Assuntos
Anticorpos/efeitos adversos , Disartria/imunologia , Encefalite/imunologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Anticorpos/sangue , Disartria/complicações , Disartria/diagnóstico por imagem , Disartria/patologia , Encefalite/complicações , Encefalite/diagnóstico por imagem , Encefalite/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Neuroimagem , Tegmento Mesencefálico/patologiaRESUMO
Progressive supranuclear palsy (PSP) is the most common atypical Parkinsonism. Although PSP shares some symptomology with Parkinson's disease (PD), PSP has a different underlying pathology characterized by tau aggregation. Furthermore, PSP sufferers respond poorly to PD medications and there are no effective alternative therapeutics. The development of both palliative and disease altering therapeutics has been hampered by the lack of an animal model that displays relevant PSP-like pathology and behavioral deficits. Previously, our lab found that in rats the selective removal of cholinergic pedunculopontine neurons (whose axonal projections overlap with areas of PSP pathology), mimics the extensive loss of cholinergic pedunculopontine neurons seen in PSP, and produces a unique PSP-like combination of deficits in: startle reflex, attention, and motor function. The present study extends those findings by allowing the lesion to incubate for over a year and compares behavioral and post-mortem pathology of pedunculopontine-cholinergic-lesioned and sham-lesioned rats. There was an early startle reflex deficit which did not improve over time. Progressive declines in motor function developed over the course of the year, including an increase in the number of "slips" while navigating various beams and poorly coordinated transitions from an elevated platform into homecages. Histological analysis discovered that the loss off cholinergic pedunculopontine neurons precipitated a significant loss of substantia nigra tyrosine hydroxylase-positive neurons and a significant enlargement of the lateral ventricles. The latter is a distinguishing feature between PSP and PD. This preclinical animal model of PSP has the potential to further our understanding of PSP and aid in the testing of potential therapeutic agents.
Assuntos
Colinérgicos/farmacologia , Neurônios Colinérgicos/patologia , Atividade Motora/efeitos dos fármacos , Paralisia Supranuclear Progressiva/tratamento farmacológico , Tegmento Mesencefálico/patologia , Acetilcolina/farmacologia , Animais , Neurônios Colinérgicos/efeitos dos fármacos , Modelos Animais de Doenças , Masculino , Doenças do Sistema Nervoso/tratamento farmacológico , Doenças do Sistema Nervoso/patologia , Ratos Long-Evans , Substância Negra/efeitos dos fármacos , Substância Negra/patologia , Paralisia Supranuclear Progressiva/fisiopatologia , Tegmento Mesencefálico/efeitos dos fármacos , Tirosina 3-Mono-Oxigenase/efeitos dos fármacosRESUMO
The induction of general anesthesia shares many features with the transition from wakefulness to non-rapid eye movement (NREM) sleep, suggesting that the two types of brain-state transition are orchestrated by a common neuronal mechanism. Previous studies revealed a brainstem locus, the mesopontine tegmental anesthesia area (MPTA), that is of singular importance for anesthetic induction. Microinjection of GABAergic anesthetics there induces rapid loss-of-consciousness and lesions render the animal relatively insensitive to anesthetics administered systemically. Here we show that MPTA lesions also alter the natural sleep-wake rhythm by increasing overall wake time at the expense of time asleep (NREM and REM sleep equally), with nearly all of the change occurring during the dark hours of the light-dark cycle. The effect was proportional to the extent of the lesion and was not seen after lesions just outside of the MPTA, or following sham lesions. Thus, MPTA neurons appear to play a role in natural bistable brain-state switching (sleep-wake) as well as in loss and recovery of consciousness induced pharmacologically.
