Original article with video sequences

Epileptic Disord 2013; 15 (1): 44-8

Head atonic attacks: a new

type of benign non-epileptic
attack in infancy strongly
mimicking epilepsy
Giuseppe Capovilla 1 , Alessandra Montagnini 1 ,
Cinzia Peruzzi 2 , Francesca Beccaria 1
1 Epilepsy Center, Department of Child Neuropsychiatry, C Poma Hospital, Mantova
2 Department of Child Neuropsychiatry , AOU Maggiore della Carità, Novara, Italy

Received August 21, 2012; Accepted December 7, 2012

ABSTRACT – Non-epileptic attacks (NEAs) are a heterogeneous group

of clinical entities which often complicate the differential diagnosis of
epilepsy. NEAs usually have a benign course and are limited to a specific
period of life. If motor manifestations are strongly suggestive of an epileptic
phenomenon, the risk of misdiagnosis is greater. Here, we describe a novel
NEA with infantile onset, characterised by repeated head drops, mimicking
epileptic negative myoclonus of the neck. The frequency of the episodes
was very high, at hundreds or thousands per day. The episodes appeared in
the second semester of the first year of life and spontaneously disappeared
within a few months. [Published with video sequences]
Key words: NEAs, non-epileptic attacks, infancy, paroxysmal disorders,
atonic attacks

The false diagnosis of epilep- Pachatz et al., 2002; Fujikawa et al.,

tic infantile spasms for patients 2003; Fejerman, 2008), and the exis-
with myoclonic manifestations has tence of this benign non-epileptic
been known since the seventies condition has been repeatedly
(Fejerman, 1977a; Fejerman, 1977b). confirmed. Polygraphic video-EEG
Similar cases were subsequently recordings of these non-epileptic
described worldwide (Vanasse et al., attacks (NEAs) has provided evi-
1976; Fejerman and Medina, 1977; dence of different types of motor
Fejerman, 1977b; Lombroso and manifestation and include: true
Fejerman, 1977; Giraud, 1982; Gobbi myoclonic activity, brief tonic acti-
et al., 1982; Fejerman, 1984; Holmes vity, loss of tone in the trunk, and
doi:10.1684/epd.2013.0548

Correspondence:
and Russman, 1986; Dravet et al., shuddering of the head, shoulders,
Giuseppe Capovilla 1986; Galletti et al., 1989; Caviedes and upper limbs or facial muscles
Epilepsy Center Altable et al., 1992; Pachatz et al., with a long duration of up to seve-
Dept of Child Neuropsychiatry 1999; Kanazawa, 2000; Maydell et al., ral seconds. A recent report by
C Poma Hospital
46100 Mantova, Italy 2001; Fernandez Alvarez and Aicardi, Caraballo et al. (2009) described a
<[email protected]> 2001; Fejerman and Caraballo, 2002; large group of infants with NEAs,

44 Epileptic Disord, Vol. 15, No. 1, March 2013

 Non-epileptic head atonic attacks

presenting with all four types of motor manifestation, Clinical manifestations

with rarely more than one type of motor phenomenon
in the same patient. Among these patients, cases of Both home videos and ictal video-EEG polygraphic
head drops were included, however, the head drops recordings were obtained for all patients. Ictal manifes-
were more prolonged and sustained, and often late- tations were stereotyped for all infants. The movement
ral, completely different from the cases presented was an abrupt loss of muscle tone of the neck, caus-
here (Caraballo, personal communication). Subse- ing a head drop (figure 1 and video sequences 1, 2,
quently, Capovilla (2011) described a large number and 3). Head drops were more or less intense and
of patients presenting with a peculiar clinical phe- accompanied by crying of the infant. In all cases, the
nomenon of non-epileptic nature and named this episodes occurred several times per day, characteristi-
condition “shaking body attacks”. More than 20 years cally in cluster, and tended to be present throughout
ago, Brunquell et al. (1990) described a group of 24 pae- the day. Triggering factors were not reported for any
diatric patients presenting with three different kinds of infants (table 1). None of the patients presented with
head drop, of both epileptic (17/24) and non-epileptic different types of attacks included in the so called
(7/24) nature. However, the seven cases with non- “spectrum of benign myoclonus of early infancy” or
epileptic attacks presented with different types of Fejerman syndrome.
epileptic seizures and were often mentally retarded.
Here, we describe three neurologically and men- Evolution and prognosis
tally normal non-epileptic patients, both at onset and
during follow-up. The patients presented with a novel For all the individuals, the episodes tended to reduce
NEA with infantile onset, characterised by repeated in frequency over time and disappeared by the end of
head drops that could be easily confused with an infan- the first year of life. The attacks persisted for a period
tile epileptic condition in the first year of life. lasting for five to six months. Language and cognitive
development were normal in all our cases.

