Presentation
Screaming was also present in patients #4,7, vocalizations were also present in patients #1, 3, 5, and prominent tachypnea was also present in patient #1. [sleepscience.org.br]
After 6 months post-surgery EDS had disappeared in the 9 patients who presented this complaint pre-operatively. [ncbi.nlm.nih.gov]
A physician and technicians were present throughout the recordings to assess vigilance and consciousness in case of seizures. [academic.oup.com]
Published on Mar 1, 2018 A 14-year-old boy presented to the University of Rochester Pediatric Movement Disorder clinic for abnormal movements of 1 year’s duration. He was referred to the Movement Disorder clinic for evaluation of complex tics. [youtube.com]
This case demonstrates the importance of a careful clinical evaluation and investigation of patients presenting with possible panic attacks, especially if panic attacks are treatment resistent or have atypical features (for example automatisms). [radiopaedia.org]
Entire Body System
- Crying
They may turn their eyes and head to one side or raise one arm, and may cry out. Status epilepticus is common in frontal lobe epilepsy. There are few well-defined frontal lobe seizure syndromes. [aboutkidshealth.ca]
Motor and vocal agitation are similar to that of the SMA with short repetitive thrashing, pedaling, thrusting, laughing, screaming and/or crying. [en.wikipedia.org]
The individual may also sing songs, produce different voice, groaning sounds or cry. This disease can be misunderstood as night terrors or night mares. [biomedpharmajournal.org]
[…] characterized by motor agitation and gestural automatisms; viscerosensory symptoms and strong emotional feelings often described; motor activity repetitive and may involve pelvic thrusting, pedaling, or thrashing, often accompanied by vocalizations or laughter/crying [emedicine.medscape.com]
Cardiovascular
- Tachycardia
Tachycardia and irregular breathing are evident. Fig. 3 ( A ) An ENW seizure. [academic.oup.com]
Autonomic hyperactivity was remarkable in many cases: tachycardia (four cases) and sustained tachypnea with tachycardia (one case) appeared synchronously with seizure onset or accompanied the movement artifacts. [sleepscience.org.br]
- Hypertension
Korb-Savoldelli V, Gillaizeau F, Pouchot J, Lenain E, Postel-Vinay N, Plouin P-F, Durieux P, Sabatier B. 2012 Validation of a French version of the 8-item Morisky medication adherence scale in hypertensive adults. J. Clin. [royalsocietypublishing.org]
Psychiatrical
- Suggestibility
Now, a study at the Cleveland Clinic in the US suggests that early brain surgery may be more successful for controlling seizures in patients with frontal lobe epilepsy. [epilepsyresearch.org.uk]
Results suggested minimal working memory deficits as assessed with performance-based measures. However, the BRIEF showed more working memory deficits suggesting that, on a daily life level, working memory problems seem to be associated with FLE. [tandfonline.com]
These findings suggest that motor network disturbances may in part be responsible for the motor deficits observed in FLE patients. [liebertpub.com]
Ictal video electroencephalographic (EEG) studies have revealed partial seizures originating from the frontal lobe but also in parts of the insula, suggesting a defect of a broader network. [genome.jp]
The electroclinical pattern of the phasic evolution of convulsive seizure suggests a focal onset seizure with secondary generalisation. [scicombinator.com]
- Olfactory Hallucination
Orbitofrontal cortex Impaired awareness, initial repetitive automatisms, olfactory hallucinations and illusions and autonomic features may be seen. [epilepsydiagnosis.org]
- Aggressive Behavior
Frontal lobe seizure characteristics can include aggressive behavior, agitation, screaming and exhibiting physical movements similar to bicycling. These seizures are usually brief. [uwhealth.org]
- Social Isolation
They report higher levels of anxiety and stress due to social isolation, discrimination, the unpredictability of their seizures and people's reactions to them as well as fear of injury, death and brain damage from their seizures. [en.wikipedia.org]
Face, Head & Neck
- Facial Grimacing
grimacing, vocalization, or speech arrest; seizures are frequently preceded by a somatosensory aura; complex automatisms, such as kicking, laughing, or pelvic thrusting, may be present; responsiveness often preserved Primary motor cortex - Usually focal [emedicine.medscape.com]
Motor symptoms : Facial grimacing and complex automatisms like kicking and pelvic thrusting Vocal symptoms : Laughing, yelling, or speech arrest. [5] Primary motor cortex The primary motor cortex has jacksonian seizures that spread to adjacent areas of [en.wikipedia.org]
Finally, in only 15% of the episodes was the first movement opening of the eyes, then moving the lower limbs (4%) and the head (5%); in 4%, seizures began with pelvic movements; in 2% of the cases, patients had a facial grimace or swallowed (2%); in only [academic.oup.com]
Neurologic
- Agitation
Frontal lobe seizure characteristics can include aggressive behavior, agitation, screaming and exhibiting physical movements similar to bicycling. These seizures are usually brief. [uwhealth.org]
[…] frontal lobe epilepsy (ADNFLE); Nocturnal frontal lobe epilepsy (NFLE) Description Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is characterized by nocturnal, frequent, brief and stereotypic seizures that include explosive onset, screaming, agitation [genome.jp]