Assuntos
Sono/fisiologia , Tegmento Mesencefálico/fisiopatologia , Vigília/fisiologia , Anestesia Geral , Anestésicos Gerais/farmacologia , Animais , Eletrodos Implantados , Eletroencefalografia , Eletromiografia , Ácido Ibotênico , Masculino , Atividade Motora/fisiologia , Músculo Esquelético/fisiologia , Fotoperíodo , Ratos Wistar , Sono/efeitos dos fármacos , Tegmento Mesencefálico/efeitos dos fármacos , Tegmento Mesencefálico/patologia , Fatores de Tempo , Vigília/efeitos dos fármacosRESUMO
BACKGROUND: Basilar artery occlusion can cause locked-in syndrome, which is characterized by quadriplegia, anarthria, and limited communication via eye movements. Here, we describe an uncommon stroke syndrome associated with endovascular recanalization of the top of the basilar artery: "reverse locked-in syndrome." METHODS: We report the case of a patient with atypical neurological deficits caused by acute ischemic stroke of the midbrain tegmentum. We perform neuroanatomic localization of the patient's infarcts by mapping the magnetic resonance imaging (MRI) data onto a brainstem atlas. RESULTS: A 61-year-old man presented with acute coma and quadriplegia due to top of the basilar artery occlusion. He underwent emergent endovascular thrombectomy, with successful recanalization of the basilar artery at 4 h and 43 min post-ictus. The patient regained consciousness and purposeful movement in all four extremities, but the post-procedure neurological examination demonstrated bilateral ptosis with complete pupillary and oculomotor paralysis. MRI revealed infarction of the bilateral oculomotor nuclei in the midbrain tegmentum. At 9-month follow-up, he had anisocoria and dysconjugate gaze, but was living at home and required minimal assistance in performing all activities of daily living. CONCLUSIONS: Since the patient's deficits were the exact opposite of those described in locked-in syndrome, we propose the term "reverse locked-in syndrome" to describe this neurological entity characterized by bilateral ptosis, non-reactive pupils, and ophthalmoplegia with preservation of consciousness and extremity motor function.
Assuntos
Artéria Basilar/patologia , Blefaroptose/etiologia , Infarto Cerebral/complicações , Oftalmoplegia/etiologia , Tegmento Mesencefálico/patologia , Infarto Cerebral/terapia , Humanos , Masculino , Pessoa de Meia-Idade , TrombectomiaRESUMO
A 79-year-old man presented with a slowly progressive gait disturbance. Brain MRI demonstrated ventriculomegaly and the hummingbird sign. A lumbar puncture showed no abnormalities of the cerebrospinal fluid. The improvement of the gait disturbance after the ventriculoperitoneal shunt led to a diagnosis of idiopathic normal pressure hydrocephalus. Interestingly, postoperative brain MRI demonstrated the disappearance of not only ventriculomegaly, but also the hummingbird sign. The disappearance of the hummingbird sign suggests that an increase in the cerebrospinal fluid in the lateral and third ventricles could cause the compression of the superior surface of the midbrain tegmentum, which manifests as the hummingbird sign.
Assuntos
Hidrocefalia de Pressão Normal/patologia , Hidrocefalia de Pressão Normal/cirurgia , Idoso , Transtornos Neurológicos da Marcha/etiologia , Humanos , Hidrocefalia de Pressão Normal/complicações , Imageamento por Ressonância Magnética , Masculino , Tegmento Mesencefálico/patologia , Derivação VentriculoperitonealRESUMO
There has been increasing interest in the rostromedial tegmental nucleus (RMTg), given its potential regulatory role in many aversion-related behaviors. The RMTg contains mostly GABAergic neurons, sends a dense inhibitory projection to dopamine neurons in the midbrain, and is rich with µ-opioid receptors (MOR). Like most addictive drugs, ethanol has both aversive and rewarding properties. However, the cellular mechanisms underlying the effects of ethanol, particularly the aversive effect that limits its intake are not well understood. Recent studies have linked aversion with synaptic inhibition of dopamine neurons in the ventral tegmental area. To determine a potential role that the RMTg plays in the effect of ethanol, in this study, we employed a neurotoxin, dermorphin-saporin (DS), to lesion RMTg neurons prior to assessing ethanol-related behaviors. Rats were infused with DS bilaterally into the RMTg. This manipulation substantially increased the intake and preference for ethanol but not sucrose. It also reduced the number of neurons with MOR and glutamic acid decarboxylase 67 immunoreactivity within the RMTg. These changes did not occur after intra-RMTg infusion of blank saporin or vehicle. Importantly, intra-RMTg DS infusion significantly enhanced expression of conditioned place preference induced by ethanol (2 g/kg, i.p.), and slowed the extinction process. These results suggest that MOR-expressing GABAergic neurons in the RMTg contribute significantly to the regulation of ethanol consumption and related behaviors.
