cirurgia , Craniotomia/métodos , Feminino , Humanos , Imagem por Ressonância Magnética , Bulbo/cirurgia , Pessoa de Meia-Idade , Neoplasia Residual/cirurgia , Doenças Raras , Remissão Espontânea , Tumores Fibrosos Solitários/cirurgia
10.
World Neurosurg ; 130: 154-156, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31301440

RESUMO

BACKGROUND: Intracranial de novo arachnoid cysts in adults are rare, suggesting the involvement of head trauma and inflammatory diseases. We report a symptomatic adult case of nontraumatic de novo arachnoid cyst on the ventral medulla oblongata. CASE DESCRIPTION: A 56-year-old man came to our hospital complaining of dysphagia and writing difficult since 3 months ago. There was no history of head injury or inflammatory disease. A 25-mm cystic lesion was found on the ventral side of the medulla oblongata on brain magnetic resonance imaging, and the lower cranial nerve and medulla oblongata were highly compressed. The lesion did not exist on magnetic resonance imaging performed 9 years ago. Capsular resection was performed, and the histologic diagnosis was a typical arachnoid cyst. After the operation, all neurologic symptoms disappeared and no recurrence has been observed after 6 months. CONCLUSIONS: The pathophysiology of nontraumatic de novo arachnoid cysts has many unknown features, and it appears necessary to accumulate further case reports.


Assuntos
Cistos Aracnóideos/patologia , Cistos Aracnóideos/cirurgia , Traumatismos Craniocerebrais/cirurgia , Bulbo/cirurgia , Recidiva Local de Neoplasia/cirurgia , Traumatismos Craniocerebrais/diagnóstico , Traumatismos Craniocerebrais/patologia , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Bulbo/patologia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Tomografia Computadorizada por Raios X/métodos
11.
Radiographics ; 39(4): 1110-1125, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31283463

RESUMO

The anatomy of the brainstem is complex. It contains numerous cranial nerve nuclei and is traversed by multiple tracts between the brain and spinal cord. Improved MRI resolution now allows the radiologist to identify a higher level of anatomic detail, but an understanding of functional anatomy is crucial for correct interpretation of disease. Brainstem syndromes are most commonly due to occlusion of the posterior circulation or mass effect from intrinsic space-occupying lesions. These syndromes can have subtle imaging findings that may be missed by a radiologist unfamiliar with the anatomy or typical manifesting features. This article presents the developmental anatomy of the brainstem and discusses associated pathologic syndromes. Congenital and acquired syndromes are described and correlated with anatomic locations at imaging, with diagrams to provide a reference to aid in radiologic interpretation. ©RSNA, 2019.


Assuntos
Imagem por Ressonância Magnética/métodos , Bulbo/diagnóstico por imagem , Mesencéfalo/diagnóstico por imagem , Neuroimagem/métodos , Ponte/diagnóstico por imagem , Encefalopatias/diagnóstico por imagem , Infarto Encefálico/diagnóstico por imagem , Anormalidades Craniofaciais/diagnóstico por imagem , Humanos , Bulbo/anatomia & histologia , Bulbo/patologia , Mesencéfalo/anatomia & histologia , Mesencéfalo/patologia , Ponte/anatomia & histologia , Ponte/patologia , Síndrome
12.
Forensic Sci Int ; 301: e49-e54, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31230858

