Slavko Janković

The Frequency of Reversible Parkinsonism and Cognitive Decline Associated with Valproate Treatment: A Study of 364 Patients with Different Types of Epilepsy

Summary:  Purpose: We report the frequency of parkinsonism and cognitive decline (P/CD) in patients treated with valproate (VPA) after 1 year of treatment and at least 1 year of follow‐up.

Frequency, causes and phenomenology of late seizure recurrence in patients with juvenile myoclonic epilepsy after a long period of remission

PURPOSE To determine frequency, causes, and phenomenology of late seizure recurrence (SR) in patients with juvenile myoclonic epilepsy (JME) after remission of at least 1 year. METHODS Among 2722 epileptic patients from tertiary referral center, we retrospectively identified 105 patients (62 females; mean age 22.3+/-7.2 years) with an established diagnosis of JME. All patients were treated with valproates (83.3%), or lamotrigine, topiramate, phenobarbital, add-on clobazam, or combinations (16.2%). RESULTS The median period of follow-up was 4.2+/-3.2 (range: 1-17) years. SR occurred in 74 patients (70.5%) after median period of 2.4+/-3.2 years. Twenty-two patients (29.7%) experienced myoclonic seizures (MS), 13 (17.7%) generalized tonic-clonic seizures (GTCS), 37 (50%) a combination of MS and GTCS, and two (2.6%) a combination of MS, GTCS and absence seizures. SR was associated most frequently with sleep deprivation and AED withdrawal, and rarely with alcohol intake, drug abuse, photostimulation, or menstruation. No provoking factors for SR were identified in 31.1% and 45% of cases with MS and GTCS, respectively. The majority of patients (59/74) had a single SR. A second SR occurred less frequently in patients in whom valproate dosage was increased after the first SR (p=0.0048). CONCLUSION Late SR (mainly MS and GTCS) is detected frequently after prolonged follow-up in patients with JME despite the use of best-known therapy, usually due to AED withdrawal or erratic life style. Instead of futile efforts to persuade the patient to conform to restrictive life style, it is probably more efficient to use initial higher doses of AEDs.

Etiology of a short-term mortality in the group of 750 patients with 920 episodes of status epilepticus within a period of 10 years (1988–1997)

PURPOSE To determine the etiology of short-term mortality in patients with status epilepticus (SE). METHODS 920 episodes of SE were recorded among 750 patients in a 10-year period. According to the clinical assessment, sequence of events that led to death in a particular case showed two major causes of death: (1) underlying disease, and (2) complications caused by convulsions, therapy or coma. RESULTS Among 920 episodes of SE, 120 (13%) patients passed away. 79 patients (65.8%) died due to the underlying disease and 27 patients (22.5%) died of the combination caused by complications of underlying disease, convulsions, therapy, and/or coma. Among remaining 14 patients (11.7%), underlying disease was not the cause of death. Those 14 patients suffered complications caused by convulsions, therapy, and coma which caused death in four; therapy and coma in three; therapy in three; coma in two; and convulsions and coma in two patients, in the order already mentioned. CONCLUSIONS Among approximately 9 out of 10 patients with SE, death was the result of underlying disease. Although with very few patients, additional factors could provoke fatal complications of SE. In case of 1 among 10 patients complications caused by coma, therapy, and/or convulsions were the immediate cause of death. In case of such patients timely and adequate treatment could prevent death.

Hippocampal metabolic dysfunction in juvenile myoclonic epilepsy: 3D multivoxel spectroscopy study

PURPOSE To investigate the metabolic differences in hippocampi of patients with juvenile myoclonic epilepsy (JME) and healthy controls using magnetic resonance spectroscopy (MRS). METHODS A 3D multivoxel SE 135 MRS study on 1.5 T scanner of both hippocampi was performed in 17 patients with JME and normal brain MRI and in 19 age and sex matched controls. Three dominant signals were measured: Choline (Cho), Creatine (tCr) and N-Acetylaspartate (NAA) and expressed as ratios of Cho:tCr, NAA:tCr, NAA:Cho and NAA:(Cho+tCr). Metabolite ratios in head, body and tail of each hippocampus in the JME group of patients were compared with ratios from corresponding structures in the control group. RESULTS We found a significant difference in metabolite ratios of both hippocampi between the JME and the control groups. We detected significant differences of Cho:tCr in the head, NAA:tCr in the head, body and tail, NAA:Cho and NAA:(Cho+tCr) in the body and tail of the left hippocampus, and NAA:Cho and NAA:(Cho+tCr) in the body and tail of the right hippocampus. DISCUSSION Although not previously recognized as a part of the epileptogenic network, our results suggest that the hippocampus, well recognized as a key player in focal epilepsies, may have a certain role in the pathogenesis of JME.

Long-term survival in patients with status epilepticus: A tertiary referral center study

Purpose:  To determine long‐term survival in patients with status epilepticus (SE).

