Hemiconvulsion–hemiplegia–epilepsy syndrome: Current understandings

Auvin, Stéphane; Bellavoine, Vanina; Merdariu, Dana; Delanoë, Catherine; Elmaleh‐Bergès, Monique; Gressèns, Pierre; Boespflug‐Tanguy, Odile

doi:10.1016/j.ejpn.2012.01.007

Cited by 55 publications

(71 citation statements)

References 42 publications

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“…The clinical and radiological findings in our case have a striking resemblance to those seen in hemiconvulsion-hemiplegia-epilepsy syndrome in the paediatric population,9 which is a rare outcome of prolonged focal febrile convulsions with hemiplegia and subsequent epilepsy later9; brain imaging of those patients showed initial unilateral oedema followed by cerebral hemiatrophy at a later stage 10. The aetiology is suggested to be mixture of inflammatory cytokines activation induced by the focal status epilepticus or presumed primary viral infection that leads to initial cytotoxic brain oedema and eventually neuronal cell necrosis and atrophy 10.…”

Section: Discussionsupporting

confidence: 73%

Unilateral brain oedema related to focal status epilepticus

et al. 2013

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SUMMARYWe present a female patient in her late 30s, with baseline vegetative state following prior traumatic brain injury, who presented with prolonged right hemispheric status epilepticus. The neuroimaging revealed a striking right-sided pancortical oedema with left (crossed) cerebellar diaschisis and dilation of right hemispheric arteries. EEG was concordant and showed nearly continuous right hemispheric seizure discharges with suppressed background. Infective and vascular aetiologies were ruled out. The patient showed clinical and electrographic improvement following treatment with antiepileptic drugs. Unilateral cerebral oedema is a rare presentation of focal status epilepticus, and should be considered as a differential diagnosis in the appropriate clinical scenario. BACKGROUND

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Section: Discussionsupporting

confidence: 73%

Unilateral brain oedema related to focal status epilepticus

et al. 2013

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“…Late-onset epilepsy, often refractory to medical treatment, ensues in more than half of cases, usually within 1 year of disease onset, delineating the full syndrome 2. Despite this patient’s presentation with epilepsy and cerebral haemiatrophy, we considered that haemiconvulsion–haemiplegia–epilepsy syndrome was unlikely because of the presentation in late adolescence and the lack of a precipitating febrile illness.…”

Section: Discussionmentioning

confidence: 96%

Intracranial germinoma causing cerebral haemiatrophy and hypopituitarism

et al. 2018

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“…This condition can be very well differentiated on the basis of imaging features, which include unihemispheric focal cortical atrophy and ipsilateral head of the caudate nucleus, without any calvarial changes21. HHE is characterized by unilateral prolonged clonic seizures followed by the development of hemiplegia in younger children22. Patients with Silver-Russell syndrome have a normal head circumference and normal intelligence but poor growth, clinodactyly, hemi-hypertrophy, and a characteristic facial phenotype (triangular face, small pointed chin, broad forehead, and a thin wide mouth)23.…”

Section: Discussionmentioning

confidence: 99%

Classical oral manifestations of Dyke-Davidoff-Masson syndrome: a case report with review of the literature

Kalaskar

2018

J Korean Assoc Oral Maxillofac Surg

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Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of café-au-late pigmentation and ocular lipodermoid. This is the first case report of Dyke-Davidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.

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Hemiconvulsion–hemiplegia–epilepsy syndrome: Current understandings

Cited by 55 publications

References 42 publications

Unilateral brain oedema related to focal status epilepticus

Unilateral brain oedema related to focal status epilepticus

Intracranial germinoma causing cerebral haemiatrophy and hypopituitarism

Classical oral manifestations of Dyke-Davidoff-Masson syndrome: a case report with review of the literature

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