Assuntos
Consumo de Bebidas Alcoólicas/metabolismo , Transtornos Relacionados ao Uso de Álcool/metabolismo , Neurônios GABAérgicos/metabolismo , Receptores Opioides mu/metabolismo , Tegmento Mesencefálico/metabolismo , Consumo de Bebidas Alcoólicas/patologia , Transtornos Relacionados ao Uso de Álcool/patologia , Animais , Depressores do Sistema Nervoso Central/administração & dosagem , Condicionamento Psicológico/efeitos dos fármacos , Condicionamento Psicológico/fisiologia , Modelos Animais de Doenças , Etanol/administração & dosagem , Extinção Psicológica/efeitos dos fármacos , Extinção Psicológica/fisiologia , Feminino , Neurônios GABAérgicos/efeitos dos fármacos , Neurônios GABAérgicos/patologia , Glutamato Descarboxilase/metabolismo , Atividade Motora/efeitos dos fármacos , Atividade Motora/fisiologia , Peptídeos Opioides , Distribuição Aleatória , Ratos Sprague-Dawley , Proteínas Inativadoras de Ribossomos Tipo 1 , Saporinas , Autoadministração , Comportamento Espacial/efeitos dos fármacos , Comportamento Espacial/fisiologia , Tegmento Mesencefálico/efeitos dos fármacos , Tegmento Mesencefálico/patologiaRESUMO
We describe a case of a 48-year-old woman who underwent a resection of a tectal pilocytic astrocytoma complicated by a sequence of fluctuating consciousness, psychosis with complex hallucinations and lasting sleeping disturbances in which she vividly acts out her dreams. Based on the clinical and anatomical evidence of this case, we propose the term locus coeruleus syndrome to describe this association of iatrogenic symptoms. Along with those of the locus coeruleus, lesions of the dorsal raphe nucleus, ventral tegmentum, substantia nigra pars compacta, the superior colliculus and other peduncular lesions (such as peduncular hallucinosis) are involved in the regulation of sleep-wake/arousal, behaviour, sleeping disorders and rapid eye movement atonia. However, iatrogenic lesion of the locus coeruleus could explain the complications on all levels in our patient.
Assuntos
Astrocitoma/cirurgia , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/cirurgia , Locus Cerúleo/patologia , Tegmento Mesencefálico/patologia , Tegmento Mesencefálico/cirurgia , Astrocitoma/patologia , Feminino , Alucinações/etiologia , Alucinações/fisiopatologia , Humanos , Locus Cerúleo/fisiopatologia , Pessoa de Meia-Idade , Transtornos do Sono-Vigília/etiologia , Transtornos do Sono-Vigília/patologia , Transtornos do Sono-Vigília/fisiopatologia , Síndrome , Resultado do Tratamento , Derivação Ventriculoperitoneal/métodosRESUMO
The effects of the ibotenic acid infused into the area of the laterodorsal tegmental nucleus (LDT) of rats on the expression of cortical and accumbal neuropeptides were assessed. The effects of this manipulation were determined in the nucleus accumbens (NAc) and medial prefrontal cortex (mPFC) by estimating the numerical density of varicosities immunoreactive for vesicular acetylcholine transporter and the total number of NAc neurons immunoreactive for choline acetyltransferase (ChAT) and neuropeptide Y (NPY) as well as the total number of mPFC neurons immunoreactive for NPY and vasoactive intestinal polypeptide (VIP). In LDT-lesioned rats, the density of the cholinergic varicosities was reduced in the ventral divisions of the mPFC and in all divisions of the NAc. In addition, in these rats, the total number of NPY-immunoreactive neurons was reduced in all subregions of the mPFC and in the NAc. Conversely, the total number of VIP-immunoreactive neurons in the mPFC and of ChAT-immunoreactive neurons in the NAc did not differ between LDT- and sham-lesioned rats. These data provide the first direct evidence for a relationship between selective damage of LDT cholinergic neurons and decreased expression of NPY in the mPFC and NAc. They also reveal that different types of cortical and accumbal interneurons respond differently to the cholinergic denervation induced by LDT lesions.
