Relationship between bone density and levetiracetam monotherapy in epilepsy patients.

PURPOSE: Levetiracetam (LEV) is an anti-seizure drug (ASD). A consensus has not been reached regarding its effects on bone health. This cross sectional study was planned to assess short, medium and long-term effects on bone density and blood parameters that are associated with bone metabolism. METHODS: The sample consisted of 47 patients with epilepsy, who had been on LEV monotherapy for more than six months. All participants were over 18 years of age and had no other risk factors for osteoporosis. None of them used any other anti-seizure drug before. Bone mineral density (BMD) was evaluated with dual energy X-ray absorptiometry (DEXA) and biochemical markers associated with bone health were measured. Patients were divided into three groups depending on how long they had been on LEV for (Group A: <1 year; Group B: 1-5 years, Group C: >5 years) and compared with each other in terms of BMD scores and blood parameters. RESULTS: The mean age of patients was 32. 6 ± 13.3 and 20 patients were female (42.5%). The mean onset age of epilepsy was 28.1 ± 13.4 years. Average LEV period of consumption was 2.7 ± 2.7 years and mean daily dose was 1041.7 ± 393.9 milligrams. Lumbar BMD scores of the group with LEV usage < 1 year were significantly lower than those of the group with LEV usage of 1-5 years (p < 0.05). Lumbar vertebra scores were found to be lower in group of LEV usage duration of < 1 year when compared with LEV usage duration > 5 years but the difference was not statistically significant. CONCLUSION: We argue that LEV might have a negative effect on bone densitometry at the lumbar level in short-time usage for less than one year. Furthermore, no deleterious impact on bone metabolism was observed in long-term treatment. LEV seems as a rational drug for treatment of epilepsy patients, particularly for those with osteoporosis, since the comparative results of one year and longer than 5-years usage data did not show any statistically significant difference.

Headache in idiopathic/genetic epilepsy: Cluster analysis in a large cohort.

OBJECTIVE: The link between headache and epilepsy is more prominent in patients with idiopathic/genetic epilepsy (I/GE). We aimed to investigate the prevalence of headache and to cluster patients with regard to their headache and epilepsy features. METHODS: Patients aged 6-40 years, with a definite diagnosis of I/GE, were consecutively enrolled. The patients were interviewed using standardized epilepsy and headache questionnaires, and their headache characteristics were investigated by experts in headache. Demographic and clinical variables were analyzed, and patients were clustered according to their epilepsy and headache characteristics using an unsupervised K-means algorithm. RESULTS: Among 809 patients, 508 (62.8%) reported having any type of headache; 87.4% had interictal headache, and 41.2% had migraine. Cluster analysis revealed two distinct groups for both adults and children/adolescents. In adults, subjects having a family history of headache, ≥5 headache attacks, duration of headache ≥ 24 months, headaches lasting ≥1 h, and visual analog scale scores > 5 were grouped in one cluster, and subjects with juvenile myoclonic epilepsy (JME), myoclonic seizures, and generalized tonic-clonic seizures (GTCS) were clustered in this group (Cluster 1). Self-limited epilepsy with centrotemporal spikes and epilepsy with GTCS alone were clustered in Cluster 2 with the opposite characteristics. For children/adolescents, the same features as in adult Cluster 1 were clustered in a separate group, except for the presence of JME syndrome and GTCS alone as a seizure type. Focal seizures were clustered in another group with the opposite characteristics. In the entire group, the model revealed an additional cluster, including patients with the syndrome of GTCS alone (50.51%), with ≥5 attacks, headache lasting >4 h, and throbbing headache; 65.66% of patients had a family history of headache in this third cluster (n = 99). SIGNIFICANCE: Patients with I/GE can be clustered into distinct groups according to headache features along with seizures. Our findings may help in management and planning for future studies.

Multiple sclerosis attack case presenting with pseudo-vestibular neuritis.

PURPOSE: Pseudo-vestibular neuritis is a clinical diagnosis for patients presenting with acute vestibular syndrome due to a central pathology. CASE REPORT: We reported a case of multiple sclerosis characterized by pseudo-vestibular neuritis. Our case was a 32-year-old male patient. The patient, who was diagnosed with multiple sclerosis in September 2019, came to the emergency clinic in January 2020 with the complaint of severe vertigo, vomiting-nausea. A newly developed demyelinating plaque was detected in the left vestibular nucleus in cranial MRI. The patient had no hearing loss. On examination of the patient, nystagmus findings supporting peripheral vestibular involvement were present on the left side. Neurologic examination showed left-sided hyperactive deep tendon reflexes, Achilles clonus, dysmetria, ataxia to the left and plantar reflex with extensor response on the left. Video head impulse test and cervical evoked myogenic potential tests were performed. Vestibular hypofunction was present on the left side. Steroid pulse therapy was administered as 1000 mg/day, i.v for 7 days. After treatment, his complaints decreased. In addition, there was an improvement in examination findings. CONCLUSION: Multiple sclerosis is shown to be an etiological factor in patients with pseudo-vestibular neuritis.