RESUMO

Gunshot wounds (GSW) are one of the most common causes of penetrating spinal injury, however few data are available regarding GSW causing an indirect fatal nervous tissue injury, such as that induced by the concussive force secondary to the bullet penetration. This report describes a rare case of a death following a GSW spine injury at the level of C2 vertebral body, without direct contact with the spinal cord, as seen with computed tomography scan performed soon after the death. At autopsy, vertebral canal and dura mater, as well as spinal cord and medulla oblongata, appeared devoid of pathologies and/or lesions, major viscera were unaltered. The cause of death was attributed to a cardiorespiratory arrest subsequent to the GSW injury of the C2 vertebral bone. Histopathological analysis of spinal cord and medulla oblongata was performed by means of conventional stainings, and glial fibrillary acidic protein (GFAP) and Neurofilaments 200kD (NF) immunohistochemistry. Histological alterations stood out against a tissue with no other evident sign of neuropathology, and could be observed from the caudalmost part of the medulla oblongata to the level of the inferior olivary nucleus. Main structural changes were found in the white matter, involving often the adjacent gray matter, where they appeared as multiple scattered areas of degeneration, lacking the usual staining affinity, and showing a disrupted fibrillary pattern as evidenced by myelin staining, and GFAP- and NF-immunolabelling. The shock wave secondary to the impact on the C2 vertebral bone is likely to have been the cause of a widespread neuronal-axonal histopathological damage at the spinal-medullary junction and caudal medulla oblongata that is compatible with a severe fatal respiratory dysfunction and dysregulation of the autonomic pathways subserving the control of blood pressure and cardiac activity.


Assuntos
Vértebras Cervicais/lesões , Vértebras Cervicais/patologia , Morte Súbita/etiologia , Bulbo/patologia , Ferimentos por Arma de Fogo/patologia , Axônios/patologia , Vértebras Cervicais/diagnóstico por imagem , Patologia Legal , Parada Cardíaca/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Insuficiência Respiratória/etiologia , Tomografia Computadorizada por Raios X , Substância Branca/patologia , Ferimentos por Arma de Fogo/diagnóstico por imagem
13.
Neurotoxicology ; 74: 40-46, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31121239

RESUMO

Cigarette smoke exposure during pregnancy into infancy affects brain growth and development in both short and long term (into adulthood). Using a mouse model of pre- into post- natal cigarette smoke exposure (SE), we aimed to determine the effects on brain derived neurotrophic factor (BDNF) and its receptor TrkB, neuropeptide pituitary adenylate cyclase activating polypeptide (PACAP) and its receptor PAC1, and astrocyte (GFAP) and microglia (Iba-1) immunohistochemical expression, in seven nuclei of the medulla and the facial (FAC) nucleus of the pons. Male pups of dams exposed to two cigarettes (nicotine <1.2 mg, CO <15 mg) twice daily for six weeks prior to mating, during gestation and lactation (n = 5; SE), were compared to pups exposed to air under the same condition (n = 5; SHAM) at postnatal day 20. Expression changes were only evident for BDNF, TrkB and PAC1 and included decreased BDNF in the hypoglossal (XII) nucleus and nucleus of the solitary tract (NTS), increased TrkB in XII but decreased TrkB in the FAC, and increased PAC1 in 4 nuclei of the medulla including the NTS. These results suggest that the effect of SE on the brainstem are region and marker selective, affecting regions of respiratory control (XII and NTS), and restricted to the BDNF system and PAC1, with no effect on activation states of astrocytes or microglia.


Assuntos
Tronco Encefálico/efeitos dos fármacos , Tronco Encefálico/metabolismo , Fator Neurotrófico Derivado do Encéfalo/biossíntese , Gliose/induzido quimicamente , Gliose/patologia , Microglia/efeitos dos fármacos , Polipeptídeo Hipofisário Ativador de Adenilato Ciclase/biossíntese , Efeitos Tardios da Exposição Pré-Natal/patologia , Poluição por Fumaça de Tabaco/efeitos adversos , Animais , Tronco Encefálico/patologia , Feminino , Imuno-Histoquímica , Masculino , Bulbo/efeitos dos fármacos , Bulbo/metabolismo , Bulbo/patologia , Glicoproteínas de Membrana/biossíntese , Camundongos , Nicotina/toxicidade , Agonistas Nicotínicos/toxicidade , Gravidez , Proteínas Tirosina Quinases/biossíntese , Produtos do Tabaco
14.
J Neuroimmunol ; 332: 176-186, 2019 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-31075641

