Sneddon’s syndrome presenting with severe disabling bilateral headache
© Springer-Verlag 2009
Received: 7 December 2008
Accepted: 4 February 2009
Published: 14 March 2009
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.
Sneddon’s syndrome is a rare, progressive, non-inflammatory vasculopathy affecting the small- and medium-size arteries of the brain and skin, leading to their occlusion due to excessive endothelial proliferation. The two main features of this syndrome are livedo reticularis and lacunar subcortical infarcts. Other frequent symptoms are dementia, heart valvulopathy, hypertension and renal failure [1, 2].
Here we describe the case of a 64-year-old woman presenting with a 4-year history of a de-novo throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom (phono-, photo-phobias, nausea nor vomiting); headache worsened in ortho-statism and improved in clinostatism. The pain, initially misdiagnosed as atypical trigeminal neuralgia, worsened up to chronic daily and such severely disabling headache that she was constrained to bed. Several drugs had been used in the previous 4 years, without any effect (benzodiazepine, Ca-antagonist, central smooth-relaxant, tricyclic antidepressants, antiepileptic drugs, SSRI); she treated pain daily with non-steroidal anti-inflammatory drugs or tramadol, without pain improvement.
Two main observations could be inferred from this case. First, we remark the importance of using headache classification to avoid diagnostic errors. Second, we describe an atypical manifestation of Sneddon’s syndrome. Headache disorders are widely described in the last international classification . This classification has mainly been conceived to avoid errors and misdiagnoses. In this case the patient has not been properly treated for a 4-year period and an atypical bilateral trigeminal neuralgia was diagnosed. This is an example of how it is important to sift through a patient’s signs and symptoms when diagnostic criteria are not met in order to hypothesise a different disorder and modify treatment accordingly. A few papers tried to evaluate a possible association between migraine and Sneddon’s syndrome, but available literature is not conclusive . Headache has never been described as the main symptom, but has only been cited as an accompanying one . This case stands out for the atypical presentation with severe, persistent, very long-lasting, disabling headache, long time before other neurological symptoms and signs manifested. Mechanism of headache pathogenesis in patients with antiphospholipid antibodies or other coagulative disorders is supposed to be “vascular” but it is less clear when such antibodies are absent . Classic pathologic findings are thrombosis and recanalization in medium-small vessels and meningeal vessels, both in patients with and without antiphospholipid antibodies . Sneddon’s syndrome is usually treated with antiplatelet or anticoagulant therapy, depending mainly on the presence or absence of antiphospholipid antibodies. A recent review does not show any significant difference in the prognosis of the patients following the two treatments . In our case antiplatelet therapy was crucial and was associated with a durable pain-free period. Unfortunately, Sneddon’s syndrome is a progressive disease and no causative therapy is yet known.
Conflict of interest
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