Adult-onset subacute sclerosing panencephalitis presenting with tonic motor seizures.

PURPOSE/AIM OF THE STUDY: Subacute sclerosing panencephalitis (SSPE) is a degenerative disease of the brain caused by a persistent measles virus infection occurring mostly in childhood or early adolescence. The spectrum of epileptic phenomena associated with SSPE is wide, varying from partial or generalized tonic-clonic seizures and atypical absences to myoclonic-atonic attacks. Tonic seizures are very rare in SSPE. MATERIALS AND METHODS: Herein, we discuss a case of 25 years old male that presented with adult-onset SSPE with tonic seizures accompanying myoclonic seizures. RESULTS: Patient was treated with clonazepam 5 mg/day and an isoprinosine regimen with a dose of 70 mg/kg/day. This is the fourth case of SSPE presenting with myoclonic and tonic seizures and the first case of SSPE with myoclonic and tonic seizures reported in an adult-onset case in the English literature. CONCLUSIONS: Adult-onset SSPE with tonic seizures is rare and may be confusing, thus, it is important to recognize the presence of this type of tonic motor seizures in SSPE patients.

Vertiginous epilepsy: documentation of clinical and electrophysiological findings of nine patients.

Epileptic vertigo is often a diagnostic problem. We aimed to present the clinical and electrophysiological features of patients with epileptic vertigo with a view to addressing the pathophysiology of this rare aura symptom. Nine epileptic vertigo patients were included in the study. All patients were subjected to neuro-otologic examination, interictal electroencephalogram (EEG), audiogram, cervical vestibular evoked myogenic potential testing (cVEMP), video head impulse testing (vHIT) and brain magnetic resonance imaging (MRI). Eight patients described their aura as epileptic vertigo and one as dizziness. In three patients, auditory hallucinations preceded epileptic vertigo. The semiology of epileptic vertigo was true vertigo in five patients, vertigo with nausea in two patients and vertigo with hearing loss in one patient. Two patients suffered from focal seizures, and in seven patients the seizures were evaluated as focal to bilateral tonic-clonic seizures. MRI was normal in all patients. EEG was abnormal in all cases and showed high-voltage spike or spike-slow-wave complexes, or both, located more frequently in the temporal region, more left than right. On vHIT examination, abnormal responses were recorded bilaterally or unilaterally in five patients. Similarly, cVEMP revealed no response bilaterally or unilaterally in five patients. In three patients, the side of no response to cVEMP corresponded to the side of epileptiform pathology based on EEG. Two patients with bilateral abnormalities on EEG showed bilateral abnormalities either on cVEMP or vHIT, or on both. Taken together, these findings support the involvement of the brainstem connections of the peripheral vestibular system in vertiginous epilepsy. The pathological results of vestibular tests in the majority of our patients, combined with the EEG abnormalities, support the hypothesis of system epilepsies which is based on the dysfunction of specific neural systems.

Electroencephalography Findings in Crohn's Disease.

OBJECTIVES: Crohn's Disease (CD) is a chronic systemic inflammatory disease associated with various extraintestinal manifestations, including seizure as a neurological finding. In this study, the prevalence of seizure and electroencephalographic abnormalities in patients with CD was investigated. MATERIALS AND METHODS: This study involved 41 patients with CD (female/male: 25/16) and 39 subjects in the control group (female/male: 25/14). Patients in the CD group were diagnosed and monitored according to the European Crohn's and Colitis Organization diagnostic criteria. The control group was composed of healthy subjects with similar age and sex as the CD group. Seizures were classified according to the criteria of the International League Against Epilepsy. Electroencephalography (EEG) was performed for all patients with CD and for healthy subjects. Seizure prevalence and EEG findings were also compared. RESULTS: One patient in the CD group had history of seizures. EEG abnormality was significantly higher in the CD group (16/41, 39%) ( P = .001). The most common EEG abnormality was intermittent generalized slow wave abnormality in theta frequency. DISCUSSION: Our study indicated that CD was associated with EEG abnormalities rather than seizure. The results also indicated that EEG was a potential indicator for detecting subclinical neurological abnormalities in CD.

A case of perioral myoclonia with absences and its evolution in adulthood?

The rare syndrome of perioral myoclonia with absences (POMA) is described as a specific type of idiopathic generalized epilepsy in which absence seizures are accompanied by prominent perioral myoclonus as a consistent symptom. We present a 52-year-old man who was referred to our department due to treatment-resistant epilepsy. Typical seizures were described as rhythmic twitching of the lips which started at six years old, and his first convulsive seizure occurred at around 20 years old. Based on video-EEG recordings, we present two distinct EEG patterns accompanied by slight differences in clinical manifestations, which appear to be atypical of POMA. Firstly, consciousness was preserved during seizures, with no manifestation of absences. Secondly, regarding the EEG features, in some of the seizures, the perioral motor symptoms were tonic rather than myoclonic. The defining features of POMA are discussed in relation to this case.

