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A child with ictal vocalizations and generalized epilepsy
Epileptic Disorders
Abstract
Ictal vocalizations in the form of both articulate speech and non-speech vocalizations have been described in focal epilepsies, with seizures originating mainly from the frontal and temporal lobe, however, this phenomenon has not been described in generalized epilepsies. We report the case of an adolescent boy with juvenile-onset generalized epilepsy who presented with ictal "ovine vocalizations" (resembling the bleating of sheep). The ictal EEG revealed a clear correlate of vocalizations with time-locked generalized spikes and polyspike discharges. The 3T cerebral MRI ruled out any focal lesion. The boy is currently seizure-free under valproic acid, after twelve months of follow-up. We conclude that ictal non-speech vocalizations may be observed not only in focal or structural epilepsies, but also in generalized epilepsies; the exact underlying mechanism of this phenomenon needs to be further delineated. [Published with video sequence].
... The guttural, expiratory sound known as the "ictal cry" is a stereotypical feature of generalized tonic-clonic seizure (GTCS). 4,5 During the tonic phase, contraction of the axial and abdominal muscles causes the diaphragm to slowly force air through the vocal cords giving rise to the ictal cry. Ictal vocalizations, which include both intelligible speech and nonspeech sounds, are frequently observed during focal seizures. ...
Objective
Ictal vocalizations have shown diagnostic utility in epilepsy patients. Audio recordings of seizures have also been used for seizure detection. The present study aimed to determine whether generalized tonic–clonic seizures in the Scn1a+/− mouse model of Dravet syndrome are associated with either audible mouse squeaks or ultrasonic vocalizations.
Methods
Acoustic recordings were captured from group‐housed Scn1a+/− mice undergoing video‐monitoring to quantify spontaneous seizure frequency. We generated audio clips (n = 129) during a generalized tonic–clonic seizure (GTCS) that included 30 seconds immediately prior to the GTCS (preictal) and 30 seconds following the conclusion of the seizure (postictal). Nonseizure clips (n = 129) were also exported from the acoustic recordings. A blinded reviewer manually reviewed the audio clips, and vocalizations were identified as either an audible (<20 kHz) mouse squeak or ultrasonic (>20 kHz).
Results
Spontaneous GTCS in Scn1a+/− mice were associated with a significantly higher number of total vocalizations. The number of audible mouse squeaks was significantly greater with GTCS activity. Nearly all (98%) the seizure clips contained ultrasonic vocalizations, whereas ultrasonic vocalizations were present in only 57% of nonseizure clips. The ultrasonic vocalizations emitted in the seizure clips were at a significantly higher frequency and were nearly twice as long in duration as those emitted in the nonseizure clips. Audible mouse squeaks were primarily emitted during the preictal phase. The greatest number of ultrasonic vocalizations was detected during the ictal phase.
Significance
Our study shows that ictal vocalizations are exhibited by Scn1a+/− mice. Quantitative audio analysis could be developed as a seizure detection tool for the Scn1a+/− mouse model of Dravet syndrome.
... 199 Another important category encompasses ictal phenomena. Ictal vocalizations (also see previous sections) may inherently cover the entire tenor of possible sounds and phonemes of humans: from the classic "ictal cry," signifying the beginning of generalized tonic-clonic seizures, 200 over echo-, pali-, and coprolalic 145,[201][202][203][204] behaviors, to animal noises ("bleating of sheep," barking), 205,206 singing, and humming. [207][208][209] Of note, weeping, moaning, and coughing may also be encountered in nonepileptic seizures. ...
In clinical practice, involuntary vocalizing behaviors are typically associated with Tourette syndrome and other tic disorders. However, they may also be encountered throughout the entire tenor of neuropsychiatry, movement disorders, and neurodevelopmental syndromes. Importantly, involuntary vocalizing behaviors may often constitute a predominant clinical sign, and, therefore, their early recognition and appropriate classification are necessary to guide diagnosis and treatment. Clinical literature and video‐documented cases on the topic are surprisingly scarce. Here, we pooled data from 5 expert centers of movement disorders, with instructive video material to cover the entire range of involuntary vocalizations in humans. Medical literature was also reviewed to document the range of possible etiologies associated with the different types of vocalizing behaviors and to explore treatment options. We propose a phenomenological classification of involuntary vocalizations within different categorical domains, including (1) tics and tic‐like vocalizations, (2) vocalizations as part of stereotypies, (3) vocalizations as part of dystonia or chorea, (4) continuous vocalizing behaviors such as groaning or grunting, (5) pathological laughter and crying, (6) vocalizations resembling physiological reflexes, and (7) other vocalizations, for example, those associated with exaggerated startle responses, as part of epilepsy and sleep‐related phenomena. We provide comprehensive lists of their associated etiologies, including neurodevelopmental, neurodegenerative, neuroimmunological, and structural causes and clinical clues. We then expand on the pathophysiology of the different vocalizing behaviors and comment on available treatment options. Finally, we present an algorithmic approach that covers the wide range of involuntary vocalizations in humans, with the ultimate goal of improving diagnostic accuracy and guiding appropriate treatment.