RESUMO

The myelin sheath is critical in maintaining normal functions of the adult central nervous system (CNS) and the loss of the myelin sheath results in various neurological diseases. Although remyelination is the intrinsic repair system against demyelination that new myelin sheath is formed around axons in the adult CNS, little has been reported on remyelination system in the medulla oblongata. In the present study, we showed that the proliferation of oligodendrocyte progenitor cells (OPCs) was increased in the medulla oblongata by lysophosphatidylcholine (LPC)-induced focal demyelination, but that of NSCs was not changed. The inhibition of vascular endothelial growth factor (VEGF)- and platelet-derived growth factor (PDGF)-signaling suppressed the proliferation of OPCs by LPC-induced demyelination. Thus, the present study indicates that resident OPCs contribute to focal remyelination and VEGF and PDGF signaling is required for the proliferation of OPCs in the medulla oblongata of the adult mouse.


Assuntos
Doenças Desmielinizantes/patologia , Bulbo/patologia , Células Precursoras de Oligodendrócitos/patologia , Fator de Crescimento Derivado de Plaquetas/fisiologia , Remielinização/fisiologia , Fator A de Crescimento do Endotélio Vascular/fisiologia , Animais , Divisão Celular/efeitos dos fármacos , Doenças Desmielinizantes/induzido quimicamente , Mesilato de Imatinib/farmacologia , Ventrículos Laterais/patologia , Lipopolissacarídeos/toxicidade , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Bainha de Mielina/fisiologia , Células-Tronco Neurais/efeitos dos fármacos , Células-Tronco Neurais/patologia , Quinazolinas/farmacologia , Receptores do Fator de Crescimento Derivado de Plaquetas/antagonistas & inibidores , Receptores de Fatores de Crescimento do Endotélio Vascular/antagonistas & inibidores , Organismos Livres de Patógenos Específicos
16.
J Neurol Neurosurg Psychiatry ; 90(8): 861-869, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30992335

RESUMO

OBJECTIVE: A hallmark of amyotrophic lateral sclerosis (ALS) caused by mutations in superoxide dismutase-1 (SOD1) are inclusions containing SOD1 in motor neurons. Here, we searched for SOD1-positive inclusions in 29 patients carrying ALS-linked mutations in six other genes. METHODS: A panel of antibodies that specifically recognise misfolded SOD1 species were used for immunohistochemical investigations of autopsy tissue. RESULTS: The 18 patients with hexanucleotide-repeat-expansions in C9orf72 had inclusions of misfolded wild type (WT) SOD1WT in spinal motor neurons. Similar inclusions were occasionally observed in medulla oblongata and in the motor cortex and frontal lobe. Patients with mutations in FUS, KIF5A, NEK1, ALSIN or VAPB, carried similar SOD1WT inclusions. Minute amounts of misSOD1WT inclusions were detected in 2 of 20 patients deceased from non-neurological causes and in 4 of 10 patients with other neurodegenerative diseases. Comparison was made with 17 patients with 9 different SOD1 mutations. Morphologically, the inclusions in patients with mutations in C9orf72HRE, FUS, KIF5A, NEK1, VAPB and ALSIN resembled inclusions in patients carrying the wildtype-like SOD1D90A mutation, whereas patients carrying unstable SOD1 mutations (A4V, V5M, D76Y, D83G, D101G, G114A, G127X, L144F) had larger skein-like SOD1-positive inclusions. CONCLUSIONS AND RELEVANCE: Abundant inclusions containing misfolded SOD1WT are found in spinal and cortical motor neurons in patients carrying mutations in six ALS-causing genes other than SOD1. This suggests that misfolding of SOD1WT can be part of a common downstream event that may be pathogenic. The new anti-SOD1 therapeutics in development may have applications for a broader range of patients.