The p.Thr11Met mutation in c19orf12 is frequent among adult Turkish patients with MPAN.

INTRODUCTION: Mutations in the C19orf12 gene cause mitochondrial membrane protein associated neurodegeneration (MPAN), an autosomal recessive form of neurodegeneration with brain iron accumulation (NBIA). A limited number of patients with C19orf12 mutations, particularly those with adult onset of symptoms, have been reported. METHODS: We sequenced the entire coding region of C19orf12 in 15 Turkish adult probands with idiopathic NBIA. We also performed haplotype analysis in families with a recurrent C19orf12 mutation. Clinical features were collected using a standardized form. RESULTS: Nine of our 15 probands (60%) carried the homozygous c.32C > T mutation in C19orf12 (predicted protein effect: p.Thr11Met). This homozygous mutation co-segregated with the disease in all affected relatives available for testing (16 homozygous subjects). Haplotypes across the C19orf12 locus were identical for a very small region, closest to the mutation, suggesting an old founder, or, two independent founders. The clinical phenotype was characterized by adult onset in most cases (mean 24.5 years, range 10-36), and broad spectrum, including prominent parkinsonism, pyramidal signs, psychiatric disturbances, cognitive decline, and motor axonal neuropathy, in various combinations. On T2- or susceptibility weighted-MRI images, all patients displayed bilateral hypointensities in globus pallidus and substantia nigra, without an eye-of-the-tiger sign; however, hyperintense streaking of the medial medullary lamina between the external and internal parts of globus pallidus was observed frequently. CONCLUSION: The C19orf12 p.Thr11Met mutation is frequent among adult Turkish patients with MPAN. These findings contribute to the characterization of this important NBIA form, and have direct implications for genetic testing of patients of Turkish origin.

Correlation between cVEMP and ABR for the Evaluation of Vestibular Migraine.

OBJECTIVE: Vestibular migraine (VM) is a clinical condition characterized by temporal overlap between vestibular symptoms and migraine. In this study, we aimed to determine the changes in vestibular myogenic potential (cVEMP) and auditory brainstem response (ABR) in patients with VM and migraine. MATERIALS AND METHODS: A total of 86 participants with no hearing loss or additional disease between the ages of 18 and 45 were enrolled in three different groups: group 1, VM; group 2, migraine without aura; and group 3, healthy controls. cVEMP and ABR were performed for all participants during attacks and attack-free periods. The differences between the right and left sides were calculated. RESULTS: There was no significant difference in cVEMP p13-n23 latencies between any of the groups. There were statistically significant differences related to cVEMP p13-n23 amplitudes between groups 1, 2, and 3. This significant difference originated from group 1 when compared with the other groups (p<0.05). When we compared the cVEMP results of patients with VM during attack and attack-free periods, a statistically significant decrease was determined in the p13-n23 amplitude values during the attack period (p<0.01). Additionally, when we compared group 1 and group 3, the wave V peak latencies in ABR were significantly prolonged in group 1 (p<0.05). CONCLUSION: cVEMP and ABR can be used as diagnostic criteria for patients with VM during attacks. Further studies with larger groups are needed to verify our findings.

High Plasma Pentraxin 3 Levels in Diabetic Polyneuropathy Patients with Nociceptive Pain.

Diabetic polyneuropathy is the most common neurologic complication of diabetes mellitus. Underlying mechanisms of diabetic polyneuropathy are related to various metabolic and inflammatory pathways. Pentraxin 3 (PTX3) is an acute phase protein that is produced locally at the inflammatory sites by several cell types. Thioredoxin binding protein 2 (TBP2) is a thioredoxin regulator involved in intracellular energy pathways and cell growth. We measured the plasma levels of PTX3 and TBP2 in type 2 diabetic patients (n = 27) with pain complaints and compared their levels with those of healthy age- and sex-matched subjects (n = 24). Moreover, the diabetic patients were divided into two groups using the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) pain scale: patients with nociceptive pain that is caused by tissue damage and patients with neuropathic pain that is caused by nerve damage. Patients with LANSS scores of < 12 were considered to have nocicceptive pain (n = 15), while patients with LANSS scores of ≥ 12 were considered to have neuropathic pain (n = 12). We found that PTX3 levels were significantly higher in diabetic patients compared to controls (p = 0.03), but there was no significant difference in the TBP2 levels. Importantly, patients with nociceptive pain had significantly higher PTX3 levels compared to patients with neuropathic pain (p < 0.05). Thus, plasma PTX3 levels can be helpful for discrimination of nociceptive pain from neuropathic pain in diabetic patients. We propose that PTX3 may contribute to the onset of nociceptive pain.