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
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Crying
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]
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]
[…] 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]
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]
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Shivering
These include feelings of oppression, shivering, and a sensation of body heat. Autonomic symptoms such as palpitations, choking, pallor, flushing, salivation and migraine like sensations are also common. [epilepsyqueensland.com.au]
The common symptoms of people suffering from ADNFLE are shivering, touchy, being pushed or feeling of falling ,a sense of fear. Some people also reported a feeling of choking, breathlessness or fast breathing. [biomedpharmajournal.org]
Respiratoric
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Tachypnea
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]
Gastrointestinal
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Choking
Autonomic symptoms such as palpitations, choking, pallor, flushing, salivation and migraine like sensations are also common. Occasionally bizarre behaviour and complex motor activities can sometimes be observed. [epilepsyqueensland.com.au]
Some people also reported a feeling of choking, breathlessness or fast breathing. It is still unclear that what brings seizures in individual with ADNFLE. Seizures that dominate male is NFLE(7:3). [biomedpharmajournal.org]
Some symptoms of sleep apnea overlap with those of ADNFLE, such as sudden awakening accompanied by a feeling of choking and on occasion motor activity which makes diagnosis difficult based on symptoms alone. [en.wikipedia.org]
Cardiovascular
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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]
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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
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Behavior Disorder
Differential diagnosis Differential diagnoses include paroxysmal dyskinesia, familial focal epilepsy with variable foci, restless legs syndrome, periodic limb movement disorders (PLMS), REM sleep behavior disorders (RBD), nocturnal panic attacks, non-REM [orpha.net]
The authors emphasized that the FLEP scale was particularly suited in distinguishing NFLE from NREM parasomnias more than in distinguishing NFLE from REM sleep behavior disorder. [sleepscience.org.br]
Persistent disruption in the bilateral vmPFC produced by frequent seizures, led to reversible interictal antisocial behavioral disorders 27. [nature.com]
Chronic behavioral disorders of human REM sleep: a new category of parasomnia. Sleep 1986 ; 9 : 293 –308. Sforza E, Montagna P, Rinaldi R, Tinuper P, Cerullo A, Cirignotta F, et al. [academic.oup.com]
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Olfactory Hallucination
Orbitofrontal cortex Impaired awareness, initial repetitive automatisms, olfactory hallucinations and illusions and autonomic features may be seen. [epilepsydiagnosis.org]
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Withdrawn
In five cases, carbamazepine was withdrawn because it was ineffective, and phenytoin (in two) or clobazam (in three, in one case associated with valproic acid) were instituted, without any significant efficacy. [academic.oup.com]
Face, Head & Neck
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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
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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]
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]
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]
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Somnambulism
The mean lifetime duration of somnambulism is 7 years ( Sours et al ., 1963 ), compared with 20 12 years in the case of our patients with NFLE. [academic.oup.com]
[…] seizures, consisting of stereotypic sudden arousals that recur throughout the night in a periodic pattern, to more elaborate seizures, with complex dystonic and dyskinetic phenomena, or to longer seizures consisting of aimless wandering, simulating somnambulic [ncbi.nlm.nih.gov]
Dissociative disorders presenting as somnambulism: polysomnographic, video and clinical documentation (8 cases). Dissociation 1989;2:194-204. [24] Mahowald MW, Schenck CH. Nocturnal dissociation—Awake? Asleep? Both? Or neither?[editorial]. [sleepscience.org.br]
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Night Terrors
Diagnosis of NFLE It is quite difficult to diagnose the frontal lobe epilepsy because the symptoms are same like sleep disorder or night terror and psychiatric problems. [biomedpharmajournal.org]
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Dysphasia
Dorsolateral frontal cortex In the dominant hemisphere, a seizure occurring in or near Broca's area can result in aphasia or dysphasia in a patient who is otherwise awake and responsive. [epilepsydiagnosis.org]
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Jacksonian Seizure
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]
Urogenital
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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]
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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
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]
Among the patients with drug-resistant NFLE, surgical treatment is considered a potential option. [ncbi.nlm.nih.gov]
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]
[…] impairment within the motor-controlled system including the cerebellum and the caudate/putamen in FLE patients, this therefore suggest that the deficits in GMV at the caudate and putamen as well as their causality would enhance understanding in the prognosis [bmcneurol.biomedcentral.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]
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, aged between 8 and 13 years, no other disease that could cause cognitive [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]
Enuresis, sleepwalking, and nightmares: an epidemiological survey in the Republic of San Marino. In: Guilleminault C, Lugaresi E, editors. Sleep/wake disorders: natural history, epidemiology, and long-term evolution. [academic.oup.com]
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
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]
Both perfusion and agonist solution contained no Cl ions to prevent contamination with endogenous Ca 2 -dependent Cl current. Oocytes were preincubated in Cl -free media for 4–16 hr. [jneurosci.org]
What can I do to help my child prevent a seizure? You may not be able to prevent every seizure. [drugs.com]