Assuntos
Colina O-Acetiltransferase/metabolismo , Neuropeptídeo Y/metabolismo , Núcleo Accumbens/metabolismo , Córtex Pré-Frontal/metabolismo , Tegmento Mesencefálico/fisiopatologia , Proteínas Vesiculares de Transporte de Acetilcolina/metabolismo , Animais , Contagem de Células , Ácido Ibotênico/toxicidade , Imuno-Histoquímica , Masculino , Neurônios/metabolismo , Neurônios/patologia , Núcleo Accumbens/patologia , Fotomicrografia , Córtex Pré-Frontal/patologia , Ratos Wistar , Tegmento Mesencefálico/efeitos dos fármacos , Tegmento Mesencefálico/patologia , Peptídeo Intestinal Vasoativo/metabolismoRESUMO
Sudden infant death syndrome (SIDS) is a major cause of infant mortality throughout the world, yet its cause and mechanism of action remain poorly understood. Here, we discuss a novel model of the etiology of SIDS which ties together what is known about the brain regions thought to be affected in SIDS infants with a defined neuroanatomical circuit and a documented preventative factor in young children. We propose that SIDS occurs due to a lack of sufficient development and plasticity of glutamatergic synapses in the mesencephalic nucleus of the trigeminal nerve (Me5) and reticular formation (RF) of the brainstem. This model is supported by evidence of brainstem dysfunction in SIDS as well as evidence of signaling through the Me5 and RF in another means of regulating cortical arousal. Furthermore, long-term plasticity of glutamatergic synapses is well known to play a critical role in learning and memory in other regions of the brain, implying that those mechanisms may also be relevant in the development of brainstem circuitry. This model clearly explains why SIDS deaths appear so suddenly with little pathological explanation and suggests a potentially novel way to prevent these deaths from occurring.
Assuntos
Modelos Neurológicos , Formação Reticular/fisiopatologia , Morte Súbita do Lactente/etiologia , Morte Súbita do Lactente/patologia , Sinapses/metabolismo , Tegmento Mesencefálico/patologia , Ácido Glutâmico/metabolismo , Humanos , Lactente , Recém-NascidoRESUMO
We describe two patients (a Filipino boy aged 2.7 years and a Kuwaiti girl aged 4.8 Years) with clinical and MRI findings consistent with the diagnosis of pontine tegmental cap dysplasia (PTCD) and compare them with 23 other cases reported in the literature. Both presented with feeding problems (VII nerve), sensori-neural deafness (VIII nerve) and hypotonia from birth and later developed corneal opacities due to loss of corneal sensation (V nerve). They have severe psychomotor developmental delay. The MRI of their brain showed a flattened ventral pons, vaulted "cap"- like structure protruding into 4th ventricle and a "molar tooth" sign. One of our patients also had Tetralogy of Fallot (TOF) successfully corrected. The other had no extracranial manifestations. The findings in our patients are similar to those reported except for the occurrence of TOF which has not been reported before in association with PTCD.
