Presentation
After encephalopathy onset, he presented with left hemiparesis, and suffered from left side dominant- or symmetrical tonic spasms. [frontiersin.org]
Results: The patient is a 2-year-old boy with a history of one prior complex febrile seizure who presented with greater than one hour of convulsive status epilepticus in the setting of fever. [pesquisa.bvsalud.org]
A consideration must be made for cerebrovascular accident as the cause for a child's neurological condition when one presents with seizures, especially when there are diffusion changes present on imaging. [thejns.org]
The present case had partial seizures with similar EEG findings. The associated hemiplegia may vary in intensity. [ncbi.nlm.nih.gov]
[…] and a concurrent febrile illness should be present. [n.neurology.org]
Entire Body System
- Camping
Eubanks Physical Medicine and Rehabilitation, University of Pittsburgh Medical Center, Pittsburgh, USA Sukhmanii Kahlon Internal Medicine, Medical University of the Americas, Camps, KNA Ethics Statement and Conflict of Interest Disclosures Human subjects [cureus.com]
Psychiatrical
- Fear
Despite treatment with valproic acid, clobazam, lamotrigine, perampanel, topiramate, and levetiracetam, daily focal seizures accompanied by mouthing, grimacing or expressions of fear continued to occur. [frontiersin.org]
Neurologic
- Pyramidal Tract Signs
They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. [link.springer.com]
- Neglect
On day 20, patient was discharged to inpatient rehab with persistent dense left hemiplegia and neglect. [aesnet.org]
- Limb Weakness
Convulsions involved left-sided limbs and face, lasted for 10–30 min with a frequency of 10–15/day. Her symptoms worsened and limb weakness deteriorated in the past 1 month with no relief from various physician and neurologist contacts. [ncbi.nlm.nih.gov]
- Hyporeflexia
We report a case of HHE syndrome in a 3-year-old boy with partial seizures (hemiconvulsion lasting 15–30 minutes) followed by left hemiplegia and hyporeflexia. [journals.viamedica.pl]
Workup
Infectious workup was significant for positive COVID-19 PCR and elevated inflammatory markers, consistent with MIS-C. [pesquisa.bvsalud.org]
Prior to presenting to us he was admitted and had received treatment for meningitis and status epilepticus in another hospital with workup showing a normal CSF exam and MRI findings as described in the images below. [jpma.org.pk]
Treatment
[…] of HHE syndrome involves medical treatment as there is no curative treatment. [ncbi.nlm.nih.gov]
In the failure of conservative treatment with anticonvulsants, epilepsy surgery may be indicated 7. History and etymology It was first described by the French neurologist Henri Jean Pascal Gastaut (1915-1995) et al. in 1960 4. [radiopaedia.org]
The severe impairments associated with HHE syndrome makes it imperative that continued research into the pathophysiology and treatment of the disorder is performed. [n.neurology.org]
[…] the treatment of cerebral herniation in HHE and advocate close monitoring for development of intracranial hypertension in these patients. [thejns.org]
Surgical treatment of delayed epilepsy in hemiconvulsion-hemiplegia-epilepsy syndrome. Neurology 2008;70:2116-22. 9. Serino D, Camassei FD, Delalande O, Marras CE, Specchio N, Vigevano F, et al. [neurologyindia.com]
Prognosis
Early recognition of the syndrome may help provide patients and families with an accurate prognosis regarding the subsequent development of epilepsy. [n.neurology.org]
Early identification of this syndrome may help the treating physician in providing families with a relatively accurate prognosis regarding the functional outcome and subsequent development of epilepsy. [infona.pl]
[…] subcortical cavitations in the affected hemisphere reduced caliber of ACA, MCA or PCA on the affected side small corpus callosum ipsilateral hemispheric volume loss with a shift of midline structures usually lack hemosiderin on T2*/SWI Treatment and prognosis [radiopaedia.org]
Prognosis is variable, from the resolution of hemiplegia and seizures to permanent hemiparesis and refractory epilepsy. Design/ Methods: This is a case report based on a chart review. [pesquisa.bvsalud.org]
Prognosis is variable, from the resolution of hemiplegia and seizures to permanent hemiparesis and refractory epilepsy. [ingentaconnect.com]
Etiology
HHE syndrome can be divided into 2 groups based on known etiologies. [n.neurology.org]
Although the etiologies of this syndrome are diverse, preexisting asymptomatic lesion of perinatal or prenatal origin is found in a number of cases. [e-jyms.org]
The etiologies and the underlying mechanisms remain to be understood. Using a review of the literature, we summarized the data of the last 20 years. It appears that idiopathic HH/HHE syndrome is the most common reported form. [pubmed.ncbi.nlm.nih.gov]
The possible etiological triggers are vasculopathy, trauma, inflamation, and viral infections. In situ ations where no trigger is identified, it is labelled as idiopathic.[2] Dengue has never been reported as a possible trigger for HHE. [neurologyindia.com]
Epidemiology
Complicación Convulsión Estudio caso Hemiplejía Lactante Sistema nervioso patología Vacunación Hombre Classification Pascal 002 Biological and medical sciences / 002B Medical sciences / 002B05 Infectious diseases / 002B05A General aspects / 002B05A02 Epidemiology [pascal-francis.inist.fr]
DISCUSSION Epidemiology. HHE syndrome was first reported by Gastaut and colleagues1 approximately 50 years ago. [n.neurology.org]
Anticipation and identification in appropriate clinical and epidemiological scenarios can help in prognostication and planning long-term management. Financial support and sponsorship Nil. [neurologyindia.com]
Pathophysiology
More research is needed to elucidate further HHE syndrome's pathophysiology and assess long-term outcomes in patients with HHE syndrome. [pesquisa.bvsalud.org]
The pathophysiology of HHE syndrome remains poorly understood though is clearly multifactorial. Factors thus far implicated are hyperthermia, proinflammatory state, and cytotoxic edema from prolonged ictal activity. [ingentaconnect.com]
Although first described by Gastaut and colleagues more than 6 decades ago,1 the pathophysiology of HHE syndrome remains unclear. [n.neurology.org]
Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: a report with pathophysiological implications.Seizure. 2007; 16: 371-376 Ingram T.T.S. [ejpn-journal.com]
Prevention
[…] is better than cure and early and aggressive termination of seizures is considered the most effective way to prevent post-convulsive hemiplegia 9. [radiopaedia.org]
Preventive treatment of longlasting febrile seizures is important, but there is no standard therapy for epilepsy in HHE syndrome, as many different clinical situations may be encountered in this context. [moh-it.pure.elsevier.com]
HHE syndrome should be preventable by continued advances related to the rapid resolution of seizures. [n.neurology.org]
Early recognition and treatment of status epilepticus is imperative to prevent permanent cerebral injury. Funding: Please list any funding that was received in support of this abstract.: None. [aesnet.org]
[…] the remote seizures, although three of our four cases required long-term preventive antiepileptic therapy [Table 1]. [pediatricneurosciences.com]