Nonspeech vocalizations are usually seen in temporal or frontal lobe epileptic focus, especially in the dominant side of the brain. There can be screaming, hooting, barking, humming grunting, etc., Barking is most characteristic, which is commonly seen in mesial frontal lobe epilepsy. In this report, we describe a unique case of ictal barking with onset at 18 years of age mistaken for unspecified psychotic disorder, which was subsequently diagnosed as complex partial seizure following electroencephalogram findings and was maintained on valproate 1500 mg/day, and levetiracetam 1500 mg/day was added along with clobazam 10 mg/day. Ictal barking is mostly focal, similar to our case; however, there has been sparse evidence of ictal barking in generalized seizures as well.
Introduction
Aphasic and other language disturbances occur in patients with epilepsy during and after epileptic seizures. Moreover, the interictal language profile in these patients is heterogeneous, varying from normal language profile to impairment in different language functions. The aim of this paper was to critically review the terms and concepts of ictal language alterations.
Material and method
For this review we performed an extensive literature search on the term “epileptic aphasia” and analyzed the semiology and terminology indicating language-associated seizure symptoms. In addition, we give an overview on EEG, etiology, and brain imaging findings and ictal language disorders.
Results
In the literature, a plethora of terms indicates language-associated seizure symptoms. Simultaneous Video-EEG monitoring represents the gold standard to correctly classify ictal versus postictal language disturbances and to differentiate aphasic symptoms from speech automatisms. Different rhythmic and periodic EEG patterns associated with ictal language disturbances are recognized. Cerebral magnetic resonance imaging (cMRI) is essential in the diagnosis of seizures and epilepsy. Brain tumors and acute or remote cerebrovascular lesions are the most frequently reported structural etiologies underlying ictal language alterations. However, it has to be recognized that brain imaging may show alterations being the consequence of seizures itself rather than its cause. Functional brain imaging might be informative in patients with inconclusive EEG and MRI findings. Overall, seizure-associated aphasia is reported to have good lateralizing significance.
Conclusion
Various language disturbances are caused by different types of seizures, epilepsies and underlying etiologies. In the clinical context, simultaneous Video-EEG monitoring facilitates precise classification of ictal versus postictal language alterations and differentiation of aphasic symptoms from speech automatisms.
The case of a 32-year-old woman with Down syndrome (DS) with new-onset abnormal vocalizations as a manifestation of gastroesophageal reflux disease (GERD) is described highlighting the clinical features, differential diagnosis, work-up, results, and treatment course. A broad differential diagnosis, including the contribution of common medical co-morbidities, should be considered in patients with DS and other neurodevelopmental disorders presenting with new-onset abnormal vocalizations. This is especially important for patients with impaired communicative and cognitive ability that may have difficulty describing physical pain and/or emotional distress.
Vocalizations may occur in focal epileptic seizures, which typically arise from frontal and temporal regions. They are commonly associated with other motor phenomena such as automatisms, tonic posturing, or head version. We report on a patient whose seizures were documented by video-EEG monitoring, but in whom the observable ictal semiology consisted solely of a brief, monotonous vocalization. Ictal EEGs showed left frontal seizure patterns. Isolated vocalizations can constitute an ictal epileptic event and may be the only observable clinical manifestation of a left frontal lobe epilepsy. [Published with video sequences].
A 75-year-old right-handed man was admitted to our emergency department complaining of recurrent episodes of involuntary 'barking' within the past 12h. The episodes had occurred after an initial two-minute attack from sleep involving tonic contraction of the upper extremities and jaw locking. By the time of admission, the patient had had a total of at least 7-10 'barking' episodes, each lasting 30-45 s. Seven months prior to his current admission, the patient had had a minor ischemic stroke causing mild left paresis, which had resolved completely. His awake EEG revealed a normal background pattern interrupted by runs of two per second slow waves mixed with low-voltage spikes in the left temporal lobe with a left mid-temporal emphasis. The patient was diagnosed with recurrent simple partial seizures, and treatment with intravenous valproic acid was initiated. He was discharged four days later without having experienced any further barking episodes. Atypical presentations of the epileptic seizures have been described in the literature, but ictal barking is very rare manifestation of epilepsy.
