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At least 1,603 records · Page 89Linked to original sources
PubMed2006

Hashimoto's encephalopathy and motor neuron disease: a common autoimmune pathogenesis?

Hashimoto's encephalopathy is a rare complication of autoimmune thyroiditis not associated with thyroidal function decline. We report a 50-year-old man presenting with lower motor neuron symptoms evolving over 3 years and changes in behavior associated with attentive and cognitive impairment occurring in the last few months. Memory deficits, emotional instability, marked dysarthria, mild symmetric weakness of the lower extremities and fasciculations were the most striking clinical features. EEG was diffusely slow, cranial MRI revealed multiple subcortical white matter lesions, CSF protein was slightly elevated, electromyographic recordings showed acute and chronic denervation and extremely high TPO antibody titers were found in the serum. Hashimoto's encephalopathy and lower motor neuron disease were diagnosed. As repeated high-dose intravenous methylprednisolone administration followed by oral tapering improved both central nervous system and lower motor neuron symptoms, the question was raised whether there was a common autoimmune pathogenesis of both clinically distinct diseases.

Cerebral Cortex↗
PubMed2006

Hypertensive brain stem encephalopathy in a patient with chronic renal failure.

Hypertensive brain stem encephalopathy is a rare disorder that can be seen in severe hypertensive encephalopathy. Patients with chronic renal failure are more prone to develop this disorder because a mild elevation of the blood pressure can already induce brain changes. It is important to diagnose this entity as soon as possible because the symptoms and brain stem lesions are reversible following treatment and because it is important to exclude brain stem ischemia in the diagnostic work-up. Brain MRI and particularly diffusion weighted images are crucial for this purpose.

Aged↗
PubMed2006

Characterization of late-onset citrullinemia 1 in a Korean patient: confirmation by argininosuccinate synthetase gene mutation analysis.

A 16-month old boy was referred to our hospital for evaluation of recurrent generalized tonic clonic seizures. Metabolic evaluation revealed significant hyperammonemia (1,112 microg/dl). Amino acid/acylcarnitine screening using tandem mass spectrometry showed markedly increased plasma levels of citrulline (1,350 microM/l) with undetectable levels of arginine and arginosuccinic acid. Urinary excretion of citrulline was markedly increased (38,617 microM/g creatinine). Brain MRI findings showed diffuse high-signal intensity lesions, that involved gray and white matter in both frontal lobes and insula with edematous changes; these findings were consistent with the acute stage of citrullinemia (CTLN). Mutation analysis of the argininosuccinate synthetase (ASS) gene, in this patient, showed a Gly324Ser mutation in exon 13, and a 67-bp duplication mutation in exon 15 (c.1128-6_1188dup67). The patient was confirmed as having late-onset CTLN1 and treated with anticonvulsants, lactulose enema, protein restricted diet and arginine. Here we describe a case of late-onset CTLN1 in a patient by biochemical analyses and ASS gene mutation confirmation. This is the first report of a Korean patient with late-onset CTLN1 confirmed by ASS gene mutation identification.

Argininosuccinate Synthase↗
PubMed2001

Intravenous rtPA thrombolysis in acute ischemic stroke.

Early intravenous thrombolysis within the first three hours has been considered in the United States as the first proven treatment in acute ischemic stroke. However, not all patients will respond to this therapy which is also associated with a risk of symptomatic, including fatal, intracranial hemorrhage. This overview addresses the issue of efficacy and safety of intravenous alteplase (tPA) in acute cerebral ischemia. The rationale for thrombolytic therapy and its limits are described. The controlled studies show that intravenous tPA is effective and safe when given under restrictive conditions within 3 hours after stroke onset, but the data for a larger therapeutic window between 3 and 6 hours remain controversial. The expected functional improvement and the risk of intracranial hemorrhage greatly depend on selective clinical and imaging criteria. For this purpose, MRI, using the diffusion- and perfusion-weighted sequences combined with MR- angiography, should be preferred to CT scan in the next future. Applicability of tPA thrombolysis in current neurological practice in Belgium is discussed. Before its generalization, this therapy should be restricted to specialized stroke centers and all treated patients should be recorded in a central data bank to guarantee continued surveillance.

Acute Disease↗
PubMed2002

[Sporadic Creutzfeldt-Jakob disease and isolated dementia. Contribution of brain MRI].

