Neuromyelitis optica with clinical and histopathological involvement of the brain

2007 ◽  
Vol 13 (5) ◽  
pp. 679-682 ◽  
Author(s):  
G.J.D. Hengstman ◽  
P. Wesseling ◽  
C.W.G.M. Frenken ◽  
P.J.H. Jongen

Diagnostic criteria for neuromyelitis optica (NMO) state that there should be no active disease outside the optic nerves and spinal cord. However, several cases have been described with symptomatic brain involvement. We describe an autopsy case of a patient with NMO and symptomatic involvement of the brain. The histopathology of the brain lesions is typical for NMO, with extensive macrophage infiltration, including perivascular accumulation of large numbers of eosinophils. This is the first case that clearly shows that in NMO, the histopathology of the brain lesions is identical to that of the lesions in the optic nerves and spinal cord. Multiple Sclerosis 2007; 13: 679-682. http://msj.sagepub.com

Physiology ◽  
2019 ◽  
Vol 34 (3) ◽  
pp. 216-229 ◽  
Author(s):  
Joline E. Brandenburg ◽  
Matthew J. Fogarty ◽  
Gary C. Sieck

Spastic cerebral palsy (CP), despite the name, is not consistently identifiable by specific brain lesions. CP animal models focus on risk factors for development of CP, yet few reproduce the diagnostic symptoms. Animal models of CP must advance beyond risk factors to etiologies, including both the brain and spinal cord.


2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Woojun Kim ◽  
Su-Hyun Kim ◽  
So-Young Huh ◽  
Ho Jin Kim

Neuromyelitis optica (NMO) is an idiopathic inflammatory syndrome of the central nervous system that is characterized by severe attacks of optic neuritis (ON) and myelitis. Until recently, NMO was considered a disease without brain involvement. However, since the discovery of NMO-IgG/antiaqaporin-4 antibody, the concept of NMO was broadened to NMO spectrum disorder (NMOSD), and brain lesions are commonly recognized. Furthermore, some patients present with brain symptoms as their first manifestation and develop recurrent brain symptoms without ON or myelitis. Brain lesions with characteristic locations and configurations can be helpful in the diagnosis of NMOSD. Due to the growing recognition of brain abnormalities in NMOSD, these have been included in the NMO and NMOSD diagnostic criteria or guidelines. Recent technical developments such as diffusion tensor imaging, MR spectroscopy, and voxel-based morphometry reveal new findings related to brain abnormalities in NMOSD that were not identified using conventional MRI. This paper focuses on the incidence and characteristics of the brain lesions found in NMOSD and the symptoms that they cause. Recent studies using advanced imaging techniques are also introduced.


Neurosurgery ◽  
2016 ◽  
Vol 80 (2) ◽  
pp. E178-E184 ◽  
Author(s):  
Azam Basheer ◽  
Richard Rammo ◽  
Steven Kalkanis ◽  
Michelle M. Felicella ◽  
Mokbel Chedid

Abstract BACKGROUND AND IMPORTANCE: Pilocytic astrocytoma (PA) is among the most common of the central nervous system gliomas in the pediatric population; however, it is uncommon in adults. PAs of the spinal cord in adults are even rarer, with only a few cases found in the literature. We report here the first case in the literature of multifocal intradural extramedullary spinal cord PAs in an adult. CLINICAL PRESENTATION: Our patient is a 56-yr-old female who presented with loss of balance and an ataxic gait. Multiple extramedullary spinal cord tumors were identified intraoperatively, the lesions completely resected, and all diagnosed as PAs. CONCLUSION: This case illustrates a unique instance of multifocal intradural extramedullary spinal cord PAs in an adult with no previous history of PA during childhood, no known familial syndromes, and no brain involvement.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Felix Schmidt ◽  
Önder Göktas ◽  
Sven Jarius ◽  
Brigitte Wildemann ◽  
Klemens Ruprecht ◽  
...  

Background. Neuromyelitis optica (NMO) is a severely disabling autoimmune disorder of the CNS, which mainly affects the optic nerves and spinal cord. However, recent studies have shown that extra-opticospinal are more common in NMO than previously thought.Objective. To investigate olfactory function (OF) in patients with neuromyelitis optica (NMO) versus healthy controls (HC).Methods. Psychophysical testing of the orthonasal OF was performed using theThreshold-Discrimination-Identificationtest (TDI), measuring different qualities of olfaction, in 10 unselected NMO patients and 10 HC.Results. Five of 10 NMO patients (50%) showed hyposmia, while all 10 HC were normosmic. Moreover, NMO patients had significantly lower meanTDI-scores compared to HC, based on a poorer performance in both theDiscriminationand theIdentificationsubtests.Conclusions. Our results suggest that hyposmia might be part of the expanding clinical spectrum of NMO.


