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 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Several hereditary conditions affecting cerebral, retinal and systemic microvessels have recently been described. They include CADASIL, CRV, and HERNS. We here report on a variant form of a hereditary systemic angiopathy (HSA) affecting two generations of a Caucasian family. Clinical symptoms of HSA appear in the mid-forties and are characterized by visual impairment, migraine-like headache, skin rash, epileptic seizures, progressive motor paresis and cognitive decline. Late symptoms include hepatic and renal failure. Retinal capillary microaneurysms and arteriolar tortuosity are associated with marked optic disc atrophy. Radiological hallmarks consist of multiple cerebral calcifications and tumor-like subcortical white matter lesions. Brain, peripheral nerve, muscle, kidney and colon biopsies have revealed a multi organ small vessel involvement with partly altered endothelium, perivascular inflammation and thrombotic microangiopathy. No curative therapeutic options are known for hereditary cerebral vasculopathies. The use of cyclophosphamide, azathioprine and methotrexate was of no benefit in our cases of HSA. Early diagnosis of hereditary systemic angiopathies is important in order to prevent patients from repetitive invasive diagnostic measures and to avoid the use of inappropriate and potentially harmful drugs.
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PMID:Hereditary systemic angiopathy (HSA) with cerebral calcifications, retinopathy, progressive nephropathy, and hepatopathy. 1820 7

A 35-year-old woman, gravida 5 para 3, presented to the emergency department 9 days after a cesarean delivery with a new-onset headache, hypertension, and hyperreflexia. Formal urinalysis did not demonstrate proteinuria. Computed tomography of the brain demonstrated bilateral parietal subarachnoid hemorrhages. The patient was subsequently transferred to a tertiary care hospital where she underwent magnetic resonance imaging and computed tomographic angiography that were not suggestive of intracerebral aneurysm, arteriovenous malformation, sinus thrombosis, or angiopathy. The patient was treated with nimodipine and was successfully discharged without any neurologic sequelae or continued hypertension. This case illustrates the potential for presumed postpartum preeclampsia to mask other serious entities, such as intracranial hemorrhage.
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PMID:Postpartum intracranial hemorrhage disguised as preeclampsia. 1827 22

A 32-year-old woman developed a headache, seizures, and stupor on postpartum day 8. An initial diagnosis of possible encephalitis was made considering the presence of fever, neck stiffness, and abnormal CSF findings. MRI demonstrated hyperintense signals consistent with bilateral borderzone areas. MRA showed severe proximal narrowing of anterior, middle, and posterior cerebral arteries bilaterally. The patient recovered completely over 2 weeks, and repeated MRI and MRA scans were normal. Reversible vasoconstrictions have been known to occur during puerperium, and the clinical symptoms of our patient resembled such cases. Prior reports attributed these cases of postpartum angiopathy to capillary leakage and edema resulting in leukoencephalopathy. Our case suggests reversible borderzone ischemia as an additional pathological process.
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PMID:Postpartum angiopathy associated with reversible borderzone ischemia. 1827 36

Memory impairment, headaches and vertigo are considered as initial appearances of chronic cerebral vascular disorder in patients with arterial hypertension (AH). The complex analysis of complaints, cognitive functioning, emotional state and MRI data was conducted in 60 patients with AH, mean age 58,4+/-7,8 years, without a history of stroke and 30 controls matched for age, sex and education. Impairment of cognitive functioning was assessed by the Psychiatric Status Scale (a short version), the Clock Drawing Test, tests of auditory and verbal memory, attention concentration, speed of test performance, speech fluency and spatial orientation. The deterioration effect of systolic arterial pressure on cognitive functioning was found. The lesions of white matter (subcortical and/or periventricular leucoaraiosis) were observed in 76% of patients and single asymptomatic lacunar infarctions--in 20%. Cerebral vascular lesions were correlated with cognitive impairment. Anxiety and anxiety-depressive disorders which were not associated with the cerebral vascular lesion but related in large to the patient's complaints on headaches and vertigo were revealed in 62% of cases. The results of the study suggest that cognitive dysfunction proves to be the early and reliable predictor of chronic cerebral vascular disorder in patients with arterial hypertension.
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PMID:[Cognitive disturbances in patients with arterial hypertension]. 1845 93

The understanding of migraine pathophysiology has evolved from the belief that migraine is a vascular disorder, to evidence that better defines migraine as a neurogenic disorder associated with secondary changes in brain perfusion. There is evidence to suggest that the early phase of migraine pain results from neurogenic inflammation affecting cranial blood vessels and dura. Allodynia, hyperalgesia, and expansion of nociceptive fields occur during most well-established migraine attacks. These clinical features of migraine are evocative of those traditionally associated with neuropathic pain. A hypothesis that defines migraine pain as a unique neuropathic pain disorder can imply the potential for neural plasticity and may provide insight into the mechanisms that underlie the transformation of episodic to chronic forms of migraine. The neuropathic pain model of migraine pathophysiology not only paves the way for mechanism-based treatment strategies that can improve the acute and preventive management of migraine attacks, but also opens the door for the discovery of novel therapeutic targets. It also lends momentum to an understanding of clinically intriguing topics such as opiate-induced hyperalgesia and medication-overuse headache (rebound headache), opioid resistance in the treatment of chronic headache, and disease modification in defending against the potential for migraine transformation.
Curr Pain Headache Rep 2006 Jun
PMID:Is migraine a neuropathic pain syndrome? 1877 70