Creutzfeldt-Jakob disease (CJD) is the most frequent transmissible spongiform encephalopathy. Its definite diagnosis is ascertained by cerebral neuropathological exam. However, diagnosis of a probable or possible case of CJD can be evoked on the basis of Masters'classification which is based on the association of different clinical and electroencephalographical criteria. We report the case of a 58-year-old woman who died in a geriatric unit of autopsy proven sporadic CJD. The clinical course over 15 months was rapidly progressive dementia without characteristic clinical and EEG signs. The case presentation did not meet the criteria of probable or possible CJD, according to Masters'classification. However, 4 months after the onset of the disease, t-Flair MRI revealed an increased signal intensity in the right frontal and occipital cortex which could suggest the diagnosis of CJD. This case therefore stresses the contribution of MRI, especially diffusion-weighted imaging, for early diagnosis of CJD. It shows also the short comings of Masters'classification which does not always enable diagnosis of CJD even though control measures would have to be rapidly undertaken, specially the decontamination of medico-surgical equipment. Finally, this case illustrates the great importance of post mortem exam in such context. In light of this clinical observation, we discuss this rare diagnosis which should be considered in geriatrics when confronted with a rapidly progressive dementia

Creutzfeldt-Jakob Syndrome↗
PubMed2002

[Diagnostic imaging techniques in the investigation of dementia].

BACKGROUND: Neuroimaging can provide valuable information in the diagnostic work-up of patients presenting with suspected dementia. MATERIAL AND METHODS: Based on our experience from a memory clinic at Ullevål University Hospital in Oslo, Norway and on relevant literature identified on Medline, we give an overview of the use of neuroimaging methods in patients with suspected dementia. RESULTS AND INTERPRETATION: CT of the brain should be offered to all patients with suspected dementia as CT can provide essential diagnostic information regarding focal cerebral pathology (tumour, haemorrhage, normal pressure hydrocephalus). A CT scan is of no value in the diagnostic evaluation of patients with mild to moderate Alzheimer's disease as age-related atrophy may be a confounding factor. CT is necessary to reveal infarcts when vascular dementia is suspected, but lacks sensitivity in the detection of diffuse cerebrovascular disease. MRI is recommended in younger patients and may be used to diagnose subcortical lesions, e.g. leukoariosis. The accuracy of SPECT in the assessment of patients with cognitive impairment is not yet established though it seems to be a promising method for the detection of frontotemporal dementia. Functional MR may play a role in the work-up of dementia in the future.

Alzheimer Disease↗
PubMed2002

[A case of slowly progressive unilateral spastic hemiparasis, hand clumsiness, limb ataxia and constructional apraxia].

A 65-year-old woman was seen in our clinic because of a 4-year history of slowly progressive left hand clumsiness and left limping. Neurologically, she had left-side spastic hemiparasis including her tongue, hand clumsiness, limb ataxia, constructional apraxia, and memory impairment. Dementia, parkinsonism and lower motor neuron sign were not confirmed. MRI study showed diffuse cerebral atrophy and shrinkage of the right cerebral peduncle and pontine base. SPECT (99mTc-ECD) study revealed hypoperfusion of the right fronto-parietal cortex. This is a rare case presented with slowly progressive left-side spastic hemiparasis, mimicking Mills' syndrome. In addition this case had other cortical signs, such as ipsilateral hand clumsiness, limb ataxia, constructional apraxia. Neuroimaging study suspects that the right front-parietal cortex is the primary lesion. Etiologically atypical motor neuron disease with adjacent cortical involvement is suspected rather than corticobasal degeneration with severe unilateral pyramidal tract degeneration.

Aged↗
PubMed2002

[Data bank project for acute stroke patients].

Stroke is the second cause of death within major adult disease and the first cause of functional disability for elderly in Japan. The diffusion rate of MRI is the highest in the world. Even though, there is only few evidence for stroke management has been reported from Japan. It is necessary to make a data bank of acute stroke patients as infrastructure making evidence for standardization of stroke management. We made Japan standard stroke registry study supported by ministry of health and welfare for 3 years. We completed computerized registry system and accumulated about 8,000 acute stroke cases from 45 stroke center hospitals. This system is also functioning as a stroke database for each hospital. From the analysis of the distribution of stroke subtype, the incidence of atherothrombotic infarction and cardiogenic embolism was similar to lacunar infarction as shown in Fig. 1. Furthermore, the 38% of ischemic stroke patients admitted within 3 hours. Thrombolytic therapy was performed in 15% of the patients who admitted within 3 hours and their initial severity were NIHSS 6-29. The outcome of the patients treated with thrombolytic therapy was significantly better than those without it. These data indicate that the stroke data bank should be useful tool to make verification of the guideline and planning of clinical trial for EBM.

