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'Wine Glass' appearance: a unique MRI observation in a case of primary lateral sclerosis.

Magnetic resonance images (MRI) in a patient with primary lateral sclerosis is described. T2WI, MRI of brain showed characteristic hyperintensities of the corticospinal tracts bilaterally, extending from the internal capsule to the brain stem - producing a 'wine glass' appearance on coronal sections, which has not been described so far to the best of our knowledge. Differential diagnosis of this interesting 'wine glass' appearance on MRI along with a review of the MR-signal abnormalities in motor neuron disease is discussed.

Brain↗

[Neurological manifestations of Behçet's disease].

The neurological complications of Behçet's Disease are observed in 5 to 35 percent of cases depending of series. They included vascular and central nervous system manifestations. Vascular involvement is dominated by cerebral venous thrombosis marked by benign intracerebral hypertension. Prognosis is favourable with steroids and anticoagulation. Arterial involvement (thrombosis or aneurysm) are seldomly observed but increasingly with angio-MRI. Parenchymal involvement is dominated by meningoencephalitis eventually associated with fever. Myelitis and cranial nerve palsy are seldomly encountered. Cerebrospinal fluid is abnormal with lymphocytosis or pleiocytosis. MRI has a high sensitivity with hypersignals in the brainstem, basal ganglia, internal capsule and thalamus. Prognosis of central nervous system involvement is severe. Initially recovery is frequent, but severe impairment occurs due to relapse. In our experience, therapy including steroids, immunosuppressive drugs and colchicine improve short term and long term prognosis due to significant reduction of relapse in patients with good observance.

Anti-Inflammatory Agents↗

[A case of Turner syndrome complicated with brain infarction].

A 21 year-old female college student with a history of Turner syndrome at age 9, and 6-year growth hormone replacement therapy noticed weakness of right extremities when she got up on March 26, 2001. On admission, she showed right hemiparesis (4+/5) and hypesthesia on the right of body. The hemiparesis progressed (3-/5) in spite of antithrombotic therapy. Brain MRI revealed a high intensity lesion with a diameter of 1.5 cm in the posterior limb of the left internal capsule and putamen on DWI and T2WI. MR angiography and TC-CFI revealed no stenosis in her left middle cerebral artery, but > 50% stenosis in the horizontal portion (M1) of her right middle cerebral artery. Branch lesions were presumed to exist in the left M1. Non-atherosclerotic angiopathy, coagulopathy, and other conventional risk factors of brain infarction were not found. Pathogenesis of Turner syndrome might have played a role in the development of brain infarction in this patient.

Adult↗

[A case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI].

A 49-year-old woman, without any past history of liver diseases and blood transfusion, was admitted to our service because of somnolence, and flapping tremor. Neurologically, she was drowsy and disoriented. She had bilateral pyramidal tract signs and flapping tremor. Although the laboratory examination showed marked hyperammonemia (217 micrograms/dl), neither abdominal CT nor liver biopsy showed any evidence of liver cirrhosis. An abdominal angiography showed portal vein hypoplasia associated with the portal-systemic shunt. A T2-weighted MRI showed the high intensity areas in the bilateral deep cerebral white matter, and the posterior limbs of the bilateral internal capsules. This is a rare case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI.

Female↗

Practical visualization of internal structure of white matter for image interpretation: staining a spin-echo T2-weighted image with three echo-planar diffusion-weighted images.