2013 ◽  
Vol 71 (7) ◽  
pp. 487-489 ◽  
Author(s):  
Eliasz Engelhardt ◽  
Marleide da Mota Gomes

The concept of shock apparently emerged in the middle of the 18th century (Whyett) as an occurrence observed experimentally after spinal cord transection, and identified as "shock" phenomenon one century later (Hall). The concept was extended (Brown-Séquard) and it was suggested that brain lesions caused functional rupture in regions distant from the injured one ("action à distance"). The term "diaschisis" (von Monakow), proposed as a new modality of shock, had its concept broadened, underpinned by observations of patients, aiming at distinguishing between symptoms of focal brain lesions and transitory effects they produced, attributable to depression of distant parts of the brain connected to the injured area. Presently, diaschisis is related mainly to cerebrovascular lesions and classified according to the connection fibers involved, as proposed by von Monakow. Depression of metabolism and blood flow in regions anatomically separated, but related by connections with the lesion, allows observing diaschisis with neuroimaging.


2020 ◽  
pp. 6100-6109
Author(s):  
Hadi Manji

Invasion of the central nervous system occurs early in the course of syphilis infection. Neurosyphilis causes a meningitis, a myeloradiculopathy due to pachymeningitis, gummatous (granulomatous) cord and brain lesions; endarteritis may cause infarction and a low-grade meningoencephalitis affecting the brain results in dementia (general paralysis of the insane) and in the spinal cord, a sensory ataxic syndrome (tabes dorsalis). The introduction of highly active antiretroviral therapies has greatly reduced the frequency of these complications in patients with access to these treatments. However, newer complications are now increasingly recognized such as neurological immune reconstitution inflammatory syndrome, a compartmentalization syndrome (cerebrospinal fluid escape). This chapter looks at these and other important issues regarding the background, diagnosis, treatment, and outlook for neurosyphilis and neuro-AIDS.


1996 ◽  
Vol 33 (3) ◽  
pp. 365-366 ◽  
Author(s):  
D. Y. Rim ◽  
D. Y. Cho ◽  
H. W. Taylor

Lysosomal storage disease involving the brain, spinal cord, liver, and spleen was discovered in a 6-month-old male emu ( Dromaius novaehollandiae). The diagnosis was based on light and electron microscopic studies and histochemical staining characteristics. This is the first case of lysosomal storage disease reported in a ratite.


2011 ◽  
Vol 18 (1) ◽  
pp. 113-115 ◽  
Author(s):  
Ju-Hong Min ◽  
Byoung Joon Kim ◽  
Kwang Ho Lee

We report the case of a patient who developed extensive brain lesions during fingolimod (FTY720) treatment in the TRANSFORMS study. His initial diagnosis was multiple sclerosis, but after encephalopathy anti-aquaporin4 antibody (anti-AQP4 Ab) was detected, it was changed to neuromyelitis optica spectrum disorder. After treatment with fingolimod, he developed bilateral extensive brain lesions. The brain MRI showed lesions predominantly involving the right frontal and parietal lobes, with vasogenic edema and enhancement. He had residual encephalomalacia and no recurrence with steroid treatment over 3 years following withdrawal of fingolimod.


1993 ◽  
Vol 51 (3) ◽  
pp. 329-332 ◽  
Author(s):  
Aílton Melo ◽  
Luciana Moura ◽  
Solana Rios ◽  
Marcos Machado ◽  
Gersonita Costa

Magnetic resonance imaging of the brain and spinal cord were carried out for seventeen consecutive patients with HTLV-1 associated myelopathy (HAM). Eight patients had brain abnormalities and four had decreased thoracic spinal cord diameter. Brain lesions were mostly located in subcortical and periventricular areas. Our data suggest that diffuse central nervous system lesions are present in patients with HAM.


2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Ilya Kister ◽  
Joseph Herbert ◽  
Yongxia Zhou ◽  
Yulin Ge

Background. Brain lesions are common in neuromyelitis optica spectrum disorder (NMOsd) and may resemble lesions of multiple sclerosis (MS).Objectives. To describe the imaging characteristics of supratentorial lesions in NMOsd on ultrahigh-field (7 T) MRI with special attention to vessel-lesion relationship.Methods. Ten NMOsd patients, all women and all seropositive for NMO IgG, with mean age of 51.3 ± 15.4 years and disease duration of 9.2 ± 6.4 years, were scanned at a 7 T whole-body human MR system with high-resolution 2D gradient echo sequence optimized to best visualize lesions and venous structures, T2- and T1-weighted imaging.Results. In 10 patients with NMOsd, a total of 92 lesions were observed (mean: 9.2 ± 8.8; range: 2–30), but only 8 lesions (9%) were traversed by a central venule. All lesions were <5 mm in diameter, and 83% were located in subcortical white matter. There were no lesions in the cortex or basal ganglia. Two patients exhibited diffuse periependymal abnormalities on FLAIR.Conclusions. Small, subcortical lesions without a central venule are the most consistent finding of NMOsd on 7 T MRI of the brain. Ultrahigh-field imaging may be useful for differentiating between NMOsd and MS.


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