Postpartum cerebral angiopathy mostly occurs in the large or medium-sized cerebral arteries. In this case, we aimed to report a case of postpartum cerebral angiopathy presented as an asymmetrical penetrating arterial territory infarct with severe surrounding vasogenic edema. A 26-year-old woman admitted because of sudden headache after an attack of seizure. On initial computerized tomography (CT), hypodense lesion in the right basal ganglia was observed. The diffusion-weighted image on 5th day revealed focal acute ischemic infarction with surrounding extensive vasogenic edema in right basal ganglia. The CT angiography showed multifocal arterial narrowing of intracranial cerebral arteries that completely resolved on the follow-up study. This case suggested that asymmetrical small penetrating arterial territory infarct can occur as an atypical presentation of postpartum cerebral angiopathy.
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PMID:Atypical presentation of postpartum cerebral angiopathy shown as a small penetrating arterial territory infarct with severe peri-infarct edema. 1880 76

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatheromatous segmental angiopathy. The renal arteries are affected most commonly, followed by the internal carotid and vertebral arteries. FMD of the internal carotid and vertebral arteries usually occurs in the extracranial portions and is mostly observed at the level of the second cervical vertebra. FMD of the intracranial arteries is rare, but tends to occur in children and young adults. FMD is more common in females than in males, and it is often observed in middle-aged women. Although the etiology of FMD is not well understood, several mechanisms have been proposed, such as genetic predisposition, hormonal factors, and arterial wall ischemia. The pathology of FMD is characterized by smooth muscle hyperplasia or thinning, elastic fiber destruction, fibrous tissue proliferation, and arterial wall disorganization. Cerebrovascular fibromuscular dysplasia (cFMD) is relatively rare in Japan but is regarded as one of the cardinal causes of stroke in the younger population. cFMD without complications causes nonspecific symptoms such as headache or vertigo, but when it results in an arterial dissection or aneurysm, it leads to cerebral infarction or subarachnoid hemorrhage. Conventional angiographic findings mostly reveal a pattern called the "string of beads", which is pathologically correlated to medial fibromuscular dysplasia. Doppler echography, computed tomography and magnetic resonance angiography (MRA) may be useful for detecting cFMD lesions in some cases. MRA should be performed to rule out the presence of intracranial aneurysms. Antiplatelet and anticoagulation agents are prophylactics against cFMD complications. Surgical treatments such as graduated intraluminal dilatation had previously been the mainstays for treating cFMD. Percutaneous transluminal angioplasty with or without stenting has now become the preferred invasive treatment for symptomatic cFMD.
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PMID:[Cerebral infarction attributable to cerebrovascular fibromuscular dysplasia]. 1897

Thunderclap headache (TCH) is a neurological emergency that warrants immediate and comprehensive diagnostic determination. When no pathology can be identified the condition is classified as primary TCH, which is considered benign and self-limiting. TCH has also been reported as the initial symptom of reversible cerebral vasoconstriction syndrome (RCVS), which subsumes a variety of conditions, inconsistently coined Call-Flemming syndrome, benign angiopathy of the central nervous system, drug-induced arteritis, or migrainous vasospasm. Serious complications such as borderline ischaemic stroke have been reported. Although no standardized treatment regime exists, one commonly described but unproven therapy is parenteral or oral application of the calcium channel blocker nimodipine. Here, we report on a case of RCVS, where a progressive course prompted intra-arterial application of nimodipine, which resolved vasoconstriction immediately. We discuss the use of intra-arterial nimodipine application as a potential emergency treatment for a complicated or treatment-refractory course of RCVS.
Cephalalgia 2009 Jun
PMID:Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. 1923 77

Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion due to excessive endothelial proliferation. The two main features of this syndrome are livedo reticularis and lacunar subcortical infarcts. Here, we describe the case of a 64-year-old woman presenting with a 4-year history of a throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom. The pain, initially misdiagnosed as atypical trigeminal neuralgia, worsened up to chronic daily and such severely disabling headache that she was constrained to bed. She presented with reduced cognitive functions, diffuse and severe livedo reticularis, severe myalgias and mild stiffness. All diagnostic test for different diseases were performed and other diseases excluded except for Sneddon's syndrome. Her symptoms were reduced firstly using acetylsalicylic acid, then ticlopidine 250 mg bid was begun and then Pentoxyphillin, resulting in a significant improvement of symptoms with the disappearance of headache. Her worsening in the first year was characterized by obsessive-compulsive behaviours, body-image misperceptions and panic attacks, improved for a period using olanzapine. Considering this case, we remark the importance of using headache classification to avoid diagnostic errors, secondly, we describe an atypical manifestation of Sneddon's syndrome and therapeutic efficacy of using ticlopidine and pentoxyphillin.
J Headache Pain 2009 Jun
PMID:Sneddon's syndrome presenting with severe disabling bilateral headache. 1928 55

Benign angiopathy of central nervous system (BACNS) is rare in children. We report a seven-year-old boy presenting with sudden severe headache and progressive external ophthalmoplegia. Magnetic resonance angiography (MRA) showed diffuse segmental narrowing of major cerebral arteries. Following a course of glucocorticoid, there was complete resolution of vascular lesions and follow-up MRA did not show any evidence of new lesions.
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PMID:Benign angiopathy of central nervous system. 1963 64


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