Assuntos
Esclerose Amiotrófica Lateral/genética , Demência Frontotemporal/genética , Mutação/genética , Deficiências na Proteostase/genética , Superóxido Dismutase-1/genética , Adulto , Idoso , Esclerose Amiotrófica Lateral/patologia , Feminino , Demência Frontotemporal/patologia , Genes/genética , Humanos , Corpos de Inclusão/metabolismo , Masculino , Bulbo/metabolismo , Bulbo/patologia , Pessoa de Meia-Idade , Córtex Motor/metabolismo , Córtex Motor/patologia , Neurônios Motores/metabolismo , Neurônios Motores/patologia
17.
Am J Physiol Endocrinol Metab ; 317(2): E388-E398, 2019 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31013147

RESUMO

The impaired ability of the autonomic nervous system to respond to hypoglycemia is termed "hypoglycemia-associated autonomic failure" (HAAF). This life-threatening phenomenon results from at least two recent episodes of hypoglycemia, but the pathology underpinning HAAF remains largely unknown. Although naloxone appears to improve hypoglycemia counterregulation under controlled conditions, hypoglycemia prevention remains the current mainstay therapy for HAAF. Epinephrine-synthesizing neurons in the rostroventrolateral (C1) and dorsomedial (C3) medulla project to the subset of sympathetic preganglionic neurons that regulate peripheral epinephrine release. Here we determined whether or not C1 and C3 neuronal activation is impaired in HAAF and whether or not 1 wk of hypoglycemia prevention or treatment with naloxone could restore C1 and C3 neuronal activation and improve HAAF. Twenty male Sprague-Dawley rats (250-300 g) were used. Plasma epinephrine levels were significantly increased after a single episode of hypoglycemia (n = 4; 5,438 ± 783 pg/ml vs. control 193 ± 27 pg/ml, P < 0.05). Repeated hypoglycemia significantly reduced the plasma epinephrine response to subsequent hypoglycemia (n = 4; 2,179 ± 220 pg/ml vs. 5,438 ± 783 pg/ml, P < 0.05). Activation of medullary C1 (n = 4; 50 ± 5% vs. control 3 ± 1%, P < 0.05) and C3 (n = 4; 45 ± 5% vs. control 4 ± 1%, P < 0.05) neurons was significantly increased after a single episode of hypoglycemia. Activation of C1 (n = 4; 12 ± 3%, P < 0.05) and C3 (n = 4; 19 ± 5%, P < 0.05) neurons was significantly reduced in the HAAF groups. Hypoglycemia prevention or treatment with naloxone did not restore the plasma epinephrine response or C1 and C3 neuronal activation. Thus repeated hypoglycemia reduced the activation of C1 and C3 neurons mediating adrenal medullary responses to subsequent bouts of hypoglycemia.


Assuntos
Glucose/farmacologia , Hipoglicemia/complicações , Hipoglicemia/fisiopatologia , Bulbo/efeitos dos fármacos , Neurônios/efeitos dos fármacos , Animais , Sistema Nervoso Autônomo/efeitos dos fármacos , Sistema Nervoso Autônomo/metabolismo , Sistema Nervoso Autônomo/fisiopatologia , Doenças do Sistema Nervoso Autônomo/sangue , Doenças do Sistema Nervoso Autônomo/etiologia , Doenças do Sistema Nervoso Autônomo/patologia , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Glicemia/metabolismo , Encéfalo/efeitos dos fármacos , Encéfalo/metabolismo , Encéfalo/patologia , Modelos Animais de Doenças , Hipoglicemia/sangue , Hipoglicemia/patologia , Insulina/sangue , Masculino , Bulbo/patologia , Bulbo/fisiopatologia , Neurônios/fisiologia , Ratos , Ratos Sprague-Dawley , Recidiva
18.
Mult Scler Relat Disord ; 31: 72-73, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30947064

RESUMO

A 17-year-old girl with relapsing-remitting multiple sclerosis (MS) was referred for a 2-year history of visual blurriness. Her bedside examination was remarkable for gait unsteadiness only. Optical coherence tomography was performed as part of her workup. Unexpectedly, Spectralis© video imaging revealed left torsional nystagmus that was not apparent on bedside examination. Review of previous brain magnetic resonance images (MRI) revealed left ocular deviation, as well as a left dorsolateral medullary MS plaque, which was the cause of her torsional nystagmus. We highlight how Spectralis© scanning confocal laser ophthalmoscopy allows video imaging that can capture torsional ocular movements.