Acute Disease↗
PubMed2003

[Transient dementia during hyperthyroidism of painless thyroiditis. A case report].

We report transient dementia in a 71-year-old woman, that was caused by hyperthyroidism of painless thyroiditis. Her friends, who found her very forgetful and performing abnormal behavior, brought her to our hospital. She looked alert and cooperative. There was neither goiter nor pain of the thyroid. Neurological examination revealed impairment of recent memory with a low score of memory test (MMSE: 20/30) indicating mild dementia. EEG showed slow alpha waves (8 Hz) with random theta and delta waves in the frontotemporal regions bilaterally. MRI showed mild diffuse atrophy of the brain, and SPECT revealed diffuse decrease in the cerebral blood flow most predominantly in the frontotemporal regions bilaterally. Abnormal findings included low levels of thyroid stimulating hormone (TSH), high levels of free triiodothyronine and free thyroxine. Antithyrogloblin antibody was positive and TSH receptor antibody was negative, while CRP was normal. Radioactive Tc uptake of the thyroid gland was low. A diagnosis of painless thyroiditis was made. Thyroid function turned to euthyroid state spontaneously in four weeks without antithyroid therapy, and subsequently memory disturbance and abnormal behavior disappeared. SPECT after clinical recovery showed improvement in the cerebral blood flow of the frontotemporal lobes. To our knowledge, dementia-like symptoms secondary to hyperthyroidism of painless thyroiditis have not been reported previously, and should be kept in mind as one of the causes of treatable dementia.

Aged↗
PubMed2003

[Intractable epilepsy (apneic seizure) in an infant with 18q deletion syndrome].

We report here an infant with 18q deletion syndrome, and intractable apneic seizures. He had intrauterine growth retardation and dysmorphic features. Chromosomal analysis demonstrated mosaicism of 18q interstitial deletion (q12.3-q22.3). From the age of 3 months, apneic attacks occurred from once a week to over 10 times a day despite many oral antiepileptic agents, and were diagnosed as complex partial seizures. Ictal electroencephalogram and 18F-fluorodeoxyglucose-positron emission tomography at the age of 10 months identified the epileptic focus in the right parieto-temporal region. He also had severe psychomotor retardation. Head MRI examination revealed diffuse cerebral atrophy and severe white matter dysmyelination, which was caused by the deletion of myelin basic protein gene at the locus of 18q22.3. This locus may be responsible for the clinical manifestations of 18q deletion syndrome. Detailed description of the onset, seizure types, and prognosis of epilepsy associated with 18q deletion syndrome is rare. It was suggested that the locus of 18q21.3-q22.3 was responsible for autonomic seizures in 18q deletion syndrome.

Apnea↗
PubMed2003

[Cerebral strokes in patients with systemic lupus erythematosus].

We examined a group of 83 patients (81 females and two males) diagnosed as systemic lupus erythenatosus (SLE) aged 19-77, with the mean duration of the disease of seven years. All patients had neurological examination and electroencephalography (EEG) complemented with magnetic resonance image (MRI) and single photon emission computed tomography (SPECT) in same cases. Among 83 patients we found the group of five (females) showing acute, stroke-like symptoms. Subarachnoid haemorrhage from a vascular malformation was diagnosed in one case. In four cases ischemical syndromes were observed mainly without strong clinical expression, with tendency to recede. In one patient the stroke was the first symptom of SLE. All these patients revealed abnormalities both in neurological examination and in neuroimaging techniques--as small, hyperintensive foci in MRI and multiple, diffused areas of decreased cerebral blood flow found in all but one cases in SPECT which seems to be the most sensitive method. In conclusions we want to stress that observed ischemic syndromes in patients with SLE were acute but of moderate intensity, with tendency to recede swiftly; transient ischemic attacks (TIA) were comparatively frequent. Acute stroke-like episodes might be the first symptom of SLE.

Adult↗
PubMed2004

Hypertensive encephalopathy mimicking brainstem tumour in psychiatric patient.