BACKGROUND AND PURPOSE: To our knowledge, no method satisfactory for clinical use has been developed to visualize white matter fiber tracts with diffusion-weighted MR imaging. The purpose of this study was to determine whether superposition of a spin-echo T2-weighted image and a color-coded image derived from three orthogonal diffusion-weighted images could show fiber tract architecture of the brain with an image quality appropriate for accurate reading with a computer monitor. METHODS: MR images from 50 consecutive cases were reviewed. Three diffusion-weighted images per section were acquired with three orthogonal motion-probing gradients. These images were registered to a corresponding spin-echo T2-weighted image. A color-coded image was synthesized from three diffusion-weighted images by assigning red, green, or blue to each diffusion-weighted image and then adding a spin-echo T2-weighted image with a weighting factor. The ability of the superposed image to delineate the white matter pathways was evaluated on the basis of the known anatomy of these pathways and qualitatively compared with that of the spin-echo T2-weighted image. RESULTS: The main white matter fiber pathways, in particular the superior longitudinal fascicle, corpus callosum, tapetum, optic radiation, and internal capsule, were more clearly and easily identified on the superposed image than on the spin-echo T2-weighted image. The time required to produce the superposed image was approximately 40 minutes. CONCLUSION: Superposition of a spin-echo T2-weighted image and a color-coded image created from three orthogonal diffusion-weighted images showed structures of the brain that were not clearly visible on the spin-echo T2-weighted image alone. Such superposition presents images that are easy to interpret correctly.

Adolescent↗

[A case of idiopathic portal hypertension (IPH) with hypermanganemia presenting as spastic gait].

A 48-year-old women was admitted to our hospital because of gradually developed spastic gait. She showed spasticity of the lower extremities with mild weakness. Laboratory tests disclosed decreased WBC and platelet counts and mild increases of transaminase and total bilirubin. Blood manganese level was markedly increased (6.0 micrograms/dl). Abdominal ultrasound showed splenomegaly, and abdominal angiography showed a dilatation of the portal and paraumbilical veins. T1-weighted MR images showed high signal intensities at the bilateral globus pallidus and cerebral peducles, and T2-weighted images showed high signal intensities at the bilateral deep white matter, posterior limbs of the internal capsule and right upper cervical spinal cord. Following the diagnosis of IPH, splenectomy was performed. The blood level of manganese decreased thereafter and her neurological deficits gradually improved. Hepatic diseases often show high signal intensities at the basal ganglia on T1-weighted images, and this seemed to be due to accumulation of manganese in our case. Because demyelination or axonal injury of the spinal cord are found in hepatic disease, we speculate that the high signal intensities at the spinal cord on T2-weighted images of our case reflect hepatic myelopathy, which may also be caused by high blood levels of manganese.

Brain↗

[Stereotactic microelectrode-guided posteroventral pallidotomy for Parkinson's disease].

OBJECTIVE: To assess postoperative effects of microelectrode-guided posteroventral pallidotomy (PVP) for Parkinson's disease. METHODS: Intraoperative microelectrode recordings and microstimulation were used to explore the globus pallidus to performance of posteroventral pallidotomy in 48 patients with Parkinson's disease (47 unilateral and 1 bilateral). Assessment was made at baseline preoperatively and at 6 months intervals postoperatively, with unified Parkinson's disease rating scale (UPDRS). RESULTS: All patients were significantly improved on the limbs contralateral to the lesion side 6 - 34 months after operation (mean 24 months). The improvement was seen in the 'on' or 'off' state: UPDRS scores with patients on levodopa were improved by an average of 28.7%, while off medication scores showed reductions (47.6%) at 24 months. There were no deaths and no visual complications, but there were 4 patients (8.3%) of a delayed contralateral limbs dystonia after pallidotomy. CONCLUSIONS: The techniques of microelectrode recording and microstimulation indicate the location of the internal capsule and optic tract, which allow easy identification of these structure and facilitate PVP target in conjunction with radiofrequency microelectrode stimulation.

Adult↗

Mini-infarct encephalopathy associated with uncommon microvessel convolute formation presenting with presenile dementia.

