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PubMed1992

[A case of adrenoleukodystrophy having progressed from the frontal lobes].

A 12-year-old boy was admitted to our hospital in May 1990. Since two years previously, he became restless and could not adapt himself to his new school mates and teacher. He came to refuse going to school, and his intellectual performance rapidly deteriorated, thereafter. During the following two years, he gradually became mutic and incontinent with dystonic involuntary movements, and was admitted to our hospital. Examination revealed melanoderma, mutism, dementia and grossly normal visual and auditory system except for bilateral optic atrophy. Volitional movements were severely limited because of marked truncal and extreme dystonia and spastic paresis. Urinary 17-KS and 17-OHCS were decreased. Serum cortisol did not increase normally on rapid ACTH test. Very-long-chain fatty acids in serum were increased. Serial CTs and MRI demonstrated marked ventricular dilatation and diffuse white-matter lesions involving the frontal lobes, corpus callosum, internal capsules, and less markedly parieto-occipital regions with slight calcification in the optic radiations. Those in the frontal lobes had been visualised in CTs taken 18 months after the onset. School refusal, the initial symptoms of the patient, is one of serious socioeducational problems in Japan. While the majority of children with school refusal might be of non-organic affections, those with apparent mental deterioration and behavioral abnormalities should undergo non-invasive CT or MRI evaluation during the early stage of the disorder. And if diffuse white matter lesions is suggested even in the frontal lobe, the possibility of adrenoleuko-dystrophy should be considered.

Adrenoleukodystrophy↗
PubMed2003

Delayed-onset cerebral arterial gas embolism in a commercial airline mechanic.

A commercial airline mechanic was evaluated for right-sided hemianesthesia. Thorough diagnostic testing failed to identify a definitive etiology, and the mechanic was assessed as having symptoms of a left internal capsule lesion, likely from an ischemic event. On day 12 after symptom onset, he consulted a diving medicine specialist for clearance to continue recreational scuba diving. A thorough history revealed that the patient worked regularly in a compressed air environment of commercial aircraft and had experienced a rapid decompression approximately 48 h prior to onset of the hemianesthesia. The specialist considered pulmonary barotrauma-induced cerebral arterial gas embolism as a possible diagnosis. On day 13 he was treated with hyperbaric oxygen using Treatment Table VI, which produced immediate relief. Following three additional hyperbaric oxygen treatments in the next 11 d, he reported nearly total resolution of his symptoms. This occurrence is believed to be the second report of a cerebral arterial gas embolism in an aircraft mechanic or maintenance crewman and suggests that the latency between time of depressurization and the development of symptoms from a pulmonary barotrauma-induced cerebral arterial gas embolism may extend longer than previously believed.

Adult↗
PubMedDate not supplied

Forthergillian Lecture. Imaging human brain function.

The non-invasive brain scanning techniques introduced a quarter of a century ago have become crucial for diagnosis in clinical neurology. They have also been used to investigate brain function and have provided information about normal activity and pathogenesis. They have been used to investigate functional specialization in the brain and how specialized areas communicate to generate complex integrated functions such as speech, memory, the emotions and so on. The phenomenon of brain plasticity is poorly understood and yet clinical neurologists are aware, from everyday observations, that spontaneous recovery from brain lesions is common. An improved understanding of the mechanisms of recovery may generate new therapeutic strategies and indicate ways of modulating mechanisms that promote plastic compensation for loss of function. The main methods used to investigate these issues are positron emission tomography and magnetic resonance imaging (M.R.I.). M.R.I. is also used to map brain structure. The techniques of functional brain mapping and computational morphometrics depend on high performance scanners and a validated set of analytic statistical procedures that generate reproducible data and meaningful inferences from brain scanning data. The motor system presents a good paradigm to illustrate advances made by scanning towards an understanding of plasticity at the level of brain areas. The normal motor system is organized in a nested hierarchy. Recovery from paralysis caused by internal capsule strokes involves functional reorganization manifesting itself as changed patterns of activity in the component brain areas of the normal motor system. The pattern of plastic modification depends in part on patterns of residual or disturbed connectivity after brain injury. Therapeutic manipulations in patients with Parkinson's disease using deep brain stimulation, dopaminergic agents or fetal mesencephalic transplantation provide a means to examine mechanisms underpinning plastic change. Other models of plastic change, such as normal visuospatial learning or re-establishing speech comprehension after cochlear implantation in the deaf illustrate how patterns of brain function adapt over time. Limitations of the scanning techniques and prospects for the future are discussed in relation to new developments in the neuroimaging field.