Assuntos
Ponte/anormalidades , Ponte/patologia , Tegmento Mesencefálico/anormalidades , Tegmento Mesencefálico/patologia , Idade de Início , Tronco Encefálico/patologia , Pré-Escolar , Surdez/congênito , Doenças do Nervo Facial/congênito , Doenças do Nervo Facial/patologia , Feminino , Quarto Ventrículo/patologia , Humanos , Recém-Nascido , Kuweit , Imageamento por Ressonância Magnética , Masculino , Tetralogia de Fallot/complicações , Doenças do Nervo Vestibulococlear/congênito , Doenças do Nervo Vestibulococlear/patologiaRESUMO
The frequency and clinical characteristics of intractable hiccups due to the medullary infarction is unknown. The aim of this study was to identify the lesions of hiccups using by brain MRI. Ninety acute medullary patients admitted to our stroke center within 14 days of stroke onset between April 2004 and August 2012 were retrospectively studied. We evaluated clinical characteristics and the frequency of the patients causing intractable hiccups among acute medullary stroke patients. We divided the patients into two groups, intractable hiccups group, and not hiccups group. Of 90 patients, five (5.5%) had intractable hiccups. Hiccups group had more frequently involved right middle medullary lesion than not hiccups group (hiccups group vs. not hiccups group; (60% vs. 4%, p < 0.001). In 16 cases reported the lesion of intractable hiccups, the right middle medullary lesion was 11 cases. We suspected that the lesion of the intractable hiccups was associated with the right middle medullary.
Assuntos
Soluço/etiologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/patologia , Tegmento Mesencefálico/patologia , Idoso , Idoso de 80 Anos ou mais , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosAssuntos
Atetose/diagnóstico , Neoplasias Encefálicas/diagnóstico , Coreia/diagnóstico , Linfoma Difuso de Grandes Células B/diagnóstico , Atetose/etiologia , Neoplasias Encefálicas/complicações , Coreia/etiologia , Humanos , Cápsula Interna/patologia , Linfoma Difuso de Grandes Células B/complicações , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Paresia/diagnóstico , Paresia/etiologia , Núcleo Subtalâmico/patologia , Tegmento Mesencefálico/patologia , Tálamo/patologiaRESUMO
A 69-year-old man presented with a sudden headache followed by unconsciousness. There was no head injury. The Glasgow Coma Scale (GCS) score was 3/15 and there was a left mydriasis, unreactive to light. The CT-scan showed a left acute subdural haematoma causing a remarkable mass effect. A supratentorial hemispheric craniotomy was performed. Nevertheless, after several weeks at the intensive care unit (ICU), the patient was still unresponsive to external stimuli and did not show any motor activity. A comfort care attitude was decided on with the family and the patient was extubated. However, a few days later, the patient subsequently showed a surprisingly favourable course, with improved wakefulness. Indeed, the GCS score improved, and the treatment plan was modified so that the patient benefited from rehabilitation. The MRI showed a right cerebral peduncle lesion, consistent with a Kernohan-Woltman notch phenomenon (KWNP). Six months later, the patient was able to walk and live quite normally.