A female patient started to suffer from transient ischemic attacks when she was 47 years of age, followed by increasing predominantly left-side spastic tetraparesis, generalized seizures and progressive dementia over a period of 11 years. She died when she was 58 years of age. On gross examination the brain showed enlarged ventricles and arteriosclerotic changes of large extracerebral vessels of the circulus arteriosus. Microscopic examination of the atrophic brain showed innumerable incomplete microinfarcts in the white and gray matter throughout all parts of the brain. In the white matter these lesions were characterized by small foci of demyelination and loss of oligodendrocytes while occasionally some scavenger cells were seen. Axons seemed to be unaffected or displayed irregular axonal regeneratory growth. Any inflammatory reaction failed. In the cerebral cortex and subcortical nuclei the lesions showed loss of neurons and decrease in synaptophysin expression. Intracerebral arteries showed fibrosis or fibrohyalinosis of the entire intracerebral small-vessel network. In addition, numerous uncommon clusters of angioma-like telangiectatic vessels were observed. Medium-sized ischemic infarcts were found in the right putamen and adjacent internal capsule region, left-side dorsolateral brain stem and cerebellar hemisphere as well as a left-side pyramidal tract degeneration. Contralateral pseudohypertrophy of the inferior olivary nucleus was seen. The clinical and the neuropathologic observations made in this patient are compatible with small vessel disease characterized by a multicentric special and not yet described type of incomplete mini-infarcts in cerebral cortex and white matter accompanied by some larger ischemic infarcts of the common type in brain stem and cerebellum.

Alzheimer Disease↗

[A case of postural and kinetic tremor caused by diffuse axonal injury].

We reported a 25-year-old woman with postural and kinetic tremor caused by diffuse axonal injury. The patient demonstrated consciousness disturbance, left oculomotor palsy and tetraparesis because of an automobile accident. T2-weighted and FLAIR MRI showed features of diffuse axonal injury. Hyperintense lesions appeared in the corpus callosum, fornix, dorsal portion of midbrain, right cerebral peduncle, and bilateral internal capsules. About 3 weeks later, head tremor and left hemiparesis appeared with improvement of consciousness. Administration of trihexyphenidyl decreased the tremor. Ten weeks after the accident, a coarse tremor in the head and right upper extremity developed after withdrawal of trihexyphenidyl. Tremor in the right upper limb predominantly occurred while maintaining an upright posture and with intended movements. Re-administration of trihexyphenidyl decreased the tremors. The dentatothalamic pathway is one of the lesions responsible for posttraumatic tremor. Our patient demonstrated lesions of diffuse axonal injury involving the dentatothalamic pathway. We considered that these lesions were associated with postural and kinetic tremor in our case. The tremor occurred at least 3 weeks after the accident. This finding suggested that the tremor was caused by transsynaptic alternations of thalamus or the extrapyramidal system secondary to involvement of the dentatothalamic pathway.

Accidents, Traffic↗

[Merosin-positive congenital muscular dystrophy, white matter abnormalities, and bilateral posterior occipital cortical dysplasia].

Congenital muscular dystrophy (CMD) is one of the most frequent dystrophies of childhood, which is commonly characterized by neonatal muscle impairment with or without clinical evidence of central nervous system involvement. CMDs were classified into five clinically distinct forms: the two classical CMDs with and without deficit of the a2 laminin chain (merosin) caused by mutations on chromosome 6q2, the Fukuyama CMD (severe form, initially described in Japanese patients and recently linked to the chromosome 9q31-33), Walker-Warburg syndrome and the muscle-eye-brain disease described in Finnish patients. The majority of these forms have severe clinical and imagiological involvement of SNC. This aspect is rarely observed on classical CMD, particularly in the merosin-positive form. We describe a case of a 28 year-old woman, with clinical and histopathological signs of classical CMD merosin-positive (no deficient), without mental retardation, but with epilepsy. MRI T2 weighted images, revealed diffuse and symetrical high signal white matter of both cerebral hemispheres, affecting corpos calosum, posterior arms of internal capsules and the piramidal tract to mesencephalon. It also disclosed diffuse and symetrical high signal of basal ganglia, specially, the head of caudate nuclei. These were associated with bilateral occipital posterior cortical dysplasia. The observed imagiological pattern could represent a new subtype of CMD, hybrid between classical CMD and the severe forms, however it is not clear where it fits in the spectrum. This case denotes the possible envolvement of SNC in patients merosin-positives. Based on this findings we suggest doing MRI scans to all patients with CMD no deficient in merosin.

Adult↗

[Amnesia, confabulation and nonaphasic misnaming after left thalamic infarct].