These include using obscenities, jumping or pedaling movements, agitation of the upper limbs as if struggling, or rubbing the genitals. [epilepsyqueensland.com.au]
`Agitated' sleep-walking, in our opinion, often represents a useful diagnostic clue to NFLE. [academic.oup.com]
The latter group of seven patients with temporal lobe origin had anamnestic and clinical features that mirrored the clinical features found in the 18 patients with frontal onset, with agitated movements, high seizure frequency and absent history of febrile [sleepscience.org.br]
- Aura
Daytime dyskinesia, generalized seizures and auras may occur. Intellect is usually preserved or mildly reduced and psychiatric comorbidity may occur. [orpha.net]
All auras and seizure were recorded by VEEG monitoring system for preoperative analysis. [alliedacademies.org]
Record any auras your child has before a seizure. The aura may happen seconds before a seizure, or up to an hour before. Your child may feel, see, hear, or smell something. Examples include part of your child's body becoming hot. [drugs.com]
Frontal lobe seizures may begin with a brief aura, even when seizures occur from sleep. Seizures are typically brief, and can have prominent vocalization, bizarre behavior, urinary incontinence, and head and eye deviation. [epilepsydiagnosis.org]
[…] speech disturbances Supplementary motor area (SMA) - Typically involves unilateral or asymmetrical, bilateral tonic posturing; may be associated with facial grimacing, vocalization, or speech arrest; seizures are frequently preceded by a somatosensory aura [emedicine.medscape.com]
- Screaming
Frontal lobe seizure characteristics can include aggressive behavior, agitation, screaming and exhibiting physical movements similar to bicycling. These seizures are usually brief. [uwhealth.org]
[…] nocturnal frontal lobe epilepsy (ADNFLE); Nocturnal frontal lobe epilepsy (NFLE) Description Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is characterized by nocturnal, frequent, brief and stereotypic seizures that include explosive onset, screaming [genome.jp]
Signs and symptoms of frontal lobe seizures might include: Head and eye movement to one side Complete or partial unresponsiveness or difficulty speaking Explosive screams, including profanities, or laughter Abnormal body posturing, such as one arm extending [mayoclinic.org]
Motor and vocal agitation are similar to that of the SMA with short repetitive thrashing, pedaling, thrusting, laughing, screaming and/or crying. [en.wikipedia.org]
- Generalized Tonic-Clonic Seizure
[…] spike/wave on EEG, which may be difficult to distinguish from primarily generalized absence seizures; may present as generalized tonic-clonic seizures without obvious focal onset Physical examination in focal lobe epilepsy is typically normal but may [emedicine.medscape.com]
Altered resting state networks in epileptic patients with generalized tonic-clonic seizures. Brain research 1374, 134–141, https://doi.org/10.1016/j.brainres.2010.12.034 (2011). 22. Roland, J. L. et al. [nature.com]
For patients, we included the number of seizures (generalized tonic-clonic seizures and other seizure types separately) in the last three months according to a seizure calendar commonly used in clinical routine and current anticonvulsive treatment (number [royalsocietypublishing.org]
- Somnolence
In conclusion, an investigation of excessive daytime somnolence should be an integral part of each visit in patients with NFLE. [ncbi.nlm.nih.gov]
Only one patient had to stop carbamazepine because of somnolence and ataxia. [academic.oup.com]
Urogenital
- Incontinence
NFLE) Description Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is characterized by nocturnal, frequent, brief and stereotypic seizures that include explosive onset, screaming, agitation, stiffening, kicking or bicycling of the legs, and incontinence [genome.jp]
Seizures are typically brief, and can have prominent vocalization, bizarre behavior, urinary incontinence, and head and eye deviation. Frontal lobe seizures may be exclusively nocturnal and often cluster. [epilepsydiagnosis.org]
[…] seizures were recorded in 27 patients, including 2 cases of aura, 4 cases of bilateral asymmetric tonic seizure, 9 cases of unilateral tonic, 3 cases of hypermotor, 5 cases of GTCS, 4 cases of autonomic symptom, 4 cases of versive seizure, and 2 cases of incontinence [alliedacademies.org]
- Urinary Incontinence
Seizures are typically brief, and can have prominent vocalization, bizarre behavior, urinary incontinence, and head and eye deviation. Frontal lobe seizures may be exclusively nocturnal and often cluster. [epilepsydiagnosis.org]
Workup
[…] frontal lobe epilepsy frequently require invasive EEG monitoring On intracranial EEG, Ictal onset most often appears as a low-voltage, high-frequency discharge (ie, buzz), although rhythmic activity at alpha, theta, or delta frequencies may be seen See Workup [emedicine.medscape.com]
Treatment
Among the patients with drug-resistant NFLE, surgical treatment is considered a potential option. [ncbi.nlm.nih.gov]
Management and treatment The treatment of choice for ADNFLE includes use of carbamazepine (200-1,000 mg/day). Carbamazepine abolishes seizures in 20% of cases, and gives significant relief (at least 50 % seizure reduction) in another 48%. [orpha.net]
Researchers at the clinic studied the effects of surgery on more than 150 patients, all of whom were at different stages in their treatment for epilepsy. [epilepsyresearch.org.uk]
