Isolated Aphasic Status Epilepticus as Initial Presentation of Nonketotic Hyperglycemia

Abstract

Diagnosis of aphasic status epilepticus is sometimes not easy because of its rarity and electroclinical dissociation. Although most cases are associated with organic brain lesions, nonketotic hyperglycemia (NKH)-related aphasic status epilepticus is rare, especially if it is isolated (without other clinical seizure activity). On the other hand, unlike other metabolic disorders, or hypoglycemia-related generalized seizures, focal motor seizure and epilepsia partialis continua can occur in 25% of NKH, with seizures being the initial manifestation in up to 50% of patients. However, the presentation of epileptic aphasia is rare in NKH patients. We report a rare case of NKH presenting initially as persistent and isolated aphasic status epilepticus. Brain magnetic resonance imaging did not reveal any focal lesion, but ictal electroencephalography (EEG) disclosed left frontotemporal continuous theta to delta waves, intermingled with epileptiform discharges. Correcting the hyperglycemia failed to improve the language disorder, and the seizure was controlled only by the addition of carbamazepine. Patients with NKH may initially present with isolated aphasic status epilepticus. Unlike stroke-related aphasia, accurate diagnosis is difficult if based solely on neurologic examination and brain neuroimaging. Use of EEG and blood sugar determination should be helpful in this special condition.

Isolated Aphasic Status Epilepticus as a Manifestation Induced by Hyperglycemia Without Ketosis

“…NKHrelated ASE seems to have its initial presentation as a mixed aphasia [12], similar to our case. This kind of aphasia is similar to that of patients with isolated aphasia, making it more difficult to establish an accurate diagnosis of NKH-related ASE, because of being very rare.…”

“…On literature review, initial status epilepticus presenting with aphasia has been reported in multiple sclerosis [10], diabetes [11], NKH [3,12], and AIDS-toxoplasmosis complex [13] or induced by the cephalosporin cefepime [14]. NKHrelated ASE seems to have its initial presentation as a mixed aphasia [12], similar to our case.…”

“…This kind of aphasia is similar to that of patients with isolated aphasia, making it more difficult to establish an accurate diagnosis of NKH-related ASE, because of being very rare. To our knowledge, isolated NKHrelated ASE has been reported in only two cases without complications in the brain such as an old cerebral infraction [3,12]. Table 1 shows a summary of published cases of patients with isolated NKH-related ASE up to the present.…”

De novo status epilepticus with isolated aphasia

“…These conditions could more or less be excluded by normal spinal tap, and resolving aphasia under anticonvulsant therapy may support ictal origin. De novo status epilepticus presenting with aphasia has been reported in multiple sclerosis [11,12], diabetes [13], nonketotic hyperglycemia [14,15], and AIDS-toxoplasmosis complex [16] or induced by the cephalosporine cefepime [17]; these conditions could also be excluded.…”

EEG in adults in the laboratory or at the patient's bedside

“…Finally, even though the possibility of seizures with purely ''negative'' clinical symptomatology remains highly controversial, there are in the literature some very rare descriptions of long-lasting motor or language neurological deficits considered to be related to partial status epilepticus [28,71,110,165]. Of course, EEG in these situations would in fact represent the key to the diagnosis.…”