Ictal nonspeech vocalizations have been described as manifestations of either frontal or temporal epileptogenicity originating mainly from the dominant hemisphere. Ictal barking, particularly, has been considered a manifestation of mesial frontal epilepsy. A 42-year-old right-handed male with posttraumatic drug-resistant complex partial epilepsy manifested ictal barking near electrographic onset. Extraoperative electrocorticography with subdural electrode coverage of the right frontoparietal and temporal and left frontal surfaces provided surveillance of ictal origin and propagation. Ictal origin was identified in the right mesial temporal lobe with barking vocalization manifesting within 3s of electrographic onset. No subsequent spread of activity was noted beyond the temporal lobe. Resection of the mesial temporal structure resulted in seizure freedom. Pathology identified hippocampal sclerosis. This case supports the notion that an intrinsic, intralobar epileptogenic neural network in either hemisphere can act as a conduit into the limbic and memory circuits without a laterality bias to manifest as barking.
To investigate the lateralization value of ictal vocalizations in temporal lobe epilepsy (TLE).
We reviewed video-recordings of 97 patients who had undergone presurgical evaluation programs with video-EEG (electroencephalography)-recorded complex partial seizures (CPS) and high-resolution magnetic resonance imaging (MRI). All patients had surgery due to TLE and became seizure-free. In 57 patients, determination of speech dominance was necessary by using Wada tests or functional MRI (fMRI). To reevaluate the archived seizures, we reviewed one to three consecutively recorded CPS of each patient. Altogether 223 archived seizures were analyzed. Ictal vocalization was considered to be present in a particular patient if it occurred in at least one of the recorded seizures.
Ictal vocalizations occurred in 22 patients. They occurred in 37% of left-sided and in 11% of right-sided patients with TLE (p = 0.003). In patients with determined speech lateralization, ictal vocalizations occurred in 37% of the dominant and in 14% in patients with nondominant epileptogenic zone (p = 0.04). In patients with ictal vocalizations, epilepsy began at age 8.7 +/- 6, whereas in the remaining patients, epilepsy started at age 14.0 +/- 9 (p = 0.017). Logistic regression showed that both hemispheric dominance and age at onset were independently associated with pure ictal vocalization (PIV).
Ictal vocalization is a frequent phenomenon, occurring in 23% of patients with TLE. It is more often associated with left-sided and early onset TLE. Our results may improve the lateralization of the epileptogenic zone and suggest that nonspeech vocalizations in humans are related to the dominant (left-sided) hemisphere. Our study is a further argument that there are different subtypes of TLE depending on the age at onset.
To evaluate the role of speech manifestations in lateralization of temporal lobe seizures, we reviewed videotapes of 100 complex partial seizures in 35 patients who underwent temporal lobectomy for intractable epilepsy. All patients had prolonged electroencephalographic video monitoring with scalp and subdural electrodes, and their speech dominance was determined with an intracarotid amobarbital test. Speech manifestations were observed in 79 seizures and were classified as vocalization, normal speech, or abnormal speech. Vocalization of sounds without speech quality occurred ictally in 48.5% of patients. Normal speech (identifiable speech) occurred ictally in 34.2% of patients. Abnormal speech (speech arrest, dysphasia, dysarthria, and nonidentifiable speech) occurred in 51.4% of patients, either ictally or postictally. Of all the above speech manifestations, only postictal dysphasia and ictal identifiable speech had significant lateralizing value: 92% of patients with postictal dysphasia had their seizures originating from the dominant temporal lobe (p less than 0.001), and 83% of those with ictal identifiable speech had their seizures from the nondominant side (p = 0.013). This study shows that speech manifestations are common in complex partial seizures of temporal lobe origin and can provide an excellent clinical tool for lateralization of seizure onset.
In 84 patients with complex partial seizures evaluated by EEG and video telemetry, who went on to have seizure surgery, complex intraictal vocalizations and postictal language dysfunction were found to have strong lateralizing significance. Thirteen patients had ictal speech, and 12 of these had right-sided seizure foci. Twenty-eight had intact postictal language, and 21 of these had right-sided foci. Of the 32 with impaired postictal language, 21 had left-sided foci.
The purpose was to analyse whether non-speech vocalisations in seizures originating in the frontal lobe do have lateralising value. Patients were included who had undergone presurgical evaluation with ictal video-EEG monitoring at the Epilepsy Centre, had had resective epilepsy surgery involving the frontal lobe, and who had remained seizure free>1 year postoperatively. Twenty seven patients aged 1-42 years (mean 18) met the inclusion criteria. Age at epilepsy onset ranged from 1 month to 41 years (mean 7.1 years). All selected patients had a unilateral MRI detected lesion within the frontal lobe. Fifteen patients had right sided, 12 patients had left sided epileptogenic zones. Seizures recorded during EEG-video monitoring were re-evaluated to identify the occurrence of ictal vocalisations. Pure ictal vocalisations were distinguished from ictal sound productions due to motor or vegetative seizure activity (for example, cloni or respiratory sounds). Pure ictal vocalisation occurred in 11 patients of whom nine had a left frontal epileptogenic zone (p<0.01). It is concluded that ictal vocalisation could be an additional lateralising sign in frontal lobe epilepsy. The results suggest that not only speech, but vocalisation at a subverbal level also shows a left hemispheric dominance in humans.