Brain↗
PubMed1992

[Isolated abducens nerve palsy caused by pontine infarction].

An 82-year-old hypertensive man suddenly developed diplopia during right lateral gaze. Neurological examination revealed right isolated abducens nerve palsy without any other findings. By cranial CT scan, a low density area over the posterior limb of right internal capsule and tortuosity of basilar artery were noted. 3 months later, his symptom disappeared and then he was well in next 2 years til he felt diplopia during left lateral gaze. On this time he showed left isolated abducens nerve palsy. Though cranial CT scan failed to find out new abnormality, T2-weighted cranial MRI disclosed high intensity spot over left pontine base located between medial lemniscus and pyramidal tract, which was supposed to coincide to fascicle of left abducens nerve Three months later, he recovered in the same manner as 2 years before. Hemilateral isolated abducens nerve palsy may be caused by many origins, but pontine infarct was not detected so much in pre-MRI era. Being the long-term prognosis of the lacunar infarction not satisfactory, it is important for the cases of isolated abducens palsy to ascertain whether there is pontine small infarction or not. So in these cases, precise examination including MRI should be needed.

Abducens Nerve↗
PubMed2003

[Clinical characteristics of X-linked adrenoleukodystrophy].

OBJECTIVE: X-linked adrenoleukodystrophy (ALD) is a genetically determined disorder that involves the nervous system white matter, axons, adrenal cortex and testes. The typical clinical manifestations are progressive psychomotor regression, vision and/or auditory impairment and adrenal insufficiency. The clinical manifestation, biochemical change and genetic counseling work of X-linked ALD were analyzed. METHODS: The clinical features of 29 cases with ALD were summarized and analyzed, including symptoms and signs, measurement of blood very long chain fatty acids (VLCFA), adrenal function, cranial magnetic resonance imaging (MRI) and pedigree investigation. RESULTS: Among these 29 cases, the clinical phenotype could be classified into childhood cerebral (22 cases), adolescent cerebral (4 cases), adrenomyeloneuropathic (1 case), Addison's disease (1 case) and asymptomatic or presymptomatic (1 case) types. Nine of them had positive family history. Pedigree investigation was consistent with typical sex-linked recessive inheritance. There were 45 ALD patients in these 29 pedigrees. The neurological manifestations varied among members of the same family. Nine cases died during follow up. The causes of death were central respiratory failure or other complications of ALD and so on. Laboratory tests demonstrated abnormally high plasma levels of VLCFA in ALD patients; MRI demonstrated symmetric butterfly-like low T(1) and high T(2) signals in the parieto-occipital white matter. The impairment in the splenium of corpus callosum made the bilateral lesion region converge into one. It could progress anteriorly and injure the bilateral posterior limb of internal capsule and the temporal lobe, and could injure the brainstem inferiorly. Following intravenous injection of contrast material, thin stripe of lacelike enhancement could be observed. CONCLUSIONS: The atypical initial symptom of ALD was seizures. The MRI showed abnormal signal in the cerebellar white matter. This disease can influence the normal development of children, this was more pronounced in the childhood cerebral ALD type. It tended to progress rapidly with dementia, vegetative state or death. Since antenatal diagnostic method is available now, emphasis should be made on the antenatal examination in order to make an early diagnosis and abort pregnancy if necessary.

Adolescent↗
PubMed1992

[Palilalia and acquired stuttering in a case of Parkinson's disease].

