Acute repetitive giratory seizures as a manifestation of nonketotic hyperglycemia]

This 71 years old women without any history of epilepsy had diabetes mellitus. She was admitted for repetitive giratory seizures in relation with non-ketotic hyperglycaemia. The EEG showed right centro-parietal paroxysmal slow activity. Symptomatology disappeared within 48 hours after insulin therapy. One month later, she presented with a left hemiplegia in relation with a right sylvian infraction. The role of focal transitory ischaemia in connection with hyperglycaemia is discussed.     

Focal crisis induced by movement in a patient with nonketotic hyperglycemia]

Case report of a long term diabetic patient with partial motor crisis originating from passive movement of the right arm. This is a rare type of crisis when compared to spontaneous partial crisis in diabetic patients. Partial crises are often the initial manifestation of diabetes mellitus (about 19% of the cases reported). As in those cases registered in the literature, crisis control in this case was obtained by normalization of glycose serum levels. Possible mechanisms involved in the pathogenesis are discussed.     

Epileptic seizures in non-ketotic hyperglycemia]

Seizures are common in hyperglycemia and are often the first manifestation particularly in non-ketotic hyperglycemia (NKH). Published reports emphasize partial motor seizures almost exclusively. Here we present the clinical, biological and electrophysiological features of 22 consecutive patients with NKH who developed focal seizures and were treated in our department. Neurological exam was normal in 15 cases. When abnormal, it showed postictal obnubilation or diabetic polyneuropathy. Interictal EEG (obtained in 17 patients) showed focal or generalised slowing in 65% of cases, and ictal EEG (six patients) showed rapid spikes most often unilateral. CTs were normal in 77% of cases, and showed age-compatible cortico-subcortical atrophy in the others. All patients were alert, with glucose values between 13.6 and 55 mmoles/l and osmolarity values increased in all cases to a mild or moderate extent (266-309.20 mosm/l). Three out of 22 patients (14%) presented with motor epilepsy partialis continua. In 11/22 cases (50%), diabetes mellitus had not been diagnosed previously. Seizures associated with NKH were resistant to anticonvulsant treatment but responded well to insulin therapy and rehydration. They subsided completely in an average of 4 days, and only one patient had to be transiently transferred to ICU. We conclude on the importance of an early diagnosis of this condition to prevent malignant evolution of the epileptic syndrome into a state of hyperosmolarity and coma associated with a much higher mortality.     

Acute hydrocephalus in nonketotic hyperglycemia

We present four patients with typical neonatal onset non-ketotic hyperglycinemia (NKH) who developed hydrocephalus requiring shunting in early infancy. Brain imaging revealed acute hydrocephalus, a megacisterna magna or posterior fossa cyst, pronounced atrophy of the white matter, and an extremely thin corpus callosum in all. The three older patients had profound developmental disabilities. This suggests that the development of hydrocephalus in NKH is an additional poor prognostic sign.     

Chorea due to nonketotic hyperglycemia

A 62 year old diabetic and hypertensive male presented with sudden onset generalized chorea. Investigations revealed uncontrolled diabetes with absent ketones and normal serum osmolality. Achievement of euglycemia with insulin therapy abolished the involuntary movements completely within a day. The direct effect of hyperglycemia causing striatal neuronal dysfunction could be the pathogenesis of the chorea in our patient.     

Transient nonketotic hyperglycinemia in an asphyxiated patient with pyridoxine-dependent seizures

An asphyxiated neonate with pyridoxine-dependent seizures and associated transient nonketotic hyperglycinemia is reported. Frequent seizures and their resultant hypoxic-ischemic insult may have led to the elevation of the cerebrospinal fluid glycine level in this patient. Early diagnosis and treatment of pyridoxine-dependent seizures is essential for an improved neurologic outcome.     

Reflex epilepsy and nonketotic hyperglycemia in the elderly: a specific neuroendocrine syndrome

We present five elderly patients with focal reflex or posture-induced seizures and nonketotic hyperglycemia (NKH). Each patient exhibited interictal focal findings, such as hemiparesis or hemisensory or aphasic deficits. With control of the hyperglycemia, the seizures stopped, and the neurologic deficits resolved. The syndrome of focal reflex epilepsy and neurologic deficits in the elderly is transient and almost invariably related to NKH, thus representing a specific neuroendocrine syndrome.     

Focal seizures and non-ketotic hyperglycaemia.

The clinical features of seven patients with non-ketotic hyperglycaemia who developed focal seizures are presented. All patients were alert except one who was mildly confused. Glucose values varied from 17.8 to 55.1 mmol/l, while calculated osmolarity values were elevated in all cases to a mild or moderate extent (299.1 to 346.5 mmol/l). In three cases diabetes mellitus was a new diagnosis. Four patients had recurrent episodes of focal seizures when glycaemic control was lost. Movement induced or kinesigenic seizures were seen in three cases and epilepsia partialis continua in one case. Seizures associated with hyperglycaemia are resistant to anticonvulsant treatment and respond best to insulin and rehydration. Focal seizures in adults may indicate diabetes mellitus.     

Focal motor seizures mimicking hemifacial spasm

A young female patient, who presented acutely with an unusual movement disorder characterized by hyperkinetic facial movements, was referred to us for hemifacial spasm. The only abnormality on neurological examination was myoclonus of the left perioral and bilateral periorbital muscles, exacerbated by mental tasks. A week later, the patient also presented two generalized tonic–clonic seizures on awakening and was successfully treated with antiepileptic drugs. Laboratory and neuroimaging investigations yielded normal findings.Although we were unable to identify a cortical generator, the concomitant occurrence of generalized seizures, the disappearance of symptoms after treatment and the topography of the myoclonus support an epileptic origin of this myoclonus.     