Learn more about epilepsy symptoms, treatment and diagnosis at UCLA » [neurosurgery.ucla.edu]
Treatment effectiveness can vary greatly amongst frontal lobe epilepsy patients. People with brain injuries or malformations often must take medication for the duration of their lives. [uwhealth.org]
Prognosis
Engel class I and Engel class II were defined as good prognosis, and Engel class III and Engel class IV were defined as poor prognosis. The ILAE classification system 1, 2 and 3 were defined as good prognosis, 4 and 5 were defined as poor prognosis. [alliedacademies.org]
Prognosis ADNFLE is lifelong but not progressive. As an individual reaches middle age, attacks may become milder and less frequent. The documents contained in this web site are presented for information purposes only. [orpha.net]
An important feature in prognosis is the early recognition of frontal lobe seizures as an epileptic syndrome rather than as a parasomnia or a psychiatric condition. Patient Education Patient education is important for all patients with epilepsy. [emedicine.medscape.com]
This not only requires early seizure control but also the recognition of the poor global prognosis of this nonlesional form of epilepsy, which should help us obtain special help from educational professionals for these children at the time of diagnosis [onlinelibrary.wiley.com]
[…] characterize brain connections in a larger sample of patients with FLE in an effort to provide deep insights into the clinical implications and resulting medical interventions utilized in FLE treatment, and may also contribute to better evaluating seizure prognosis [nature.com]
Etiology
We believe that in children, FLE, even without a known etiology, can not be considered benign. [onlinelibrary.wiley.com]
Etiology ADNFLE results from malfunction in the thalamo-cortical loops. The genes involved are CHRNA4 (20q13.33), CHRNB2 (1q21.3), CHRNA2 (8p21), KCNT1 (9q34.3), DEPDC5 (22q12.3), CRH (8q13), and CABP4 (11q13.2). [orpha.net]
Etiology Developmental lesions With improvements in neuroimagine, cortical dysplasias are increasingly being identified as epileptogenic lesions. This is particularly true for patiens who were initially assumed to be nonlesional. [emedicine.medscape.com]
Inclusion criteria were: a clinically confirmed cryptogenic (i.e., based on clinical presentation, EEG and MRI findings, presumed to be symptomatic, but with unknown etiology) localization-related epilepsy with an epileptic focus in the frontal lobe, [journals.plos.org]
Epidemiology
Summary Epidemiology Over 100 families have been described in the literature to date. Males and females are affected equally. Clinical description The age of onset varies between 3 and 47 years (usually < 20 years, with a peak during childhood). [orpha.net]
Sex predilection No significant sex-based frequency difference has been reported for frontal lobe epilepsy in epidemiologic studies. [emedicine.medscape.com]
Also, in regards to the question of whether any forms of epilepsy are different between Taiwanese and Caucasian populations (viz. epidemiology and clinical characteristics), a PubMed search did not reveal any identified differences. [sleepscience.org.br]
Further research in the genetic and epidemiological fields together with elucidation of the motor component of the nocturnal attacks by video-PSG, not only in sleep disorders but also in normal subjects, may help to define the border between these two [touchneurology.com]
«The epidemiology of the epilepsies: future directions, International League Against Epilepsy». Epilepsia (n.º 38, págs. 614-8). Jallon P.; Loiseau, P.; Loiseau J. (2001). [cv.uoc.edu]
Pathophysiology
An overview of epilepsy: its history, classification, pathophysiology and management. Brain and nerve=Shinkei kenkyu no shinpo 65, 509–520 (2013). 33. Yan, C. G., Wang, X. D., Zuo, X. N. & Zang, Y. F. [nature.com]
CrossRef Google Scholar × Access Volume 18, Issue S4 November 1991, pp. 559-563 Abstract: The first section of this article deals with specific anatomic and pathophysiologic factors which contribute to a poor EEG localization of the interictal epileptic [cambridge.org]
Low therapy adherence seems to have a multifactorial origin with some factors being associated with neuropsychological impairments and psychiatric co-morbidities of chronic diseases [ 11 ], and others reflecting pathophysiological changes of neural networks [royalsocietypublishing.org]
Pathophysiological conclusions NFLE has a peculiar relationship to the physiology of sleep. Polygraphic recordings show that a mesiofrontal epileptic focus is often activated during NREM sleep. [academic.oup.com]
Prevention
What can I do to help my child prevent a seizure? You may not be able to prevent every seizure. [drugs.com]
Medications [ edit ] Anticonvulsants are the most successful medication in reducing and preventing seizures from reoccurring. [en.wikipedia.org]
About the American Epilepsy Society Founded in 1946, the American Epilepsy Society (AES) is a medical and scientific society whose members are dedicated to advancing research and education for preventing, treating and curing epilepsy. [aesnet.org]
There is a large range of anticonvulsants which has both types of different abilities to prevent some types of seizures and modes of action. [biomedpharmajournal.org]
Controlling seizures as well as possible with medication appears to be the best prevention for SUDEP. Depression and anxiety. Both are common in people with epilepsy. [mayoclinic.org]