Multiple rhythmic movement disorders in a teenage boy with excellent response to clonazepam

Rhythmic movement disorders (RMDs) refer to a group of behaviors characterized by stereotypic rhythmic movements of the body (body rocking), head (head banging), or limbs (leg rolling) appearing in relation to sleep,1 frequently self-limited to childhood and occasionally continuing into adulthood. Herein, we describe a 16-year-old right-handed man with four different types of RMDs. At the age of 2.5 years symptoms spontaneously appeared as repetitive, rhythmic, self-noninjurious, stereotypic, sleep-related rhythmic movements consisting of forward and backward swaying of the trunk (body rocking). The frequency of movements progressively increased for 1 year and then slowly decreased and disappeared at the age of 4 years. After that time the movements transformed to head banging (or jactatio capitis nocturna; JCN) against the pillow. The movements appeared in quiet wakefulness or in superficial sleep, with infrequent and random remissions for only a short period. Head banging increased in frequency after the divorce of his parents (at the age of 10 years) and particularly after separation from his mother 6 months before the admission (at the age of 16). He also complained that from the age of 7 he had body rolling and head rolling as presleep behavior. Head banging, body or leg rolling could all appear in random succession, in different combinations or independently as isolated symptom on a particular night. Perinatal period was uneventful and the milestones of psychomotor development were successfully acquired. Patient never hurt himself, claimed to be amnesic for the events with daily activities unaffected. PSG with sleep architecture, neurologic, psychiatric, cardiologic, ophthalmologic, and hematologic examinations, with laboratory and brain MRI were normal. Total IQ score was 101, suggesting average cognitive abilities. We observed head banging virtually every night, often grouped in multiple episodes identical in intensity and pattern but with different duration: from only a few head movements (particularly during morning awakening), to episodes of prolonged head banging (longer than 90 min) mainly during quiet wakefulness or non-REM-stage 1 sleep. Low dose of clonazepam (1 mg, 30 min before going to bed) induced complete disappearance of all RMDs during the 5-month of follow-up. One night polysomnography identified two episodes of rhythmic and stereotypic head banging (Video 1), one episode of body rolling (Video 2), and leg rolling-like movements (Video 3), respectively, with the patient lying on his right side. RMDs are considered as “self-stimulating” behavior or a tension-reliving maneuver with a hypnogenic potential.2 They were seen to arise from all stages of sleep, but usually occurred in transition from wake to sleep,1 as in our patient. We did not observe RMDs during REM sleep or the signs of REM sleep without atonia as reported by Kempenaers et al.2 and Stepanova et al.,3 nor autonomic activation (i.e. tachycardia).4 Since RMDs in our patient started at an early age we could not establish their connection (other than aggravation) with either psychological profile or the family events. RMDs are characteristic of early childhood, but have been reported with increased frequency in adolescents and adults.4,5 A case of the 27-year-old man with head banging being restricted to REM sleep was described recently.6 It has been postulated that RMDs persisting in adulthood prevailed in REM and SWS sleep,4 while in our teenage patient they appeared only during quiet wakefulness and superficial sleep. Our case is in accordance with reports showing that patients with head banging persisting into adulthood are not necessarily mentally retarded.7 The etiology of RMDs remains unknown. No effective pharmacologic or behavioral therapy has been established. However, beneficial response to imipramine in a case of posttraumatic jactatio1 and successful treatment of RMD with clonazepam have already been described.5,8 We present a case report to illustrate a polysomnographic presentation of multiple RMDs with prompt and complete cessation on clonazepam therapy, although the follow up was only 5 months.

Validation of the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) Serbian version

The Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) was developed and proven efficient for the rapid detection of a major depressive episode in people with epilepsy. This study describes the development, validation, and psychometric properties of the NDDI-E Serbian version. A consecutive sample of 103 patients with epilepsy was assessed using the Beck Depression Inventory (BDI) and the NDDI-E. All patients had no major difficulties in understanding or answering the questions of the Serbian version. Cronbachs alpha coefficient was 0.763. Receiver operating characteristic analysis showed an area under the curve of 0.943 (95% CI; 0.826 to 0.951), a cutoff score of ≥14, a sensitivity of 72.2%, a specificity of 95.2%, a positive predictive value of 81.3%, and a negative predictive value of 94.3%. The NDDI-E Serbian version scores were significantly and positively correlated with those of the BDI (p<0.001). The NDDI-E Serbian version constitutes a concise and consistent depression screening instrument for patients with epilepsy.

Bálint-like syndrome as an unusual representation of non-convulsive status epilepticus

The clinical signs of posterior cortex dysfunction are, due to their paucity and subtlety, very often ignored as non-specific during clinical evaluation of non-convulsive status epilepticus. Therefore, focal non-convulsive status epilepticus emerging from the posterior cortex, and especially the parietal lobes, can be fairly under-recognised. We report a 66-year-old patient with focal non-convulsive status epilepticus presenting as isolated Bálint-like syndrome, successfully treated to full clinical and electrophysiological recovery. The diagnostic and pathophysiological features are discussed. Focal non-convulsive status epilepticus can be associated with negative phenomena such as neuropsychological deficits mimicking those detected more often in degenerative and vascular brain diseases.

Coexistence of Unverricht‐Lundborg disease and congenital deafness: Molecular resolution of a complex comorbidity

Purpose:  We report on genetic analysis of a complex condition in a Serbian family of four siblings, wherein two had progressive myoclonic epilepsy (PME) and congenital deafness (CD), one had isolated congenital deafness (ICD), and one was healthy.