Hemiplegic migraine and progressive hemifacial atrophy

Sagild, J.C.; Alving, Jørgen

doi:10.1002/ana.410170621

Cited by 25 publications

(14 citation statements)

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“…Ocular involvement seen in 10-35%, the most frequent manifestation is the enophthalmos, due to fat loss around the orbit, was not observed in our patient (5). The neurological complications seen in 45%, such as trigeminal neuralgia, facial paresthesia, severe headache and contra- lateral epilepsy can also be present, but were not diagnosed in our case (11). Differential diagnosis of localised scleroderma, hemifacial microsomia, Bell’s palsy, unilateral ankylosis, hemifacial hypertrophy and trauma or burn scar can be considered.…”

Section: Discussionmentioning

confidence: 55%

Parry Romberg Syndrome with localized scleroderma: a case report

Khan¹,

Negi

Gupta

2014

J Clin Exp Dent

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Parry Romberg syndrome(PRS) is a rare acquired poorly understood neurocutaneous syndrome of unknown etiology characterized by slow progressive atrophic changes commonly affecting one half of the face. The exact incidence and etiology towards the syndrome remains unclear. Apart from the multifactorial etiology proposed, the possible primary cause is mainly attributed to the cerebral disturbance of the fat metabolism. The syndrome overlaps with “en coup de sabre” morphea, with an ill defined relationship existing between the two. Parry Romberg Syndrome is an invalidating lesion that may be associated with different neurological, cutaneous, ocular, dental and autoimmune abnormalities. This report presents one rare case of 22 years old female patient with Parry Romberg syndrome associated with localized scleroderma, accompanied by a brief review of literature with classical clinical, radiographic, histological findings and the treatment of progressive hemifacial atrophy. Key words:Parry Romberg syndrome, progressive facial hemiatrophy, morphea, localized scleroderma.

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Section: Discussionmentioning

confidence: 55%

Parry Romberg Syndrome with localized scleroderma: a case report

Khan¹,

Negi

Gupta

2014

J Clin Exp Dent

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“…Third, it is not clear if patients with DMD have consistent gross or histological abnormalities of the brain. Available studies show conflicting results, from no change in brain weight or gross histology [20;77;78] to marked brain abnormalities [79–82], including ventricular dilation [18;83;84]. Fourth, we have a relatively small sample size for all the experiments and the corresponding histological sections were not quantified.…”

Section: Discussionmentioning

confidence: 99%

Abnormalities in brain structure and biochemistry associated with mdx mice measured by in vivo MRI and high resolution localized 1H MRS

Shi

Pratt

et al. 2015

Neuromuscular Disorders

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Duchenne muscular dystrophy (DMD), an X-linked disorder caused by the lack of dystrophin, is characterized by the progressive wasting of skeletal muscles. To date, what is known about dystrophin function is derived from studies of dystrophin-deficient animals, with the most common model being the mdx mouse. Most studies on patients with DMD and in mdx mice have focused on skeletal muscle and the development of therapies to reverse, or at least slow, the severe muscle wasting and progressive degeneration. However, dystrophin is also expressed in the CNS. Both mdx mice and patients with DMD can have cognitive and behavioral changes, but studies in the dystrophic brain are limited. We examined the brain structure and metabolites of mature wild type (WT) and mdx mice using magnetic resonance imaging and spectroscopy (MRI/MRS). Both structural and metabolic alterations were observed in the mdx brain. Enlarged lateral ventricles were detected in mdx mice when compared to WT. Diffusion tensor imaging revealed elevations in diffusion diffusivities in the prefrontal cortex and a reduction of fractional anisotropy in the hippocampus. Metabolic changes included elevations in phosphocholine and glutathione, and a reduction in γ-aminobutyric acid in the hippocampus. In addition, an elevation in taurine was observed in the prefrontal cortex. Such findings indicate a regional structural change, altered cellular antioxidant defenses, a dysfunction of GABAergic neurotransmission, and a perturbed osmoregulation in the brain lacking dystrophin.

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“…Atrophy of underlying muscles, bones, and cartilage is responsible for the typically aged appearance of the patient (Glass, 1963;Schnall and Smith, 1974;Souyris et al, 1983;Moore et al, 1993). Neurological complications such as paresthesia, trigeminal neuralgia, severe migraine, and epilepsy, often of the sensory Jacksonian type, can be associated phenomena (Gorlin and Pinborg, 1964;Paradise et al, 1980;Jurkiewicz and Nahai, 1985;Sagild and Alving, 1985). Ocular localization is described as enophthalmos and other less frequent signs (Wolf and Weber, 1940;Glass, 1963;Gorlin and Pinborg, 1964;Rees, 1976;Miller et al, 1987).…”

mentioning

confidence: 95%