Assuntos
Erros de Diagnóstico , Hematoma Subdural Agudo/diagnóstico , Tegmento Mesencefálico/patologia , Inconsciência/diagnóstico , Idoso , Anisocoria/etiologia , Craniotomia , Hematoma Subdural Agudo/complicações , Hemiplegia/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Midríase/etiologia , Tomografia Computadorizada por Raios X , Inconsciência/etiologiaRESUMO
UNLABELLED: Acute necrotizing encephalopathy is a rare neurologic disease most often triggered by a febrile viral event affecting an otherwise healthy infant. The clinical course is characterized by rapid deterioration of the neurological condition that often leads to coma and requires intensive care. The diagnosis is usually suggested by MRI, which shows symmetrical and focal necrotic lesions of thalami. Acute necrotizing encephalopathy has been linked in recent studies to an autosomal-dominant mutation of the gene for the protein RAN-binding protein 2. CASE REPORT: We report three cases in siblings of Tunisian origin. Two of them presented with acute necrotizing encephalopathy at the age of 9 months in the immediate aftermath of a viral infection. The molecular study conducted in the family showed that both patients and their mother were carriers of the missense mutation gene RAN-binding protein 2. COMMENTS: Although the role of Ran BP2 protein is incompletely known, mutation of the RANBP2 gene causes rare, reversible central neurologic disorders. Suspected diagnosis is facilitated by MRI, which shows specific lesions of multifocal, symmetric involvement of the thalami, brainstem tegmentum, supratentorial white matter, and cerebellum. Due to the low frequency of the disease and its non-specific clinical presentation, the diagnosis of acute necrotizing encephalopathy is a major challenge, while preventative measures can be proposed in familial mutation.
Assuntos
Análise Mutacional de DNA , Emigrantes e Imigrantes , Genes Dominantes/genética , Leucoencefalite Hemorrágica Aguda/genética , Chaperonas Moleculares/genética , Mutação de Sentido Incorreto/genética , Complexo de Proteínas Formadoras de Poros Nucleares/genética , Cerebelo/patologia , Aberrações Cromossômicas , Diagnóstico Diferencial , Progressão da Doença , Dominância Cerebral/fisiologia , França , Triagem de Portadores Genéticos , Humanos , Lactente , Leucoencefalite Hemorrágica Aguda/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Exame Neurológico , Tegmento Mesencefálico/patologia , Tálamo/patologia , Tunísia/etnologia , Viroses/complicaçõesRESUMO
Using magnetic resonance-diffusion tensor imaging (DTI), we examined white matter changes within the brains of patients diagnosed with idiopathic normal pressure hydrocephalus (INPH). We analyzed data for 24 INPH patients who were presented with typical clinical symptoms (gait disturbance, dementia, and/or urinary incontinence) and Evans index > 0.3, and compared these with the control data from 21 elderly persons (≥ 60 years). DTI brain images were obtained with a 3T scanner. Fractional anisotropy (FA) brain maps were generated using a computer-automated method, and tract-based spatial statistics (TBSS) were then applied to compare the FA brain maps of the INPH and control groups in standard space. The TBSS data were further investigated using region-of-interest (ROI) analyses. ROIs were set within the corpus callosum, the posterior limb of the internal capsule (PLIC), and the cerebral peduncle in reference to a standard brain template. Compared with the control group, FA values in the INPH group were significantly lower in the corpus callosum and just significantly higher in the PLIC, but no significant differences were evident in the cerebral peduncle. The much lower FA values in the corpus callosum, but not the slightly higher FA values in the PLIC, were associated with more severe clinical symptoms such as gait disturbance. The lower FA values in the corpus callosum may offer a clue to solve the pathophysiology of INPH.
Assuntos
Hidrocefalia de Pressão Normal/patologia , Idoso , Idoso de 80 Anos ou mais , Anisotropia , Estudos de Casos e Controles , Corpo Caloso/patologia , Imagem de Tensor de Difusão , Feminino , Humanos , Cápsula Interna/patologia , Masculino , Pessoa de Meia-Idade , Tegmento Mesencefálico/patologiaRESUMO
Navigation depends on a network of neural systems that accurately monitor an animal's spatial orientation in an environment. Within this navigation system are head direction (HD) cells which discharge as a function of an animal's directional heading, providing an animal with a neural compass to guide ongoing spatial behavior. Experiments were designed to test this hypothesis by damaging the dorsal tegmental nucleus (DTN), a midbrain structure that plays a critical role in the generation of the rodent HD cell signal, and evaluating landmark based navigation using variants of the Morris water task. In Experiments 1 and 2, shams and DTN-lesioned rats were trained to navigate toward a cued platform in the presence of a constellation of distal landmarks located outside the pool. After reaching a training criteria, rats were tested in three probe trials in which (a) the cued platform was completely removed from the pool, (b) the pool was repositioned and the cued platform remained in the same absolute location with respect to distal landmarks, or (c) the pool was repositioned and the cued platform remained in the same relative location in the pool. In general, DTN-lesioned rats required more training trials to reach performance criterion, were less accurate to navigate to the platform position when it was removed, and navigated directly to the cued platform regardless of its position in the pool, indicating a general absence of control over navigation by distal landmarks. In Experiment 3, DTN and control rats were trained in directional and place navigation variants of the water task where the pool was repositioned for each training trial and a hidden platform was placed either in the same relative location (direction) in the pool or in the same absolute location (place) in the distal room reference frame. DTN-lesioned rats were initially impaired in the direction task, but ultimately performed as well as controls. In the place task, DTN-lesioned rats were severely impaired and displayed little evidence of improvement over the course of training. Together, these results support the conclusion that the DTN is required for accurate landmark navigation.