A 72-year-old right handed woman developed amnesia, confabulation and abnormal (bizarre) verbal response after the left thalamic infarction. Clinical features including disorientation, euphoria and various kinds of paraphasia coincided in nonaphasic misnaming. MR images showed that lesions involved the genu of the internal capsule, the anteroventral nucleus, the lateroventral nucleus, intralaminar nuclei, the mamillothalamic tract and the region around the ventral thalamus. 99mTc-HMPAO-SPECT revealed decreased uptake in the left frontal lobe, probably due to the disconnection from the thalamus. These findings suggest that the dysfunction of the thalamus (mainly ventrolateral) and the left frontal lobe caused the disturbance of the self-monitoring in the language use, which generated confabulation and nonaphasic misnaming in our case.

Aged↗

MR imaging findings in African trypansomiasis.

We report the initial and follow-up brain findings in a 42-year-old male patient with CNS involvement with African trypansomiasis. Initial MR imaging demonstrated diffuse hyperintensity in the basal ganglia bilaterally as well as involvement of the internal capsule, external capsule, and extreme capsule. Follow-up examination at 1 year revealed decreased signal intensity in the previously affected areas; however, ventricular enlargement indicative of atrophy was readily apparent.

Adult↗

[Three cases of non-differentiated leukodystrophy with involvement of cerebral hemisphere's white matter and cerebellum and specific lesions of cerebral pathways. Neuroimaging and clinico-biochemical characteristics].

We presented three cases of the non-differentiated White Matter's disease. There are clinical resemblance and the similar MRI patterns including MR spectroscopy in all patients. They are outpatients: girl (K), 7 years old, boy (N), 13 yrs and young men (N), 24 yrs without any family relationship, and were investigated in Burdenko Neurosurgical Institute, in 2002 year. The cerebral MRI examinations all of that patients have shown extensive abnormalities in the hemispheric's white matter of cerebrum and cerebellum. Meanwhile there are some specific lesions of the brain stem's tracts (the posterior limb of the internal capsule, the splenium of the corpus callosum, the medial lemniscus and the mesencephalic trigeminal tracts). Step by step progression of suffer ness with unknown time of beginning, mild mnestic reduction and hemyparesis and moderate cerebellar disorder without loss of sensitivity were characteristic for all patients. The results of the biochemical markers for leucodistrophy testing are negative. A proton spectrum of the abnormal white matter showed elevated lactate in one case (girl, 7 yrs). In our opinion clinical diagnosis in all three cases is the new pathology that some body named as "WMDL" (White matter disease with lactate). It should be differentiated with adrenoleucodistrophy, Refsum disease and NARP syndrome (neuropathy, ataxia, pigmentive retinitis).

Adolescent↗

[Carbon dioxide laser and stereotaxic craniotomy].

This article describes the development and contemporary clinical applications for a computer interactive volumetric stereotactic system in CT and/or MRI-based resection of superficial and deep seated intracranial lesions. The carbon dioxide laser was found to be particularly useful in the resection of deep seated lesions by this volumetric stereotactic method for three reasons: 1) the CO2 laser is a convenient tool for removing tissue from the depths of a deep cavity, 2) it is relatively hemostatic, and 3) the precision provided by the CO2 laser renders it safer than other methods for dissecting tumors from important brain tissue, e.g. the internal capsule. The specific clinical methods of the technique: data acquisition, computer based surgical planning, and interactive stereotactic open surgery are discussed. The clinical experience in 500 consecutive cases in the resection of various lesions from specific anatomical areas is presented. Total overall morbidity was 7% and mortality 1%. We have found the technique of most benefit in the resection of histologically circumscribed intra-axial lesions.

Adolescent↗

Elevated expression of type II Na+ channels in hypomyelinated axons of shiverer mouse brain.