Patients and methods Patient 1

A male patient was born with uneventful pregnancy
We reviewed the clinical charts and EEG data of and normal perinatal period. Growth and psychomo-
patients referred to the Epilepsy Center of Mantova tor development was normal. At 6 months and 3 weeks
between January 1999 and December 2010 for non- of age, the infant showed head dropping movements.
epileptic abnormal movements in the first year of Initially, these movements were observed rarely by the
life. Exclusion criteria were the presence of abnor- parents, but on some days they became very frequent
mal neurological examination or neuropsychological (20-30 per day). There was no triggering factor and
development prior to onset and interictal or ictal often episodes clustered without any circadian cor-
EEG epileptiform activity. We selected three patients relation. Episodes were recorded during repeat EEG
with similar features, with regards to both electro- performed at another epilepsy centre. Artefacts resem-
clinical manifestations and profile of evolution. In bling high-voltage, slow waves were considered to be
these cases, we evaluated: family and personal his- the EEG correlate of head drops and the infant was
tory of neurological disease, age at onset, pattern of believed to be epileptic. Levetiracetam was started
occurrence (isolated or in clusters), frequency per day, (20 mg/Kg/die), but episodes persisted. At the age of
time of day of occurrence, triggering factors, age at 9 months, the infant was referred to our epilepsy
disappearance, neuropsychological development, and centre where we recorded the episodes during a
association with developmental disorders. A minimum video-EEG polygraphic recording (video sequence 1)
follow-up period of 18 months from disappearance which revealed the non-epileptic nature of the attacks.
of non-epileptic abnormal movements was a required Levetiracetam was rapidly withdrawn and episodes
inclusion criterion. persisted until the age of 12 months when they
completely disappeared. Psychomotor development
was normal during follow-up at age 2 years and 10
months.
Results
Patient 2
General data
A female was born with uneventful pregnancy and
Data was collected for three patients (two female and normal perinatal period. Psychomotor profile was nor-
one male). The age at onset ranged from 6 to 7 months. mal. Febrile seizures were reported for the mother.
For one patient, there was a family history of febrile The patient presented with several episodes of head
convulsions. dropping per day at the age of 7 months. In some

Epileptic Disord, Vol. 15, No. 1, March 2013 45

G. Capovilla, et al.

Fp2 F4 ancora ripetuti

F4 C4
C4 P4
P4 O2
F8 T4
T4 T6
Fz Cz
Cz Pz
Fp1 F3
F3 C3
C3 P3
P3 O1
F7 T3
T3 T5
EMG1+ EMG1-
EMG2+ EMG2-
EMG3+ EMG3-
EMG4+ EMG4-
MKR+ MKR-

Figure 1. Case 3. Polygraphic recording of repeated head atonic attacks.

The EMG trace of the neck muscles (EMG1) is characterised by a flattening (ellipsis), not evident in the other muscles examined (right
deltoid EMG2, left deltoid EMG3, and right radial triceps EMG4). Note the absence of EEG correlate.

Table 1. Clinical data of patients.

