Oral ketamine in paediatric non-convulsive status epilepticus.

In children, non-convulsive status epilepticus (NCSE) is rare and difficult to treat. Response to steroids and GABAergic medication is variable and often decreases with increasing duration of NCSE. We present our experience with oral ketamine, an NMDA-receptor antagonist, administered to five children with severe epilepsy (Lennox-Gastaut Syndrome, myoclonic-astatic epilepsy, progressive myoclonic epilepsy and Pseudo-Lennox Syndrome) during an episode of NCSE. Resolution of NCSE was documented in all cases clinically and electroencephalographically within 24-48 hours of starting ketamine. No significant side effects were noted.Clinical TrialComparative StudyJournal Articleinfo:eu-repo/semantics/publishe

Unilateral cortical necrosis following status epilepticus with hypoglycemia.

Isolated status epilepticus or severe hypoglycemia rarely causes irreversible focal neurologic deficits in children. We describe three children who presented with status epilepticus and prolonged hypoglycemia resulting in hemiplegia due to unilateral hemispheric damage. The non-vascular cortical topography of the lesions is consistent with selective neuronal necrosis, confirmed by histopathology in one patient. This suggests increased neuronal vulnerability to necrosis secondary to energy failure resulting from combination of hypoglycemia and status epilepticus.Case ReportsJournal Articleinfo:eu-repo/semantics/publishe

Crossed cerebellar diaschisis secondary to refractory frontal seizures in childhood.

We report a girl with refractory partial seizures since 7 years of age, secondary to right frontal cortical dysplasia, who developed MRI and SPECT abnormalities in the contralateral hemicerebellar cortex. These became more marked, leading to left hemicerebellar atrophy. Crossed cerebellar diaschisis has been described mostly in hemispheric stroke and supratentorial tumours, but less often in epilepsy. It is usually a transient phenomenon. This report shows that crossed cerebellar diaschisis can develop within two years of seizure onset and evolve over time.Case ReportsJournal Articleinfo:eu-repo/semantics/publishe

Benign intracranial hypertension: atypical presentation of Miller Fisher syndrome?

Acute ocular paresis, nausea, vomiting, and headaches associated with high intracranial pressure without obvious intracranial pathology are typical features of benign intracranial hypertension. We describe two young children whose presentation, initially suggestive of idiopathic or benign intracranial hypertension, evolved to comprise ophthalmoplegia, ataxia, and areflexia. This triad characterizes Miller Fisher syndrome, a clinical variant of Guillain-Barré syndrome that occurs rarely among children. In both patients, this diagnosis was supported by the clinical course and neurophysiologic findings. Plasma serology was positive for Campylobacter jejuni and anti-GQ1b antibodies in one patient and for antimyelin antibodies in the other. This report of two children with Miller Fisher syndrome presenting with intracranial hypertension adds to the findings for a similar patient treated previously, which raises the question concerning the possible role or contribution of benign intracranial hypertension in Miller Fisher syndrome.Case ReportsJournal ArticleSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Pediatric phantom vision (Charles Bonnet) syndrome.

Visual symptomatology in childhood often presents diagnostic difficulties. Recurrent paroxysmal visual complaints, although typically associated with migraine, may also signal other disorders. We describe a 9-year-old partially sighted male with paroxysmal zoopsias resulting from Charles Bonnet syndrome. This condition is characterized by paroxysmal visual hallucinations occurring in patients with chronic visual impairment, akin to the phantom-limb phenomenon. This pediatric case is the fourth report of this condition. We have reviewed the other cases.Case ReportsJournal ArticleSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Cervical myelitis from herpes simplex virus type 1.

Although subacute ascending paralysis without sensory involvement is typically evocative of Guillain-Barré syndrome, it can alternatively be due to infection or inflammation of the spinal cord. We describe a 16-month-old female who presented with ascending flaccid paresis after an upper respiratory tract infection. She then developed signs of upper motor neuron involvement of the lower limbs associated with upper motor neuron involvement of the upper limbs. Motor nerve conduction and electromyographic studies of upper limbs demonstrated anterior horn cell involvement. Neuroimaging was consistent with cervical myelitis, and cerebrospinal fluid polymerase chain reaction was positive for herpesvirus-1. Although association with the primary infection of the respiratory tract may be fortuitous, possible neurotropic or hematogenous spread of herpesvirus-1 to the cervical spinal cord cannot be excluded. She then developed signs of upper motor neuron involvement of the lower limbs associatred with lower motor neuron involvement of the upper limbs [corrected].Case ReportsJournal ArticleSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Nonsurgical cerebellar mutism (anarthria) in two children.

Cerebellar mutism (anarthria) is a well-described complication of posterior fossa tumor resection. It is accompanied by a characteristic behavior including irritability and autistic features. This syndrome is typically reversible within days to months. Underlying pathophysiology is unknown. We describe two children who presented with a similar clinical finding after nonsurgical cerebellar involvement, hemolytic-uremic syndrome in one and cerebellitis in the other. Postmortem pathologic findings in the first patient indicated cerebellar ischemic necrosis. Single-photon emission computed tomography in the second patient revealed diffuse cerebellar hypoperfusion with no supratentorial abnormalities, refuting a phenomenon of diaschisis between cerebellar and frontal connections. These findings confirm that this clinical syndrome may occur in a nonsurgical, nontraumatic context. They are consistent with recent integrative hypotheses explaining cerebellar anarthria.Case ReportsJournal ArticleSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Pediatric SUNCT Syndrome.

This report describes a 5-year-old male with sudden unilateral headache attacks (2-50 seconds) accompanied by conjunctival injection, lacrimation, and nasal congestion. The episodes occurred without a precipitating factor, never during sleep. Brain imaging was normal. The attacks resolved spontaneously within 5 months. This headache syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) was previously described in two other children aged 10 and 11.Case ReportsJournal Articleinfo:eu-repo/semantics/publishe