Type I and type III Na+ channels are localized mainly in neuronal cell bodies in mouse brain. Type II channels are preferentially localized in unmyelinated fiber tracts but are not detectable in normally myelinated fibers. In shiverer mice, which lack compact myelin due to a defect in the myelin basic protein gene, elevated expression of type II Na+ channels was observed in the hypomyelinated axons of large-caliber fiber tracts such as the corpus callosum, internal capsule, fimbria, fornix, corpus medullare of the cerebellum, and nigrostriatal pathway by immunocytochemical analysis with subtype-specific antibodies. No difference was observed in the localization of type I and type III Na+ channels between wild-type and shiverer mice. These findings support the hypothesis that type II Na+ channels are preferentially localized in axons of brain neurons and suggest that their density and localization are regulated by myelination. The selective increase in the number of type II channels in hypomyelinated fiber tracts may contribute to the hyperexcitable phenotype of the shiverer mouse.

Animals↗

[Detection of the myelination process of Chinese infants by 1.0T MRI].

To establish the myelination process of brain of Chinese infants, our study consisted of seventy-five infants ranging in age from 5 days to 2 years old. They were studied with a 1.0 T superconductive MRI. In the new born, myelination could be seen in the brainstem, middle cerebellar peduncle, and posterior limb of the internal capsule in both T1WI and T2WI. Then the myelination process rapidly progressed month by month. Myelination of the centrum semiovale and optic radiation could be seen in T1WI in the newborn up to 3 months old, and in T2WI at 3-5 months old. The myelination of the splenium and the genu of corpus callosum were seen at 3-6 months in T1WI and 5-6 months in T2WI. Myelination of the occipital lobe and frontal lobe were seen at 3-6 months and 5-10 months in T1WI, and 8-14 months, 10-16 months in T2WI respectively. In 7-10 months, the brain appeared as an "early adult pattern" in T1WI; while, in T2WI, the brain appeared as an "early adult pattern" at 14-18 months old. The understanding of the myelination process in infants is important and essential for the judgement of the infantile brain and for the diagnosis of many CNS diseases of infants.

Age Factors↗

[Gadolinium-MRI findings of two adrenoleukodystrophy cases treated with gamma-globulin].

Two cases of adrenoleukodystrophy were reported. We treated them with intravenous gamma-globulin infusion. MRI with gadolinium enhancement was performed before and after the therapy to evaluate the effect of gamma-globulin. Case 1 was a 19-year-old boy, who noticed left hemianopsia when he was 18 years old. Case 2 was a 36-year-old man, who developed ataxic gait and character change when he was 33 years old. Very long chain fatty acids in plasma and RBC membrane were elevated in both cases. On T2-weighted image of MRI, high signal lesions were seen in the white matter of occipital lobe in case 1, cerebellum, internal capsule, and around the corpus callosum in case 2. On T1-weighted image, the rim of every lesion was enhanced by gadolinium infusion. After the gamma-globulin therapy, gadolinium enhancement of the rim remarkably reduced in case 1. No reduction was observed in case 2. MRI findings of case 1 indicate the possibility that gamma-globulin may suppress the inflammation of the adrenoleukodystrophy.

Adrenoleukodystrophy↗

[Unilateral persistent hyperhidrosis after ischemic stroke].

A 64-year-old right hemiplegic woman, who had been treated for hypertension for 15 years, was admitted to our hospital. Neurologic examination on admission disclosed right hemiplegia and motor aphasia; however, ophthalmoparesis, pupillary abnormality, and blepharoptosis were not evident. Excessive sweating on the right side of the body, which was most marked on the face, was observed. Amount of sweating on the left side of the body was normal. Unilateral hyperhidrosis persisted for more than 2 months. MRI revealed hemorrhagic infarctions in the left basal ganglia, internal capsule, thalamus, hypothalamus, and medial part of the cerebral peduncle. 123I-IMP SPECT disclosed hypoperfusion in the left striatum, thalamus, occipital cortex, and right cerebellar hemisphere. Cerebral angiography revealed arteriosclerotic changes in the basilar artery, but that the left posterior cerebral artery and its branches were not occluded. Unilateral persistent hyperhidrosis is rare after ischemic stroke. Hypothalamic lesion was thought to be responsible for the hyperhidrosis in this patient. As the hypothalamus receives its blood supply from the posterior cerebral artery, unilateral persistent hyperhidrosis may be an important sign of cerebral infarction in the posterior cerebral artery region.

Cerebral Infarction↗