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MR imaging of shaken baby syndrome manifested as chronic subdural hematoma.

Shaken baby syndrome (SBS) is a form of child abuse that can cause significant head injuries, of which subdural hematoma (SDH) is the most common manifestation. We report the MRI findings of chronic SDH in three cases of SBS, involving two-, three- and eight-month-old babies. The SDH signal was mostly low on T1-weighted images and high on T2-weighted images, suggesting chronic SDH. In chronic SDH, a focal high signal on T1-weighted images was also noted, suggesting rebleeding. Contrast-enhanced MRI revealed diffuse dural enhancement.

Brain↗

[Young-adult-onset hereditary subcortical vascular dementia: cerebral autosomal recessive arteriosclerosis with subcortical infarcts and leukoencephalopathy (CARASIL)].

The clinical features of a probably autosomal recessive syndrome ("CARASIL"), yet to be confined in Japan and characterized by prematurity of vascular dementia, alopecia and spondylosis deformans are reviewed through comparison with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), which has been reported in Europe and North America, and recently in Japan. These two syndromes have many common features, such as familiality, encephalopathy of Binswanger type, and absence of vascular risk factors. There exists, however, a number of differences as follows: (1) Onset of encephalopathy is 32 years of age in "CARASIL" vs. 45 in CADASIL. (2) Male to female ratio is 3.2: 1 vs. 2:1.(3) Two thirds of "CARASIL" patients show stroke and/or stepwise deterioration, while almost all CADASIL patients have stroke. (4) Associated psychiatric features are euphoria, emotional lability and loss of spontaneity vs. severe mood disorders. (5) Migraine is a cardinal feature of CADASIL and vasospasm may occur during cerebral angiography. (6) White matter lesions on MRI are diffuse and homogeneous vs. punctuated and nodular. The latter four differences may mirror the difference in the pathology of arteriopathies. "CARASIL" is clearly different from CADASIL and reflect a second genetic condition with a seemingly direct effect upon the cerebral vasculature.

Age of Onset↗

[Therapeutic case of general paresis manifested by bradykinesia and recent memory loss].

We reported a 32-year-old man with general paresis. He showed slowly progressive bradykinesia and recent memory loss. Argyll Robertson pupils were not present. Muscle strength and sensations were normal except for slight vibratory disturbance. Tendon reflexes were slightly exaggerated. MMSE, HDS-R and WAIS-R scores showed the intellectual impairment. His laboratory investigations revealed elevated both TPHA and FTA-ABS titers in the serum and the CSF. The CSF contained leukocytosis (25/mm3) and protein 80 mg/dl. Cranial CT and MRI demonstrated diffuse cortical atrophy. SPECT revealed marked reduction of the blood flow in bilateral cerebral hemisphere. Cerebral angiography revealed moderate stenosis of the major vessels. The diagnosis of neurosyphilis (general paresis) was made and the treatment of intravenous benzyl penicillin potassium 24 million units per day was started. After 6 weeks of the treatment, the clinical signs (includes MMSE, HDS-R and WAIS-R scores) and the findings of SPECT and cerebral angiography showed improvement. Although the cell count and protein in the CSF became decreased, the titers of TPHA and FTA-ABS in the serum and the CSF were not decreased. Neurosyphilis should always be considered in a etiologically unknown case with bradykinesia and dementia.

Adult↗

[Leukoencephalopathy with swelling, megalencephalopathy and surprisingly mild course. A case report].

Leukoencephalopathy with swelling and a discrepantly mild clinical course is a recently identified syndrome described by van der Knaap et al. It is characterised by macrocephaly occurring during the first year of life, initially normal or nearly normal development, slowly progressive ataxia and spasticity with initial preservation of intellectual functions. MRI shows diffuse abnormality of signal intensity in the hemisphere white matter with subcortical cyst-like spaces in the fronto-parietal and anterior temporal areas. The case of a 5-year-old boy whose clinical and neuroimaging findings are consistent with this syndrome is reported. The clinical and neuroimaging findings of this case may provide further information about this new syndrome which could possibly be useful also for genetic counselling.

Brain↗

[A case of familial type IIa hypercholesterolemia with the clinical features similar to cerebrotendinous xanthomatosis].

A 63-years-old woman noticed unsteady gait at the age of 56 years and then developed dysarthria two years later. A general physical examination at age 56 revealed mild hypertrophy of both Achilles tendons. On neurological examination, she had scanning speech, moderate limb and truncal ataxia, and moderate hyperreflexia of all limbs. A soft tissue X-ray examination disclosed hypertrophy of both Achilles tendons with multiple punctate calcification. Brain MRI showed diffuse cerebellar atrophy. Motor evoked potentials in the right limb disclosed a prolonged central conduction time. Blood chemistry showed familial type IIa hypercholesterolemia (cholesterol 320 mg/dl, and LDL-cholesterol 245 mg/dl), yet cholestanol level was normal. A examination of CTX gene mutation at hot spots revealed no mutation. Her mother and two siblings also had hypertrophy of Achilles tendons as well as type IIa hypercholesterolemia. In addition, the one sibling showed mild ataxia of lower limbs, respectively. This report suggests a possible link between familial type IIa hypercholesterolemia and cerebellar degeneration syndrome clinically mimicking CTX.