We report palilalia and acquired stuttering in a 60-year-old Japanese male with Parkinson's disease. At the age of 54, he presented with resting tremor in the hand and foot on the left, and gradual slowness in voluntary movements. Two years later, resting tremor involved the right foot, and an expressionless face and frozen gait occurred. A diagnosis of Parkinson's disease was made and treatment with L-dopa and carbidopa resulted in conspicuous improvement. At the age of 57, he developed compulsive repetitions of syllables, words and phrases, and sentences infrequently when he spoke. They have been persisting for four years. Repetitions increased in spontaneous speech while they decreased in oral reading and repetition of sentences. These repetitions in speech were symptomatologically diagnosed as palilalia and acquired stuttering. Brain CT showed slight brain atrophy, and brain MRI disclosed a few lesions indicating lacunae in the left substantia nigra, left putamen, and right internal capsule. SPECT showed a slight decrease in blood flow in the frontal lobes and basal ganglia bilaterally. Full IQ on WAIS was 105, and neither agnosia nor apraxia was detected. Palilalia and acquired stuttering, though the pathomechanism has not been clarified, have been reported to occur usually secondary to cerebral vascucular lesions and very rarely in Parkinson's disease. In the present case, they may have been produced by the parkinsonian nigro-striatal lesions. Alternatively, they may have been induced by the small vascular lesions demonstrated by MRI.

Atrophy↗
PubMed1992

Comparative migration and development of astroglial and oligodendroglial cell populations from a brain xenograft.

In previous studies of brain transplantation, the fate of the implanted glial cells has been investigated separately; that is, the interest has been focused either on the astroglia or on the oligodendroglia. However, the two populations of implanted glial cells may interact with each other, for example by secreting species-specific factors or by inducing reactions by the host. We have used two different models of brain transplantation: one that allows the identification of the implanted astrocytes, and another that allows the identification of the implanted oligodendroglia. The present model is a combination of both; it consists of the grafting of embryonic rabbit brain fragments into the brains of neonatal Shiverer mice. The myelin made by the implanted oligodendrocytes is identified by anti-myelin basic protein immunohistochemistry. The implanted astrocytes are identified by a monoclonal antibody that combines with rabbit but not with mouse glial fibrillary acidic protein. This study shows that although they use the same major routes of migration, both populations of glial cells tend to move differently. They demonstrate areas of common settlement but also areas where only one population of implanted glia is present. From the site of implantation in the dorsal striatum, the major routes of migration are the corpus callosum, the white matter fascicles in the striatum, and the internal capsule. After a delay of 6 weeks, no significant prevalence of one population of implanted glial cells over the other was observed.

Animals↗
PubMed2003

Deep brain stimulation in treatment refractory obsessive compulsive disorder.

Obsessive-compulsive disorder is a worldwide psychiatric disorder with a lifetime prevalence of 2% and mainly characterized by obsessional ideas and compulsive behaviors and rituals. Many patients show improvement under cognitive behavioral and/or pharmacological treatment. A minority of patients is refractory to all available therapy and may benefit from capsulotomy. This study aims to investigate deep brain stimulation (DBS) as a last resort treatment alternative to capsulotomy in treatment refractory obsessive-compulsive disorder. Eight patients have been implanted bilaterally in the anterior limbs of the internal capsules with a quadripolar electrode. This paper presents the results of the first 4 operated patients. Acute deep brain stimulation displays an immediate improvement of the speech, mood, eye contact and motor function. Chronic deep brain stimulation improves significantly the obsessional and compulsive symptomatology in three out of the four patients. This study definitely needs replication, but the results are promising. Reversible deep brain stimulation may improve the symptomatology of treatment refractory OCD patients, without significant side effects.

Adult↗
PubMed2003

Magnetic resonance imaging and spectroscopy in hepatic encephalopathy.

Hepatic encephalopathy (HE) is a potentially reversible neuropsychiatric syndrome secondary to cirrhosis and other severe liver diseases. Magnetic resonance imaging (MRI) and MR spectroscopy (MRS) have been explored to provide new insight into the pathophysiology, diagnosis and treatment of HE. MRI shows brain atrophy especially in the frontal region. Globus pallidus, putamen and portions of the internal capsule appear hyperintense on T1-weighted images; this is likely to be due to deposition of manganese as a result of portosystemic shunting and liver dysfunction. MRS permits the detection and quantification of certain brain metabolites in vivo. There is decrease in myoinositol and choline concentrations and increase in glutamine concentrations. There is no change in n-acetyl-aspartate. Depletion of myoinositol is the most sensitive and specific spectroscopic marker in HE. Its loss is most likely a compensatory mechanism for the accumulation of glutamine. In conclusion, MRI and MRS examine different aspects of hepatocerebral disease.

