A. Cerullo

Nocturnal Paroxysmal Dystonia with Short‐Lasting Attacks: Three Cases with Evidence for an Epileptic Frontal Lobe Origin of Seizures

The epileptic or nonepileptic origin of noctur‐ nal paroxysmal dystonia (NPD) has been debated. We studied three patients with frequent attacks during non‐REM sleep. During prolonged video‐EEG monitoring, two patients had a convulsive seizure after a typical NPD episode and on these occasions EEG showed epileptiform discharge. In the three patients, attacks occurred repeatedly with different intensity, representing “fragments” of the same seizure. These fragments of the attack could occur periodically every 20–40 s. We postulate that short NPD attacks are actually epileptic seizures originating from the frontal lobes. The rhythmicity of the episodes may be due to rhythmic oscillation of cortical function during non‐REM sleep.

Paroxysmal periodic motor attacks during sleep: clinical and polygraphic features

Three patients complained of paroxysmal motor attacks during sleep. Videopolygraphic recordings showed that motor activity could be divided into events of increasing behavioural complexity. Simpler motor events often represented the initial fragment of more complex attacks. Clinical features suggested the attacks represented frontal lobe epileptic seizures. The attacks recurred during NREM sleep with a periodic repetition every 20-60 sec. This periodicity could be related to the analogous physiological oscillation during light sleep and the periodicity of K complexes, exerting a facilitating influence upon epileptic mechanisms.

Partial Epilepsy of Long Duration: Changing Semiology with Age

There are few data on changing patterns of localization‐related epileptic syndromes with time and particularly on changes in seizure semiology as patients age. We retrospectively reviewed 53 patients aged >60 years who had had partial epileptic seizures for a mean duration of 44 years. In 20 patients, seizures became progressively less elaborate and briefer with time. In 29 patients, seizures were unchanged. In four patients, seizures worsened, with the appearance of drop attacks, secondarily generalized seizures, increasing drug resistance, and mental deterioration. These latter 4 patients had cerebrovascular disease with multiple strokes.

AUTONOMIC AND HORMONAL ICTAL CHANGES IN GELASTIC SEIZURES FROM HYPOTHALAMIC HAMARTOMAS

OBJECTIVES We describe two patients with hypothalamic hamartoma and gelastic seizures. METHODS We performed ictal neurophysiological studies with polygraphic recordings of autonomic parameters and hormonal ictal plasma concentration measurements. RESULTS Ictal recordings showed a stereotyped modification of autonomic parameters: increase in blood pressure and heart rate, peripheral vasoconstriction and modification of respiratory activity. At seizure onset, the norepinephrine plasma level was high and epinephrine unchanged, whereas prolactin and adrenocorticotropic hormone were increased in both cases. Growth hormone and cortisol plasma concentrations in each patient showed a different response to seizures. CONCLUSIONS These data provide evidence that gelastic seizures are accompanied by an abrupt sympathetic system activation, probably due to the direct paroxysmal activation of limbic and paralimbic structures or other autonomic centres of the hypothalamus and medulla.

Epileptic drop attacks in partial epilepsy: clinical features, evolution, and prognosis

OBJECTIVES Sudden falls have been described in patients with partial epilepsy. However, no study has detailed the clinical, EEG, and evolutive features of partial epilepsies with drop attacks. METHODS In a consecutive series of 222 patients with partial epilepsy admitted for uncontrolled seizures over a 10 year period, 31 patients presented with epileptic drop attacks during evolution of their illness. Twenty two patients had frontal, five temporal, and four multifocal or undefinable lobe epilepsy; 74% of the cases showed an EEG pattern of secondary bilateral synchrony during evolution. A statistical comparison of some clinical and EEG features between the patients with epileptic drop attacks and patients with partial epilepsy without drop attacks (control group of 191 patients) was carried out. RESULTS Seventy four per cent of patients had a poor prognosis and 45% were mentally retarded; 52% of patients with epileptic drop attacks continued to have epileptic falls associated with partial seizures and mental deterioration at the end of the follow up. These characteristics of patients with epileptic drop attacks were significantly different from the control group. CONCLUSION Almost all literature reports concur that the physiopathogenetic substrate of epileptic drop attacks is a mechanism of secondary bilateral synchrony. A localised epileptic focus may lead to a process of secondary epileptogenesis involving the whole brain, causing a progressive cerebral disturbance with worsening of the epileptic seizures and higher cerebral functions.

