Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Localized scleroderma in children includes morphea mainly on the trunk and linear scleroderma on the limbs, scalp, and face. Progressive systemic sclerosis is very rare in children and change from localized to progressive disease is extremely rare. Laboratory abnormalities occur with localized scleroderma, including eosinophilia, positive antinuclear factor (ANF), and increased immunoglobulin (Ig)G. The diseases are usually self limited, but involvement over bones may lead to marked functional impairment. Those on the face may be associated with underlying abnormalities and, occasionally, seizures. Treatment regimens are difficult to assess as there is no chemical marker, but penicillamine has gained wide use. It has numerous side effects and patients should be carefully monitored, particularly with regard to renal function. Other modalities have been used with variable success, but series of patients are small. Many medications produce skin softening in patients with progressive systemic sclerosis. The pathogenesis of skin sclerosis involves complicated interactions between vascular responses, lymphokines, and connective tissue proliferation. The etiology is completely unknown, despite a few cases associated with increased Borrelia titers.
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PMID:Localized cutaneous scleroderma. 155 Jul 17

A patient with localized scleroderma of the head, uveitis, and Raynaud's phenomenon presented with generalized seizures, spastic hemiparesis, and local IgG production in the cerebrospinal fluid. Magnetic resonance imaging revealed progressive cortical and subcortical brain parenchymal lesions mainly adjacent to the cutaneous and bony lesions and probably of inflammatory origin.
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PMID:Progressive inflammatory lesions of the brain parenchyma in localized scleroderma of the head. 227 74

We report two cases of gradual facial hemiatrophy (Parry-Romberg syndrome). The first patient, an adolescent girl under treatment for hypothyroidism, presented with a very severe form combining advanced facial hemiatrophy, epilepsy with hemi-generalized seizures and hemiatrophy of the brain. The second patient was a girl who presented with localized scleroderma resembling a saber injury, homolateral cerebral atrophy and contralateral hemiparesis. Our two cases are evidence in support of a close relationship between saber injury-like scleroderma and the Parry-Romberg syndrome.
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PMID:["Saber-cut" scleroderma and Parry-Romberg facial hemiatrophy. Nosologic problems. Neurologic complications]. 293 Jan 26

Morphea, a specific type of cutaneous sclerosis, is known in the pediatric age group, but not as an adverse effect to the antiepileptic valproic acid. We report a 14-year-old male patient with absence seizures who, after treatment with valproic acid, developed skin tightness on the fingers. A biopsy specimen revealed dermal sclerosis consistent with morphea. His symptoms resolved gradually after discontinuation of the medication. To our knowledge, this is the first patient reported to have an apparently valproic acid-induced localized morphea.
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PMID:Localized morphea: a rare adverse effect of valproic acid. 1462 13

We report a 7-year-old boy who unexpectedly developed a multi-drug resistant epilepsy with negative neuroimaging results, followed by the insidious appearance of linear localized scleroderma involving the right leg. When the boy was 16 and severely affected by epileptic encephalopathy, we have evaluated this case for the first time: his localized scleroderma had reached the right buttock and positive anti-nuclear antibody was the only positive laboratory test. Methotrexate administered for 12 months was ineffective in improving both the organization of his electroencephalographic pattern and seizure control, though seemed to stabilize the progression of linear scleroderma. This report suggests that neurological abnormality and extracranial scleroderma might represent two own distinct processes in a same patient.
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PMID:Longstanding epileptic encephalopathy and linear localized scleroderma: two distinct pathologic processes in an adolescent. 1827 99

We describe a young woman with localized scleroderma, seizures, numerous persistently enhancing white matter lesions on brain MRI, and oligoclonal bands in the CSF. The case is remarkable in the widespread bilateral distribution of the lesions and their enhancement during more than a year of follow-up despite immunosuppression. Literature search yielded 54 case descriptions of localized scleroderma associated with neurologic symptoms and neuroimaging findings. All patients had craniofacial scleroderma: linear scleroderma en coup de sabre (LScs), progressive facial hemiatrophy (PFH, or Parry-Romberg syndrome) or both. LScs and PFH should be viewed as variants of craniofacial localized scleroderma as they often manifest in the same patient, share the same neurologic manifestations and imaging features, and evidence pathologic inflammation in skin and CNS.
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PMID:Neurologic manifestations of localized scleroderma: a case report and literature review. 1898 76

Juvenile localized scleroderma (JLS) includes several subtypes including plaque morphea, linear scleroderma and the en coup de sabre type which affects face and head. The latter variety may involve the eye and the brain with various appearance and clinical complications.We describe the case of a 6-year-old boy who presented partial complex seizures, with status epilepticus, four months before the appearance of sclerodermatous skin lesions on the face. This case report raises important questions on the pathogenesis of JLS and, particularly, on the issue whether it is a mere autoimmune condition or a neuro-cutaneous disease.
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PMID:Severe epilepsy preceding by four months the onset of scleroderma en coup de sabre. 1979 65

Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The "en coup de sabre" subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we report one patient with typical linear scleroderma "en coup de sabre" (LSCS). Initially, he presented with refractory partial seizures before the characteristic skins lesion on his head developed. This was a rare case with obvious brain parenchyma involvement. We did not prescribe medication but performed serial brain magnetic resonance imaging follow-up for the intraparenchymal lesion. The atrophic changes of the skin, face and brain remained the same, and his seizures had not worsened at the most recent follow-up. Parry-Romberg syndrome, a very similar condition, should be differentiated from LSCS.
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PMID:Linear scleroderma "en coup de sabre": initial presentation as intractable partial seizures in a child. 2002 45

Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs.
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PMID:Neurologic involvement in scleroderma en coup de sabre. 2231 46

Linear scleroderma "en coup de sabre" (LSCS) is a form of localized scleroderma presents as band-like sclerotic lesions of the frontoparietal area. It has been reported in association with diverse neurological manifestations like seizures, migraine, neuromyotonia, dystonia and abnormalities in MRI and CT studies as cerebral atrophy, white matter lesions, intraparenchymal calcification, meningeocortical alterations, and skull atrophy. We describe a patient with LSCS associated with two abnormal movements: permanent myokimia of right masseter muscle and recurrent spasmodic retraction of right cigomatic and depressor labii inferioris muscles. He was initially treated with methotrexate and steroids without response, so later on he underwent repeated Botox injections with remarkable improvement.
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PMID:Successful treatment of hemifacial myokymia and dystonia associated to linear scleroderma "en coup de sabre" with repeated botox injections. 2292 48


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