Hypertensive encephalopathy is a syndrome consisting of headache, seizures, visual changes, and other neurologic disturbances in patients with elevated systemic blood pressure. Diagnosis based on clinical and radiological findings, which are not specific, may be difficult to establish. Furthermore, hypertensive encephalopathy may develop gradually even when blood pressure is lower than that of malignant hypertension. We present clinical, magnetic resonance imaging (MRI) and autopsy findings in a 43-year-old schizophrenic patient with unrecognised hypertensive encephalopathy, which was misinterpreted by MRI as a diffusely growing brain stem tumour. Increased blood pressure was recorded several times, but it was not properly controlled and treated either during his out-door psychiatric examinations or hospitalisation. At autopsy, generalised atherosclerosis, concentric hypertrophy of the left ventricle and arteriolonephrosclerosis were found in addition to microvascular fibrinoid necroses and thromboses in the brain and kidneys, which were almost certainly caused by arterial hypertension evolving from benign into malignant stage. We discuss the differential diagnosis and give a review of the literature.

Adult↗
PubMed2005

[Neuropsychological and neuroimaging aspects of chronic cerebrovascular insufficiency in terms of structural and functional model of human brain].

Using clinical, neuropsychological and neurovisual methods, 70 patients with chronic cerebrovascular insufficiency (CI) were examined. Cognitive and emotional disorders in CI were categorized into 4 groups: mnemonic (48.6%), affective (21.4%), depressive (20%) and overvaluable ideas (10). In all groups, specific MRI characteristics of diffuse white matter lesions and cerebral atrophy were found but their expression and localization were different. A relation between brain neuropsychological and neurovisual changes was observed.

Adult↗
PubMed1992

[A case of chronic toluene intoxication with abnormal MRI findings: abnormal intensity areas in cerebral white matter, basal ganglia, internal capsule, brain stem and middle cerebellar peduncle].

We reported a case of 21 years old man of chronic toluene intoxication with abnormal intensity areas on MRI in cerebral white matter, basal ganglia, internal capsule (especially posterior limb), brain stem and middle cerebellar peduncle. The patient developed various neurological abnormalities such as blurred vision, ataxic speech, gaze evoked horizontal nystagmus, bilateral pyramidal tract sign and limb ataxia after 8 years sniffing of thinner (mainly toluene). MRI examination revealed diffuse high intensity areas in cerebral white matter on T1 weighted image. On T2 weighted image, high intensity areas of deep cerebral white matter, internal capsule (especially posterior limb), cerebral peduncle, ventral pons and middle cerebellar peduncle were noted. Basal ganglia (caudate nucleus, lenticular nucleus and thalamus) were displayed as low intensity area on T2 weighted image. These high intensity areas of internal capsule, brain stem and middle cerebellar peduncle on T2 weighted image would be significant for understanding pyramidal tract sign and cerebellar sign of this case. On the basis of neuropathological descriptions of chronic toluene intoxication, these high intensity areas of T2 weighted image were presumed to be demyelinating lesions of the central nervous system.

Adult↗
PubMed2005

Cerebral oligodendrogliomas in adults and children. Current data and perspectives.

Cerebral oligodendrogliomas represent more than 30% of glial tumors in adults. Mean age at diagnosis is 41 for grade A and 45(1/2) for grade B, epilepsy being the main revealing symptom (91.5% of A, 76% of B). Survival at 5, 10 and 15 years is respectively 75.5%, 51% and 22.4% for grade A (median: 136 months), and 45.2%, 31.3% and 0% for grade B (median: 52 months). It is influenced by age at diagnosis: median, before 40 years of age, is 12 years for A and 8(1/2) for B; between 40 and 60, is 12 years for A and 4(1/2) for B; over 60, is 4 years for A and 1(1/2) for B. In children, they represent less than 2.5% of cerebral tumors and include 23% grade A and 77% grade B (48.5% WHO grade II and 51.5% WHO grade III). Mean age at surgical removal is 9.5 +/- 5 years. One third are hemispheric, 2/3 are revealed by epilepsy. A complete surgical removal is performed in 60%. Mean survival is 13 years (154 +/- 20 months), with a mortality at 5 and 10 years of 60% +/- 9%, and a risk of recurrence of 54% +/- 9% at 5 years and 46.5% +/- 10.5% at 5 years. The main differential diagnosis of grade A oligo is dysembryoplastic neuroepithelial tumors (DNT). Inversely, thalamic locations, most often grade B, generally present with a motor deficit; complete removal can be achieved in only 15%. The only efficient treatment is chemotherapy, requiring search for chemosensitivity (1p19q deletion, expression of MGMT gene, analysis by MR-spectroscopy and TEP). Logically, surgical debulking should be as wide as possible, but with preservation of neurological and cognitive functions with the help of modern imaging progresses (fMRI, diffusion and perfusion MRI, tractography, PET) and operative techniques (navigation, cortical stimulation, and even opertaive MRI). Tumors presenting clinical and radiological progression should be removed. Operative mortality varies from 0 to 6%, transient morbidity from 20 to 25%, definit morbidity from 5 to 10%. Radiotherapy does not significantly modify survival whatever the delivered dose. The important point is the patient's radiosensitivity: survival is 148 months for radiosensitive patients versus 12 months for non responsive patients. Studies and development of pretherapeutic assays of tumoral radiosensitivity will be a fundamental research axis (PET, sample studies).