Humming is a rare automatism occurring in partial seizures that has received little attention. Its study could shed light on the neural networks underlying melodic expression. In this study, we examined the anatomoelectroclinical correlates of humming during epileptic seizures
Three patients undergoing presurgical stereoelectroencephalography (SEEG) for medically intractable temporal lobe epilepsy were studied. Coherence analysis of SEEG activity was carried out to study the functional coupling of different regions of the brain, whereas time-frequency (TF) analysis was conducted to assess epileptic discharge patterns. Changes in coherence were studied to identify the neural structures/systems implicated in humming.
Humming began after the onset of seizures generated in medial limbic regions of the temporal lobe. At seizure onset, coherence analysis showed an increase in amygdala-hippocampus coupling. Humming began after the onset of a rhythmic discharge over lateral regions of the superior temporal gyrus (STG). A highly significant increase in coherence was observed between prefrontal regions and the STG. TF analysis of the STG discharge showed a reproducible pattern with a single fundamental frequency and associated harmonics. This frequency was approximately 6 Hz for two patients and 15 Hz for one patient.
These findings suggest that the occurrence of humming during epileptic seizures of the temporal lobe is associated with activity in a neural network involving the STG and the inferior frontal gyrus.
We systematically analyzed the lateralizing value of clinical seizure semiology in patients with frontal lobe epilepsy (FLE).
We studied the incidence, positive predictive value (PPV), and the lateralizing significance of various clinical symptoms in 228 seizures (s) of 31 patients (p) with medically refractory FLE (17 with left-sided and 14 with right-sided seizure onset). Seizures recorded during prolonged video-EEG monitoring were assessed by two independent reviewers blinded for the patient's clinical data. Analysis was performed both for patients and seizures.
Version [16 p (52%); PPV, 94%; p=0.001; 47 s (21%); PPV, 75%; p=0.001], unilateral clonic movements [16 p (52%); PPV, 81%; p=0.021; 32 s (14%); PPV, 81%; p=0.001], unilateral dystonic posturing [eight p (26%); PPV, 75%; p=0.289; 46 s (20%); PPV, 80%; p=0.001], unilateral tonic posturing [10 p (32%); PPV, 80%; p=0.109; 19 s (7.4%); PPV, 79%; p=0.019], and unilateral grimacing [10 p (32%); PPV, 100%; p=0.002; 19 s (8%); PPV, 100%; p=0.001] were of lateralizing significance, indicating a contralateral seizure onset. Asymmetric ending [five p (16%); PPV, 80%; p=0.375; nine s (4%); PPV, 89%; p=0.039] after secondarily generalized tonic-clonic seizures was significantly associated with an ipsilateral seizure onset. Pure ictal vocalizations occurred significantly more frequently in seizures of right hemispheric onset [13 p (42%); PPV, 62%; p=0.581; 63 s (28%); PPV, 73%; p=0. 001], whereas in individual patients, this symptom showed no lateralizing significance. The remaining clinical symptoms (figure 4 sign, unilateral hand automatisms, early head turning, postictal nose wiping, and unilateral eye blinking) were not of lateralizing significance in our patients. The results of clinical seizure lateralization corresponded with the final lateralization of the seizure-onset zone in 81% of our patients.
Clinical seizure semiology can provide correct information on the lateralization of the seizure-onset zone in >80% of patients with medically refractory frontal lobe epilepsy.
Humming as an automatism in absence seizure: A report of 2 cases. Abstract for AES meeting 1999
- Laoprasert P
Laoprasert P. Humming as an automatism in absence seizure:
A report of 2 cases. Abstract for AES meeting 1999. Epilepsia
1999; 40(7): 2,083.
Video-EEG showing photosensitivity at 60 Hz with one episode of vocalization (a bleating sound) correlating with time-locked discharges of generalized spikes and polyspikes
- H Luders
- D Dinner
- H Morris
- E Wyllie
Video-EEG showing photosensitivity at 60 Hz with one episode of vocalization (a bleating sound) correlating
with time-locked discharges of generalized spikes and polyspikes.
Key words for video research on www.epilepticdisorders.com
Syndrome: juvenile myoclonic epilepsy
Etiology: unknown
Phenomenology: ictal vocalizations
Localization: generalised
Gabr M, Luders H, Dinner D, Morris H, Wyllie E. Speech manifestations in lateralization of temporal lobe seizures. Ann
Neurol 1989; 25(1): 82-7.