Patient Age at Family Sex Age at Age at Persistence Isolated Series Frequency Triggering
follow-up history onset disappearance factors

1 2y 10m negative M 6m 3w 12m 5m 1w yes yes daily no

2 2y 11m FC F 7m 12m 5m yes yes daily no

3 10y negative F 6m 11m 5m yes yes daily no

M: male; F: female; FC: febrile convulsions; y: years; m: months; w: weeks.

instances, the infant presented with many episodes in 12 months, the episodes spontaneously disappeared.
a few minutes without triggering factors or circadian Psychomotor development was normal during follow-
correlation. Manifestations were recorded in another up at age 2 years and 11 months.
epilepsy centre during video-EEG monitoring and a
diagnosis of epileptic spasms was made. Vigabatrin Patient 3
was prescribed but head drops persisted, therefore A female patient was born with uneventful pregnancy
a second antiepileptic drug (valproic acid) was intro- and delivery. Growth and psychomotor development
duced, without any clinical amelioration. The parents were normal. At 6 months of age, the patient had
were referred to our centre for a second opinion when several episodes a day, characterised by shock-like
the infant was 10 months old. Several episodes were head drops. These were sometimes isolated but more
recorded (video sequence 2) and their non-epileptic characteristically repetitive, without triggering factors
origin was documented. Valproic acid and vigaba- or circadian correlation. The episodes were recorded
trin were stopped after a few weeks. At the age of at another epilepsy centre at the age of 8 months.

46 Epileptic Disord, Vol. 15, No. 1, March 2013

 Non-epileptic head atonic attacks

The manifestations captured by video-EEG monitor- high risk of recurrence of epileptic seizures. In conclu-
ing (video sequence 3 and figure 1) were evaluated sion, recognition of NEA is extremely important in
by a second epileptologist and deemed to be epilep- clinical practice due to the high risk of misdiagnosis
tic, and an antiepileptic treatment was proposed. of epilepsy. Video-EEG monitoring is critically impor-
We evaluated the video-EEG recording before ini- tant in the paediatric population with “refractory”
tiation of treatment and considered the episodes paroxysmal events to prevent misdiagnosis and poten-
to be non-epileptic. Treatment was never started. tially unwarranted exposure to drugs that might impair
Head atonic attacks persisted until 11 months of age. growth and development.

During follow-up at age 10, psychomotor development
was completely normal. Disclosures.
None of the authors has any conflict of interest or financial sup-
port to disclose in connection with the published text.
Differential diagnosis
The patients were referred following a diagnosis of Legends for video sequences
epileptic seizures, and for two cases antiepileptic
therapy had already been initiated. The high frequency
Video sequence 1
of attacks and occurrence in clusters, resembling
epileptic negative myoclonus, was suggestive of a false Nine-month-old infant. A sudden, shock-like head
diagnosis of epilepsy, in particular of West syndrome drop is evident. The head returns to initial posture
or infantile spasms. The EEG polygraphic recording very rapidly.
can be misleading because the EEG artefact induced
by the head drops might be considered as an ictal Video sequence 2
paroxysmal event. Other types of non-epileptic nega-
tive myoclonus typically occur in adult age and are This 10-month-old infant presents with three
observed in metabolic encephalopathies or vascular episodes of head atonia. The intensity of the attacks
accidents of thalamic or brainstem nuclei. is variable and the last one is very mild. Note that
the compensatory movement of the infant can be
of variable intensity.

Discussion Video sequence 3

The differential diagnosis between epileptic and Eight-month-old infant. The video sequence shows
non-epileptic attacks is extremely challenging for head atonic attacks both isolated and repeated.
epileptologists. Moreover, the problem is not only the Key words for video research on
differential diagnosis but also the diagnosis per se. The www.epilepticdisorders.com
cases described here represent a new type of NEA in Syndrome: non epileptic paroxysmal disorder
infancy and this entity should be carefully consider- Etiology: not applicable
ed for the differential diagnosis of West syndrome. In Phenomenology: atonic seizure (drop attack);
fact, this type of NEA can be easily misdiagnosed as myoclonus (negative)
an epileptic condition due to a number of different Localization: not applicable
contributing factors: firstly, the lack of description of
similar cases with adequate video-EEG documentation
in the literature; secondly, EEG artefact caused by head
drops can be misinterpreted as an EEG abnormality or
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