Hepatic Encephalopathy↗
PubMed2003

[Generalized choreic involuntary movements in the course of stroke: case report].

Involuntary movements are rare ischemic stroke complication. Hemichorea, hemibalism and dystonia cases were described, especially during haemorrhage into basal ganglia and thalamus. The case of 73 years old woman is presented who was admitted to our Clinic cause of sudden choreatic involuntary movements occurrence within axial muscles and limbs. Computed Tomography showed ischemic focus in basal ganglia (head of caudate nucleus and lentiformis nucleus) and anterior branch of internal capsule. Cerebral angio--CT confirmed partial occlusion both medial arteries and atherosclerotical changes within the rest of cerebral arteries.

Aged↗
PubMed1992

A new bioactive molecule for improving vascular graft patency: exploratory trials in dogs.

Myxalin is a new bioactive molecule that we have isolated from the culture medium of Myxococcus xanthus, a non-pathogenic Gram negative bacterium. This glycopeptide possesses an antithrombotic effect in vivo and has been shown to promote human endothelial cell growth in vitro. With the object of exploring its ability to improve vascular graft healing and patency, myxalin was immobilized on 6 mm diameter knitted polyester prostheses using gelatin as a carrier, and the prosthesis was then implanted as an infrarenal abdominal arterial substitute in dogs for a period of 2 weeks. Two additional series of implantations were conducted for control purposes: one with gelatin-coated prostheses without myxalin, the other following normal preclotting of the polyester grafts. In order to select adequate sterilization conditions which can preserve the biological activity of myxalin, the prostheses were sterilized according to 3 different sterilization processes (gamma radiation and ethylene oxide either at 63 degrees C or 37 degrees C). At the sacrifice, all grafts were patent. The myxalin treated prostheses exhibited improved blood compatibility in terms of fewer thrombotic deposits and significant inhibition of platelet and fibrinogen uptake on their luminal surfaces. In addition, the development of a thin collagenous internal capsule with endothelial cells secreting high levels of prostacyclin was observed at both anastomoses of the myxalin-treated grafts sterilized by gamma radiation.

6-Ketoprostaglandin F1 alpha↗
PubMedDate not supplied

White matter lesions in panencephalopathic type of Creutzfeldt-Jakob disease: MR imaging and pathologic correlations.

BACKGROUND AND PURPOSE: A panencephalopathic type of Creutzfeldt-Jakob disease (pCJD) is characterized by the extensive involvement of the cerebral white matter as well as the cerebral gray matter. It has been a point of controversy, however, whether the white matter changes represent primary or secondary degeneration. The aim of this study was to elucidate, by using MR images and histologic examinations, whether the white matter lesions in pCJD are primary or secondary degeneration. METHODS: Serial changes of T2 hyperintensities and histologic findings of six autopsy-proved cases of pCJD were retrospectively analyzed. RESULTS: Serial MR images of brains affected by pCJD revealed that T2 hyperintensities appeared in the cerebral gray matter 2-5 months after onset and in the cerebral white matter around the lateral ventricles approximately 5 months after onset. They rapidly extended to deep and subcortical white matter during the next several months and then to the entire cerebral white matter 10 months after onset. Histologic examination of the white matter lesions revealed spongy changes or tissue rarefaction associated with gemistocytic astrocytosis, which indicates primary involvement of the white matter. At the terminal stages of cases with a longer clinical course, MR images showed T2 hyperintensities in the corticospinal tracts in the internal capsule and brain stem, which histologically disclosed loss of myelin and axons accompanied by fibrillary gliosis that indicates secondary degeneration. CONCLUSION: Cerebral white matter lesions in pCJD were considered to be primary changes of the disease, but the lesions of the corticospinal tracts were secondary to cortical or cerebral or both white matter lesions.