Occipital Lobe Epilepsy: A Chronic Condition Related to Transient Occipital Lobe Involvement in Eclampsia

Summary: Eleven hours and 6 days, respectively, after childbirth 2 women exhibited hypertension and paroxysmal visual disturbances followed by tonic‐clonic seizures and no other preeclamptic signs. Both developed partial epilepsy with occipital lobe seizures with no other neurologic defects. Neuroimaging showed no lesion during either the acute episode or 3–5 year follow‐up. Selective vulnerability of the occipital lobes during eclamptic hypertensive encephalopathy was the probable pathophysiologic mechanism. Aggressive antihypertensive treatment during the acute phase may have exacerbated the risk of permanent cerebral damage.

Ticlopidine impairs phenytoin clearance: a case report.

Ticlopidine is an antiplatelet agent used in the prophylaxis of peripheral and cerebrovascular diseases, [1-3] ischemic heart disease, [1,3] and vascular complications of diabetes mellitus. [4] In healthy volunteers ticlopidine reduces the clearance of theophylline [5] and antipyrine, [6] probably by inhibiting the activity of cytochrome P450 enzymes. On the basis of these observations, the package insert in some countries contains a warning advising caution when ticlopidine is introduced in patients receiving therapy with highly metabolized drugs. We report a case of metabolic phenytoin-ticlopidine interaction in which phenytoin plasma concentrations increased and CNS toxicity appeared when ticlopidine was added to the therapeutic regimen. A 65-year-old man had a history of complex partial seizures, sometimes with secondary generalization, which started at the age of 52 years. A brain CT scan obtained at this time was normal. He had been treated with different antiepileptic regimens for about 10 …

Arylsulfatase A pseudodeficiency and Lafora bodies in a patient with progressive myoclonic epilepsy

Summary: Since age 12 years, a 25‐year‐old woman had a syndrome with myoclonic epilepsy, cerebellar signs, and spontaneous myoclonus. Skin biopsy showed typical Lafora bodies (LB), but she lacked a progressive course and mental impairment, hallmarks of Lafora disease. Lysosomal enzyme assays showed low level arylsulfatase A (ASA) activity. DNA study disclosed a homozygous ASA Pd genotype. Both parents carried one Pd allele. The still‐unknown relationship between the pathologic level of ASA activity and myoclonic epilepsies suggests introduction of ASA assays in patients with PME.

Facial Asymmetry in Partial Epilepsies

Summary: Fifty‐six consecutive epileptic patients with partial seizures (30 temporal, 26 extratemporal) and facial asymmetry were studied. Facial asymmetry was compared with EEG, radiologic, and other clinical findings. Thirty patients had a lesional epilepsy whereas 26 were considered cryptogenic. In lesional epilepsies, 60% of patients had EEG foci ipsilateral to the smaller hemiface and only 20% had EEG foci contralaterally. In the cryptogenic group, the EEG focus was ipsilateral in 50% and contralateral to the facial smallness in 46%. No differences were noted between temporal and extratemporal epilepsies. Early acquired cerebral lesions may modify development of the hemisphere involved, leading to a small ipsilateral hemiface and seizures originating from the same side.

Celiac Disease, Epilepsy, and Occipital Calcifications: Histopathological Study and Clinical Outcome

Bye et al. in 1993 first described the pathological findings in a patient with celiac disease (CD), bilateral occipital calcifications, and drug-resistant epilepsy who became seizure-free after extensive occipital resection. We report a case with an identical anatomoclinical condition in which surgical removal of the circumscribed calcified lesion was ineffective and epilepsy worsened until a gluten-free diet was started. The poor surgical outcome may have been due to residual epileptogenic tissue or to an unidentified secondary epileptic temporal focus. Our observation confirms the histopathological characteristics of the cerebral lesion in CD and the importance of a gluten-free diet in controlling seizures.