Achilles Tendon↗

Corticobasal degeneration: an autopsy case clinically diagnosed as progressive supranuclear palsy.

We report an autopsy case diagnosed clinically as progressive supranuclear palsy (PSP), but neuropathologically confirmed as corticobasal degeneration (CBD). A 56-year-old Japanese woman slowly developed parkinsonism, dementia, character change, followed by vertical gaze palsy and dystonia. Brain MRI demonstrated diffuse cerebral atrophy with severe shrinkage of the brain stem tegmentum. The SPECT images using 123I-IMP disclosed symmetrical hypoperfusion in the frontal lobes. She died of respiratory failure at the age of 71. Gross inspection of the brain showed diffuse, symmetrical atrophy of the cerebrum and marked atrophy of the Luysian body, globus pallidus, substantia nigra and nuclei of the brain stem tegmentum. Microscopically, neuronal loss and fibrillary gliosis were observed in the Luysian body, globus pallidus, substantia nigra and nuclei of the brain stem tegmentum. The cerebellar dentate nucleus showed mild neuronal loss with some grumose degeneration. Neurofibrillary tangles were found only in the Luysian body, substantia nigra and raphe nuclei, whilst tau-positive inclusions were observed more extensively. Astrocytic plaques and swollen achromatic neurones were found in the postcentral gyrus. There were no tuft-shaped astrocytes in the brain. The clinicopathological similarities and differences between PSP and CBD are discussed.

Aged↗

[Diffusion-weighted magnetic resonance imaging of neuro-Behçet's disease].

We report 2 patients (35-year-old and 38-year-old men) with neuro-Behçet's disease and characterize their diffusion-weighted MRI (DWI) findings of brain. T2-weighted and fluid-attenuated inversion recovery images obtained during the acute phase of neurological manifestations demonstrated multiple hyperintense lesions with a characteristic distribution in the brain including brainstem, thalamus, basal ganglia, and cerebral hemisphere. Most of these lesions did not exhibit increased signal intensity on DWI. This MRI profile, especially the combination of isointensity on DWI and hyperintensity on T2-weighted image, of neuro-Behçet's disease is similar to that of multiple sclerosis, and different from that of acute cerebral infarction and herpes simplex encephalitis.

Acute-Phase Reaction↗

[A case of unilateral recurrent calf myalgia with muscle edema due to venous congestion].

We report a 38-year-old man who had three episodes of pain and swelling in the right calf from the age of 27 years. On each episode, pain worsened gradually, lasted for a few months and subsided spontaneously. Venography performed at the first episode was reported to be unremarkable. At the third episode, physical examinations showed tender and hard muscles in the right calf without findings of knee arthritis. The right calf pain became stronger when standing than when lying and on the ankle dorsiflexion. Laboratory examinations showed leukocytosis, a slightly elevated level of CRP, and normal CK levels. MRI showed diffuse high intensities in the right soleus and the lateral head of right gastrocnemius on T2-weighted images, where CT showed slightly low densities. To rule out focal myositis, the right soleus was biopsied. The muscle specimen showed perimysial and endomysial edema, dilatation of venules and capillaries, and focal mononuclear cell infiltration in the venule wall. Intermyofibrillar networks were well preserved. There were no necrotic or regenerating fibers, nor sarcolemmal expression of HLA-ABC. On electron microscopy, the processes of fibroblasts in the endomysium were swollen. This case illustrated muscle edema caused by local venous congestion possibly due to venulitis of unknown cause.

Adult↗

[A case of neuro-Behçet's disease with HLA B54 and predominant cerebral white matter lesions].

We report a 55-year-old woman with neuro-Behçet's disease with HLA B54 and predominant cerebral white matter lesions. She showed a cryptogenic high fever and cerebral cortical symptoms such as perseveration, limbkinetic apraxia and dementia. CSF study showed an increase of cell count and protein and a decrease of sugar. MRI showed diffuse T2-high signal intensity mainly in the subcortical white matter of left parieto-occipital lobes and basal ganglia. Her clinical signs greatly improved after administration of prednisolone. Her HLA type was not B51 but B54. Though our patient did not completely satisfy clinical criteria for neither neuro-Behçet's disease nor Sweet's syndrome, she showed partial features of both Behçet's disease and Sweet's syndrome.

Behcet Syndrome↗

[Familial orthochromatic leukodystrophy: clinicopathological study of two cases].