Adult↗
PubMedDate not supplied

Child abuse: acute water intoxication in a hyperactive child.

A 4-year-8-month-old boy was brought to our emergency department with coma and seizure. Initial physical examination showed evidence of physical child abuse and sudden body weight gain of 3.4 kg in one day. The laboratory results showed normal renal function with severe hyponatremia and the MRI study showed diffuse brain swelling. All of these findings were compatible with the diagnosis of acute water intoxication. Careful history taking from the boy and his parents separately confirmed the course of chronic polydipsia with acute compulsive water drinking. After clinical assessment and follow-up by psychiatrist, the patient was diagnosed with hyperactivity disorder. We present this case and show the possibility of correlation between compulsive water drinking, child abuse and hyperactivity disorder on acute water intoxication.

Acute Disease↗
PubMed2005

Hypertensive encephalopathy involving the brainstem and deep structures: a case report.

Hypertensive encephalopathy rarely presented with widespread edema in the cerebral white matters, deep structures and whole brainstem. A 80-year-old woman manifested as high arterial blood pressure, visual disturbance, severe headache, nausea, and vomiting. T2-weighted and fluid-attenuated inversion recovery magnetic resonance imaging showed high signal-intensity lesions in the cerebral white matter, cerebellum, basal ganglia, thalamus, and brainstem. Diffusion-weighted brain MRI did not show hyperintense signals in these lesions. These findings suggested the pathological basis of vasogenic edema. After control of hypertension, clinical symptoms and these edematous lesions on MRI gradually reduced.

Aged↗
PubMed2006

Diagnostic approach and management strategy of childhood stroke.

Prompt recognition and early intervention, with pertinent management and medication, may reduce subsequent neurologic deficits in stroke, which constitutes a devastating event in children. This is due to the tasking and demanding consequences including death or residual neurological deficits, which may last for many decades, in over 60% of survivors. Evidence-based treatment for children with stroke is still lacking, reflecting scarcity in baseline epidemiological data on pediatric stroke, the multitude of underlying risk factors, and the ethical and practical challenges incurred in conducting clinical trials. Based on the experience we gained from a combined prospective and retrospective study on childhood stroke (covering 10 years and 7 months and involving a cohort of 104 Saudi children), a diagnostic algorithm, which outlines the approach to a child with suspected stroke/cerebrovascular lesion, was designed. This algorithm might also be of use for managing other children with stroke from the Arabian Peninsula and Middle Eastern Region with similar demographic, socioeconomic, and ethnic backgrounds. Underlying risk factors, which need special attention, include thrombophilia and hypercoagulable states and sickle cell disease (SCD), which contrary to previous studies from Saudi Arabia, were found to constitute a common risk factor with severe manifestations. Other risk factors include infections (especially neurobrucellosis), cardiac diseases, and hypernatremic dehydration. Recognition of an identifiable syndrome or inherited metabolic cause may unravel an underlying cerebrovascular disease. This is particularly important in this region, given the large pool of autosomal recessive diseases and the high rate of consanguinity. In the evaluation of a suspected case of stroke, important imaging modalities include cranial CT, MRI (including diffusion-weighted images), magnetic resonance angiography (MRA), magnetic resonance venography (MRV) and conventional angiography. Transcranial Doppler sonography of the intracranial vessels and Duplex scan of the neck are valuable modalities for detecting large vessel vasculopathy, which occur in SCD, moyamoya syndrome, arterial dissection, and stenosis. Antithrombotic drugs are increasingly being used in the acute phase of childhood ischemic stroke. These include unfractionated heparin, low-molecular-weight heparins, aspirin or warfarin, or both. Specialized stroke care and follow-up are needed for children with stroke, as well as their families.

Algorithms↗