Focal resection for malignant partial seizures in infancy

There is little experience with excisional surgery for drug-resistant partial seizures in very young patients. We describe our experience with 5 infants under 1 year of age with malignant partial seizures and deteriorating developmental status. All were experiencing frequent daily seizures that did not respond to 1st-line antiepileptic medications at high therapeutic serum levels and considerable medication toxicity. Three infants had complete resection of epileptic tissue (frontal corticectomy and prefrontal lobectomy) and are seizure-free. Two underwent partial resection (lateral temporal lobectomy, frontal corticectomy with anterior callosotomy) and have experienced a significant reduction in seizure frequency. Surgery did not result in any significant neurologic deficit or lead to compromise of developmental status. From these data, we tentatively conclude that excisional surgery can be performed safely in selected infants with medically uncontrolled malignant partial seizures and may significantly improve long-term seizure status. Referral to a tertiary center specializing in early childhood epilepsy surgical evaluation may be considered in these circumstances.     

Two cases of hemichorea-hemiballism with nonketotic hyperglycemia: a new point of view

Hemichorea-hemiballism (HCHB) is an usually continuous, nonpatterned, involuntary movement disorder caused by basal ganglia dysfunction, commonly due to a vascular lesion, described in nonketotic hyperglycemic patients. Particular computed tomography and magnetic resonance imaging findings have been described. The pathogenic mechanism of chorea arising during hyperglycemia and the nature of neuroimaging findings are unclear. In this paper we describe two elderly women with onset of HCHB during a hyperglycemic episode. The symptoms persisted in one of them after recovery of normal glycemia. The pathophysiological mechanism of the disease is discussed in the light of clinical and neuroradiological follow-up.     

Epileptic seizures, hemiplegia and hyperglycemia: late discovery of a localized cortical dysplasia]

A 56-year-old woman was hospitalized with a right hemiplegia and aphasia evoking a cerebral infarction. In fact the neurologic deficits were of post-ictal origin, secondary to a partial epilepsy which began a few weeks before, at the same time as a polyuria-polydipsia syndrome revealing diabetes mellitus. This case illustrates the possibility for a partial epilepsy to occur in relation with a nonketotic hyperglycemia. If in most of those cases there is no underlying cortical lesion, in some observations the hyperglycemia is associated with an infarction. In our case the MRI revealed another type of lesion: a cortical dysplasia in form of a unilateral micropolygyria with a perisylvian distribution centered around the insula. The discovery of a cortical dysplasia at such an age is very unusual.     

Transient nonketotic hyperglycinemia and defective serotonin metabolism in a child with neonatal seizures

Neonatal nonketotic hyperglycinemia is usually fatal or, less commonly, severely developmentally disabling, whereas transient nonketotic hyperglycinemia has usually been followed by normal development. We report a boy who had transient neonatal nonketotic hyperglycinemia but a coexistent disorder of serotonin metabolism manifested by initially low cerebrospinal fluid 5-hydroxyindoleacetic acid (which later normalized), low whole blood serotonin, and decreased platelet serotonin uptake. He survived the neonatal period but was neurodevelopmentally delayed and developed an autistic-like disorder. Later, his positron emission tomographic (PET) scans with alpha[(11)C] methyl-l-tryptophan revealed a pattern characteristic of autistic children. Although we know of no link between glycine and serotonin metabolism, and our patient had low, rather than high, central and peripheral serotonin, this case might represent a novel infantile disorder that affects both the glycine and serotonin neurotransmitter systems.     

Focal occipital seizures with cerebral polyopia.

Polyopia is the visual perception of multiple images of a single visual stimulus. Cerebral polyopia has previously been described as an ictal phenomenon associated with temporal lobe seizures. We report the case of a man with multiple cavernous angiomas and occipital lobe seizures manifesting as cerebral polyopia.     

Focal cerebral pathology presenting as tonic seizures

We report a case of a 14-year-old boy with a dysembryoplastic neuroepithelial tumor and mental retardation with intractable seizures, which were demonstrated to be predominantly tonic seizures by video-electroencephalography (EEG). He did not have any electrographic evidence of Lennox-Gastaut syndrome. Head magnetic resonance imaging (MRI) revealed a right parietal dysembryoplastic neuroepithelial tumor. He has been seizure free since surgical removal of the tumor. Clinicians need to be aware that tonic seizures can be associated with an underlying focal pathology that might be treatable.     

Focal prefrontal seizures induced by bilateral ECT

INTRODUCTION: It has been proposed that the greater efficacy of bilateral (BL) over right unilateral (RUL) electroconvulsive therapy (ECT) at low stimulus intensity is due to differences in site of seizure initiation. We hypothesized that focal prefrontal seizures are more common with BL than RUL administration. METHOD: Records were reviewed of the 1,007 ECT treatments of 84 consecutive patients randomized to RUL or BL electrode placement. RESULTS: Eight events were identified in which there was an electroencephalographic seizure without motor manifestation. All of these events occurred at titration sessions and with BL stimuli (p = 0.002). These events were more likely to occur later in the course of treatment. DISCUSSION: We suggest that BL ECT may induce focal seizures in prefrontal areas and that these seizures are more likely to occur later in the treatment course.