Aged↗
PubMedDate not supplied

[Neurological complications arising from dengue virus infection].

INTRODUCTION: Dengue is the most common of the arbovirosis that humans can suffer from. The frequency with which the central nervous system (CNS) is affected by this viral infection remains unknown, although isolated cases with neurological complications have been reported in Asia and South America. In Rio de Janeiro, Brazil, dengue virus infection has become an important public health concern. CASE REPORTS: The authors describe two cases of immune-mediated CNS involvement following classic infection by the dengue virus: one involving post-infectious disseminated acute encephalitis and the other consisting of Guillain-Barré syndrome. In both cases dengue was diagnosed using the ELISA technique, and other viral aetiologies in the cerebrospinal fluid (CSF) were excluded. A 10-year-old female, following a bout of classic dengue, presented symptoms of a diminished level of consciousness, spastic tetraparesis, cerebellar syndrome and frontal symptoms. A resonance brain scan showed areas of hypersignal in T2 sequences in the cerebral peduncle, lentiform nuclei and internal capsule on both sides of the brain, which suggested post-infectious encephalitis. The second patient, a 14-year-old male, presented an areflexive flaccid ascending tetraparesis that suggested acute polyradiculoneuritis, following a bout of classic dengue. CSF albuminocytologic dissociation was also observed. This patient's electroneuromyogram recording showed a polyradiculoneuropathy of a primarily demyelinating nature with an associated axonal component. CONCLUSIONS: The immunological mechanisms involved in the pathophysiology of this type of neurological complications after suffering from dengue may be part of the physiological response to the viral infection.

Adolescent↗
PubMed2004

[Value of magnetization transfer imaging in judging microchanges lesions in normal-appearing white matter of multiple sclerosis].

OBJECTIVE: To explore the value of magnetization transfer imaging (MTI) in judging microscopic lesions in normal-appearing white matter of multiple sclerosis (MS). METHODS: Forty-one patients with brain MS, 17 males and 24 females, aged 13 approximately 65, and 21 healthy people, 8 males and 13 females, aged 18 approximately 57, used as controls underwent magnetic resonance imaging (MRI) using 3D-spoiled grass (3D-SPGR) series, to scan the whole brain with saturated pulses on and off respectively. The signal values were measured directly in the pictures of these 2 series. The formula MTR = (M(0)-MS)/M(0) x 100% was used, where M0 represents the signal value of region of interest with the saturated pulses off, and Ms represents signal value of region of interest with the saturated pulses on, to calculate the MTR value. With reference to T(2)WI imaging, the MTR values of 17 regions of interest (ROI) in the normal-appearing brain white matter, including the white matter of pons, bilateral cerebellar peduncles, knees of internal capsules, splenium and genu of corpus callosum, and the white matter by the anterior horn and posterior horn of lateral ventricle, body of lateral ventricle, and deep in the frontal and parietal lobes, were measured. The average MTR value of the 17 ROI was used to represent the MTR value of the whole normal-appearing brain and used to make comparison with that of the healthy samples statistically. RESULTS: Compared with those of the healthy samples, the average MTR values of each ROI of the MS patients were lower at different degrees. The difference in the MTR values of the white matter of splenium of corpus callosum, and by lateral ventricle and deep in parietal lobe were especially bigger. The MTR value of the whole brain white matter in the healthy persons ranged from 22.76% to 25.42%, with an average value of 23.97%, both significantly higher than those of the MS patients (19.45% to 24.15%, and 22.44% respectively, both P < 0.05). CONCLUSION: MTI can be used to detect the microchange of normal appearing white matter in MS. MTR is a sensitive indicator to reflect the damage of structure of tissues.

Adolescent↗
PubMedDate not supplied

[Intracranial haemorrhage secondary to a lightning strike: a case report].