Assuntos
Sinais (Psicologia) , Meio Ambiente , Aprendizagem em Labirinto/fisiologia , Tegmento Mesencefálico/fisiologia , Animais , Feminino , Masculino , Ratos , Ratos Long-Evans , Tegmento Mesencefálico/patologiaRESUMO
Diffusion tensor imaging (DTI) has allowed in vivo demonstration of axonal architecture and connectivity. This technique has set the stage for numerous studies on normal and abnormal connectivity and their role in developmental and acquired disorders. Referencing established white matter anatomy, DTI atlases, and neuroanatomical descriptions, this article summarizes the major white matter anatomy and related structures relevant to the clinical neuroradiologist in daily practice.
Assuntos
Encéfalo/patologia , Doenças Desmielinizantes/patologia , Imagem de Difusão por Ressonância Magnética/métodos , Fibras Nervosas Mielinizadas/patologia , Vias Neurais/patologia , Adolescente , Adulto , Anisotropia , Criança , Corpo Caloso/patologia , Doenças Desmielinizantes/diagnóstico , Humanos , Aumento da Imagem , Interpretação de Imagem Assistida por Computador , Leucoencefalopatias/diagnóstico , Leucoencefalopatias/patologia , Masculino , Rede Nervosa/patologia , Tratos Piramidais/patologia , Valores de Referência , Tegmento Mesencefálico/patologia , Adulto JovemAssuntos
3-Iodobenzilguanidina , Compostos Radiofarmacêuticos , Paralisia Supranuclear Progressiva/diagnóstico por imagem , Paralisia Supranuclear Progressiva/metabolismo , Idoso , Autopsia , Encéfalo/patologia , Gliose/patologia , Humanos , Masculino , Neurônios/patologia , Pneumonia/complicações , Cintilografia , Paralisia Supranuclear Progressiva/psicologia , Tegmento Mesencefálico/patologiaRESUMO
BACKGROUND AND PURPOSE: Magnetic resonance imaging (MRI) of autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) cases in Quebec and Europe was reported to show linear hypointensities in T2-weighted and Fluid Attenuated Inversion Recovery (FLAIR) images of the pons. We attempted to clarify the characteristics of the brain MRI findings in ARSACS cases. METHODS: Eight Japanese early-onset ataxia patients with ARSACS confirmed molecularly were investigated. We performed neurological examination, SACS gene analysis, and MRI in the patients. RESULTS: Hypointensity lesions in the middle cerebellar peduncles in addition to the pons were observed in T2-weighted and FLAIR images in all eight cases. Although superior cerebellar atrophy was seen in all cases, this MRI finding might not be specific for ARSACS. Upper cervical cord and medulla oblongata atrophy was not observed in 3 of the 7 patients examined. CONCLUSION: Not only pontine but also middle cerebellar peduncle hypointensity lesions observed in T2-weighted and FLAIR images could be specific findings for ARSACS even in cases with variable clinical phenotypes.