This paper reports the clinico-pathological data in a French family with orthochromatic leukodystrophy. The parents were first cousins and had seven children. Among those, two sisters and one brother presented with neurological signs, with onset around the 5(th) decade, including a dementing syndrome of frontal type, a tetrapyramidal syndrome, seizures, and, in one sibling, a cerebellar syndrome. CT scan or MRI showed diffuse involvement of the white matter. The neurological signs worsened progressively leading to death within 11 and 22 months. Neuropathological examination was performed in two cases. It revealed characteristic orthochromatic leukodystrophy. In one case, the presence of pigmented macrophages and astrocytes was suggestive of Van Bogaert and Nyssen disease. However there were some atypical features including the absence of pigmented cells in the second case whose clinical course was shorter, and the cavitary appearance of the white matter changes with a relative increase in the number of oligodendrocytes raising the issue of a possible link between this condition and cavitary orthochromatic leukodystrophies.

Aged↗

[Injuries of the cruciate ligament of the knee: diagnostic contribution of MR and spiral CT].

Lesions of cruciate ligament are common after knee trauma of different degree of gravity. Lesions evaluation is possible thanks to CT and MRI. Our purpose is to verify their value in diagnosis of cruciate ligaments lesions. We retrospectively evaluated 160 patients (80 CT and 80 MRI) with subsequent diagnosis of cruciate ligaments injury. CT and MRI were performed in the acute phase and in the subacute phase within 2-3 weeks from trauma. On the basis of our results CT and MRI shown a reduced sensitivity in acute phase owing to the intraarticular haemorrhage; in this phase MRI gives more information about fibres interruption. In subacute or chronic phase, both methods are correct in recognising the lesions but MRI is more sensitive and accurate. It is a real wish that, with more MRI machines diffusion, this method will be preferred in the study of cruciate ligaments both in acute phase and subacute-chronic phase.

Adolescent↗

[A case of subarachnoid hemorrhage associated with spontaneous intracranial hypotension].

Cerebrospinal fluid (CSF) leakage has recently been documented in most patients, as the main cause of spontaneous intracranial hypotension (SIH). No cases of SIH accompanying an episode of subarachnoid hemorrhage (SAH) has yet been reported and, to the best of our knowledge, this is the first report of a patient, who developed SAH during his clinical course of SIH. SAH was cured completely by surgical intervention. A 57-year-old man, with a history of a sustained postural headache, suffered the sudden onset of severe nuchal pain. SAH in the basal cistern, together with the finding of bilateral subdural fluid retention were verified by CT. Angiogram showed no vascular abnormalities of cerebral or cervical vessels. Gd-enhanced Magnetic Resonance Imaging (MRI) disclosed diffuse meningeal enhancement. RI cisternogram and CT-myelogram (CTM) suggested that the region of CSF leakage should be in the retrospinal area at the level of C1-2. Because orthostatic headache failed to resolve even with 3 weeks of bed rest, surgical intervention was successfully carried out to seal the site of CSF leakage with the use of a fascia and fibrin glue. We have speculated that the etiology of SAH might have been a secondary rupture of congestive intracranial veins, such as basilar plexus or bridging veins, induced by a decrease of intracranial CSF pressure.

Cerebral Veins↗

Sjogren - Larsson Syndrome: a case report.

Two male siblings aged 8 and 11 years, born of non-consanguineous parents, presented with classical triad of congenital ichthyosis, mental retardation and spastic diplegia. They had no eye, hair, nails and teeth changes. MRI showed diffuse dysmyelination.

Child↗

CSF-ACE activity in probable CNS neurosarcoidosis.

OBJECTIVE: To redefine the utility of CSF-ACE as a selective indicator of probable CNS neurosarcoidosis. METHODS: The diagnosis of probable CNS neurosarcoidosis required: (a) biopsy evidence of systemic sarcoidosis, (b) cortical, brainstem, and/or spinal cord deficits, (c) enhancing lesions on brain and/or spinal cord MRI, and (d) exclusion of other etiologies which could account for the neurological deficits. Radioassay measurement of CSF-ACE activity was performed in 11 patients who met our criteria for probable CNS neurosarcoidosis and 207 control patients. RESULTS: The M +/- SD for CSF-ACE activity was significantly higher (p < 0.05) for the 11 probable CNS neurosarcoidosis patients (9.5 +/- 6.9 nmol/mL/min) than for the control patients (2.9 +/- 2.7 nmol/mL/min). The optimal CSF-ACE activity discriminator value was 8 nmol/mL/min. At this value, the sensitivity and specificity of CSF-ACE activity was 55% and 94%, respectively. CONCLUSIONS: CSF-ACE activity is a useful biochemical marker of probable CNS neurosarcoidosis when brain and/or spinal cord MRI show diffuse enhancing lesions.