INTRODUCTION: The damage caused to the central nervous system by lightning can be immediate or delayed. Cerebrovascular accidents are usually an infrequent complication of lightning strikes. CASE REPORT: We report the case of a patient who was hit by lightning and then developed an acute bilateral intraparenchymatous haemorrhage in the basal ganglia and the left internal capsule. DISCUSSION: Few cases of intracranial haemorrhages secondary to lightning strikes have been reported. We carry out a review and analysis of the literature currently available on the subject. A number of theories have been put forward that attempt to explain the mechanism behind these haemorrhages in patients who have been hit by lightning. The reason why there is a predilection for the basal ganglia is unknown, although it could be linked to the particular features of the vascularisation of the area.

Basal Ganglia↗
PubMed1992

[Use of magnetic resonance in the diagnosis of neuro-cryptococcosis in the acquired immunodeficiency syndrome: study of 4 patients].

Neuro-cryptococcosis is a common opportunistic infection in AIDS or HIV infected patients. From a series of 10 neuro-cryptococcosis the four of them studied by magnetic resonance (MR) are reported. In AIDS patients a high suspicion of opportunistic infection of the CNS is needed as exemplified by two of the four patients who only presented cephalalgia. The other two patients suffered additional symptoms and signs of meningeal and CNS involvement, such as nuchal rigidity, cranial nerve palsies, papilloedema, gait ataxia and dismetria. Diagnosis was achieved (confirmed) by a positive culture, serology or indian ink test in CSF. CT scan did not contribute to the diagnosis and management of the patients. In contrast MR, showed in three of them a peculiar pattern of small, confluent, high-signal lesions, roughly symmetrically placed in the basal ganglia and the internal capsule. They probably correspond to the dilated Virchow-Robin spaces through which torulae migrate from the subarachnoid space.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Blepharospasm associated with neurocysticercosis.

We report a case of a 48-year-old woman who had a 4 years history of bilateral blepharospasm. CT-scan showed multiple intracranial calcifications (two of them in the left thalamus) corresponding to neurocysticerosis, and a lacunar infarction in right internal capsule area. To our knowledge this case is the first one reported in the literature of blepharospasm associated with (and perhaps symptomatic of?) neurocysticercosis.

Blepharospasm↗
PubMed2005

[A 95-year-old female with autopsy-proven cerebral necrosis due to candidiasis who developed stroke-like manifestations].

A 95-year-old woman complained of sudden onset of disturbance of consciousness and right hemiparesis on April 20, 2003 and was admitted on the next day. She was drowsy and showed moderate right motor and sensory hemiparesis. The blood laboratory tests showed slight inflammatory reaction. A low density area was found in the left basal ganglia by brain CT, which was also coincided with the high signal region in T2, FLAIR and diffusion-MR images. The MRA of the intracerebral arteries presented no remarkable abnormality. The hemiparesis and impaired consciousness improved partially in the following week. However, she did not fully recover, since aspiration pneumonia and mild generalized inflammation continued. Percutaneous gastrostomy and intravenous hyperalimentation were started to improve her nutrition. The moderate inflammatory state persisted for several weeks. Her blood pressure suddenly fell and she died on June 12. Autopsy showed a mildly brownish and necrotic lesion from the left caudate to the putamen through the internal capsule. There was no liquefaction. On the microscopic examination, the necrosis surrounded by small vessels was consisted of numerous neutrophils and macrophages with pseudohypha and blastospore of candida. Small fragments of fungus were phagocytosed by macrophages. Small abscesses and necrotic foci due to candidiasis were observed in the bladder, kidneys, lungs, myocardium and thyroid gland. In this case, cerebral candidiasis probably occurred via hematogenous dissemination from a primary focus in the urinary tract. The intracerebral arteries revealed rather mild atherosclerotic changes and there was no occlusion by thromboembolism. Intracerebral lesion was diagnosed as candidiasis and there was no cerebral infarction by thromboembolism. If the infection occurred after cerebral infarction, there should not be any inflammatory reaction in the center of necrotic area. There have been few reports of cerebral candidal infection in patients without diabetes mellitus or immunosuppressive conditions. None of them had been diagnosed before death. Caution should be exercised for the presence of systemic candidiasis in elderly patients who are bedridden and with continuous low grade inflammatory reactions.

Aged↗