Assuntos
Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Nistagmo Patológico/diagnóstico por imagem , Adolescente , Feminino , Humanos , Bulbo/diagnóstico por imagem , Bulbo/patologia , Esclerose Múltipla Recidivante-Remitente/complicações , Esclerose Múltipla Recidivante-Remitente/patologia , Nistagmo Patológico/etiologia , Nistagmo Patológico/patologia , Tomografia de Coerência Óptica
19.
J Nippon Med Sch ; 86(1): 48-54, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30918157

RESUMO

Intracranial dural arteriovenous fistula (DAVF) with perimedullary venous drainage may cause brainstem swelling and represent a diagnostic challenge. A 66-year-old man presented to the emergency room with recurrent vertigo, minimal truncal ataxia with a wide-based gait, and a slightly impaired tandem gait. Brain magnetic resonance imaging (MRI) revealed a hyperintense lesion in the left pontomedullary area on T2-weighted images (T2WIs) with partial gadolinium enhancement, but without increased signals on diffusion-weighted images. Abnormal serpentine flow void vessels surrounding the medulla and upper cervical spinal cord were initially overlooked but discovered later. An angiogram revealed DAVF with feeders from the right occipital artery and the meningeal branch of the right distal vertebral artery with drainage into the anterior medullary venous system and the perimedullary veins. The patient underwent a successful transarterial endovascular embolization and improved gradually. A brain MRI at 3-month follow-up revealed a residual hyperintense signal on the T2WIs in the left lower medulla. Six cases of patients exhibiting DAVF with isolated unilateral brainstem swelling from the literature were reviewed. Isolated unilateral brainstem swelling due to intracranial DAVF with perimedullary venous drainage is extremely rare and might mimic a tumor on MRI. Abnormal serpentine flow void vessels on the surface of the brainstem or spinal cord are crucial diagnostic clues.


Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Tronco Encefálico/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Diagnóstico Diferencial , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Bulbo/diagnóstico por imagem , Idoso , Tronco Encefálico/irrigação sanguínea , Tronco Encefálico/patologia , Vértebras Cervicais , Imagem de Difusão por Ressonância Magnética , Embolização Terapêutica , Procedimentos Endovasculares/métodos , Seguimentos , Humanos , Malformações Arteriovenosas Intracranianas/patologia , Malformações Arteriovenosas Intracranianas/cirurgia , Angiografia por Ressonância Magnética , Masculino , Bulbo/irrigação sanguínea , Bulbo/patologia , Medula Espinal/irrigação sanguínea , Medula Espinal/diagnóstico por imagem , Medula Espinal/patologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
20.
J Int Med Res ; 47(5): 2256-2261, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30880528

RESUMO

Varicella zoster virus (VZV) can invade the brainstem or brain via the glossopharyngeal, vagus , or facial nerve, resulting in brainstem inflammation or encephalitis. We report the case of a 66-year-old male patient with a primary manifestation of medulla injury of the glossopharyngeal and vagus nerves, combined with a medulla lesion, who was misdiagnosed with lateral medullary syndrome. Facial nerve injury and earache subsequently occurred and human herpes virus 3 (VZV) was detected by second-generation sequencing of the cerebrospinal fluid. The final diagnosis was varicella zoster encephalitis, which improved after antiviral therapy.


Assuntos
Encefalite por Varicela Zoster/complicações , Traumatismos do Nervo Glossofaríngeo/complicações , Bulbo/patologia , Traumatismos do Nervo Vago/complicações , Idoso , Imagem de Difusão por Ressonância Magnética , Encefalite por Varicela Zoster/diagnóstico por imagem , Traumatismos do Nervo Glossofaríngeo/diagnóstico por imagem , Humanos , Masculino , Bulbo/diagnóstico por imagem , Traumatismos do Nervo Vago/diagnóstico por imagem
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