Adult↗

Update in the management of stroke.

Recent advances have improved the outlook for patients with stroke, Singapore's fourth leading cause of death. Stroke unit care reduces death, dependency and institutionalisation, independent of age, gender and stroke severity. Neuroimaging is essential prior to initiation of specific therapies. While computed tomography (CT) Head remains the most widely used modality, magnetic resonance imaging (MRI), particularly diffusion weighted imaging (DWI) has enhanced the positive diagnosis of ischaemic stroke. General medical measures include close monitoring, adequate oxygenation, avoidance of excessive blood pressure lowering, reduction of hyperthermia, control of hyperglycaemia, adequate nutrition, prevention of complications and early rehabilitation. Despite the risk of fatal intracranial haemorrhage, thrombolysis may improve outcomes in appropriately selected patients with ischaemic stroke. No safe and effective neuroprotectant has been found. While suboccipital craniectomy is established for large cerebellar infarcts and haemorrhage, surgical evacuation of supratentorial haemorrhage has not been shown to be beneficial. Hemicraniectomy reduces mortality after massive hemispheric ischemic stroke. Early and sustained antiplatelet use after atherothrombotic stroke reduces stroke recurrence. Stroke recurrence is also reduced by sustained warfarin use for cardioembolic stroke, carotid endarterectomy for severe symptomatic internal carotid artery stenosis, blood pressure lowering starting after the acute phase of stroke and lipid lowering. On-going clinical trails are likely to provide better treatments in the near future.

Aged↗

Experimental and human ischaemia: is the penumbra present in human ischaemic stroke?

Experimental occlusion of the middle cerebral artery and the reduction of cerebral blood flow (CBF) below 15 ml/100 gm/per minute cause failure of the somatosensory evoked response and lethargy of cortical neurones. Increase of CBF to normal value normalises the bioelectrical activity within the "penumbra"--the zone surrounding the necrotic area. In this "risk zone" neurones can survive only a few hours. So the penumbra is a "therapeutic window" in which therapy can save some neurones from death. However, if decrease of CBF is continued, numerous cells within the penumbra gradually die. The phenomenon of the penumbra, based on experiments, was applied to human ischaemic stroke; however great differences between experimental and human brain ischaemia do exist. The human course of disease and the size of necrotic lesion depend on numerous additional factors. The three very important critical values of CBF which decrease in human brain ischaemia are difficult to examine. New imaging methods play a great diagnostic role in searching for the penumbra, but their diagnostic possibilities and results are limited. In the short-time duration of the therapeutic window both CT and MRI often do not indicate ischaemic lesion. So, it is not known whether the penumbra is present or not. Conclusive estimation of MRI diffusion-weighted imaging and perfusion-imaging and of their coefficient is impossible to obtain in the time exceeding the "therapeutic window'" Numerous additional various diseases and compensatory vascular mechanisms influence the necrotic zone in human focal ischaemia. Probably for this reason a difficulty in the estimation of the penumbra in human is observed. So, it seems that the penumbra--the therapeutic window or risk zone--in human probably occurs only in some cases. It may be one of numerous reasons for the limited effectiveness of ischaemic stroke therapy. It is not known when the penumbra is overspread with oedema, which always appears to a greater or a lesser degree. Maybe the efforts of experimental investigators, physicians and pharmacologists should also be taken into consideration to find drugs decreasing oedema and BBB permeability.

Animals↗

[Why cerebral infarction is an emergency].

The three main criteria for a medical emergency: sudden onset, poor prognosis and possibility of an effective medical treatment apply to cerebral infarction which is the end result of a focal ischemia, occurring all the more rapidly than perfusion pressure is low. Studies devoted to experimental cerebral infarction as well as to thrombolysis in human have shown that the therapeutic window to save the penumbral tissue is very short, presently 3 hours in human for intra venous thrombolysis but with considerable individual variations best assessed by MRI diffusion-perfusion. Caring for the patient who has a cerebral infarction is an emergency because it is necessary, in the very first hours, to properly diagnose the infarct and its etiopathogenesis, to detect the vital manifestations to prevent the general complications, to start an antithrombotic treatment, to establish a vital and functional prognosis, to apply in due time the rare surgical or intra-arterial thrombolytic treatments and to start secondary prevention in patients with transient ischemic attacks.

Animals↗

[Image-guided epidural blood patch as effective treatment of intracranial hypotension. A case report].

We report the case of a patient with postural headache. A CT scan revealed bilateral subdural hygroma. Brain MRI showed diffuse pachymeningeal enhancement. A diagnosis of intracranial hypotension was therefore made. Thoracic cerebrospinal fluid leak was proved by radionuclide cisternography and contrast myelography. Conservative medical treatment was ineffective. Two thoracic epidural blood patches with radiographic control were made. We think the blood patch is the most important element for success.

Blood Patch, Epidural↗