Magnetic resonance imaging findings in stroke patients with convulsive onset.

LETTERS
Idiopathic
Hypoparathyroidism and
Paroxysmal Dystonic
Choreoathetosis
Gabor Barabas, MD,* and Samuel M. Tucker, MDt
Micheli and associates [l) in a recent report in Annals described a patient with basal ganglia calcification and sporadic
paroxysmal dystonic choreoathetosis without hypoparathyroidism. In a Letter to the Editor, Yamamoto and Kawazawa
[ 2 ] pointed out that they had previously reported a similar
case but with hypocalcemia.
We are aware of an earlier report on idiopathic hypoparathyroidism [ 3 ] in a 13-year-old boy with curious attacks
while playing football. He was found to have low serum
calcium levels, and on skull roentgenogram “patchy cerebral
calcification” was observed “situated in the region of the
basal ganglia.” By description his attacks were not ictal phenomena per se but episodes of dystonia with risus sardonicus.
Several years ago we saw a 12-year-old boy with dizzy
spells. These occurred upon standing up or upon initiation of
movement such as beginning to run or walk rapidly. His
mother had witnessed several episodes; she said he developed a strange smile on his face after which the right hand
and arm drew up slowl!~in the flexed position, and he bowed
his head. Episodes lasted 15 to 30 seconds and could occur
up to 20 times a day. O n neurological examination there
were no abnormalities. When the patient was asked to sit for
a minute and then suddenly stand up and walk about the
chair in a clockwise ciirection, an attack was precipitated.
There was no overt loss of consciousness. An electroencephalogram in the waking state revealed normal background activity with occasional low voltage spike and spike-slow wave
complexes emanating from the right frontal region. The diagnosis of “reflex movement epilepsy” was made and phenytoin was prescribed. The frequency of episodes decreased
dramatically. After several weeks, he developed massive myoclonic spasms of the legs that would throw him to the
ground without loss of consciousness. Because of this “atypical response,” a workup was undertaken and it was found
that his serum calcium level was 5.6 and his serum phosphorus 11.0 mg/dl. The computed tomographic scan (Fig)
revealed bilateral calcification of the basal ganglia and
calcification of both frontal regions and the right cerebellar
hemisphere.
Ergocalciferol, 25,000 units twice a day, calcium lactate,
and basal jel were prescribed. Serum calcium concentration
increased to 7.4 mg/dl and all attacks ceased. Six months later
he was doing well.
It is likely that the enigmatic condition of “sporadic
paroxysmal dystonic c:noreoathetosis” in association with hypocalcemia, hypoparathyroidism, and basal ganglia calcification represents a distinct syndrome. It should be distinguished from familial paroxysmal choreoathetosis originally
described by Mount and Reback [4], a familial tendency
without hypocalcemia. In addition, all four cases raise the
Coronal CT scan without contrast, showing prominent symmetrical calcificationsand basal ganglia.
issue of the relationship of sporadic paroxysmal dystonic
choreoathetosis to “movement reflex epilepsy” and whether
in this clinical setting one may be dealing with semantics.
Some authors have pointed out that when movement precipitates the episode it is best referred to as a reflex epilepsy,
while paroxysmal dystonic choreoathetosis is generally not
precipitated by movement but, as is seen in the familial form,
by emotional upset, coffee, and fatigue.
*West Long Branch, NJ
fChildren’s Hospital of Philadelphia
Philadelphia, PA
References
1. Micheli F, Bardal MMF, Parera IC, Giannaula R. Sporadic
paroxysmal dystonic choreoathetosis associared wirh basal ganglia
calcifications. Ann Neurol 1986;20:750
2. Kawazawa S, Nogaki H, Hara T, et al. Paroxysmal dystonic
choreoarhetosis in a case of pseudoidiopathic hypoparathyroidism. Clin Neurol 1985;25:1152-1158
3. Arden F. Idiopathic hypoparathyroidism. Med J Australia 1053;
2:2 17-2 19
4. Mount LA, Reback S. Familial paroxysmal choreoathetosis. Arch
Neurol Psychiatry 1940;44:841
Magnetic Resonance
Imaging Findings
in Stroke Patients
with Convulsive Onset
Ronald Kramer, M D
I read with interest the useful study done by D r Pavlakis and
others on magnetic resonance imaging (MR1) findings in patients with suspected infarction and sickle-cell anemia [I]. I
take exception with them for including in their stroke group
patients who had a convulsive onset. Three of 18 patients in
their stroke group presented with focar convulsive disorders.
Copyright 0 1988 by the American Neurological Association
585
Ten of the 18 stroke patients did not have repeat scans. It is
unclear whether the 3 patients with convulsive onset had
repeat scans. Perhaps the so-called infarction seen on the first
scan in the 3 patients with convulsive onset was a transient
abnormality related to the convulsive event and did not represent an infarction.
Focal transient abnormalities on computed tomographic
and MRI scans have been documented in patients with focal
seizure disorders E2-51. These abnormalities are felt to represent transient edema and are confined primarily, but not
exclusively, to the white matter. Their MRI appearance is
very difficult to differentiate from cerebral infarction 121.
These abnormalities are known to occur in patients even
after a first seizure {3]. The appearance and subsequent disappearance of these lesions have been correlated with the
appearance and disappearance of postictal deficits [4].If a
patient has a postictal deficit from a seizure and an abnormal
imaging study, then a diagnosis of cerebral infarction is usually entertained. It is only in retrospect, after the scans and
clinical deficits resolve, that the correct diagnosis is made and
the misdiagnosis of stroke is revealed [ 2 , 4 , 51. Resolution o f
abnormal scans usually occurs within 4 weeks. The illustrations and descriptions of the 3 cases presented by Pavlakis
and coworkers fit with such transient abnormalities. If followup scans were not performed on these 3 patients, and
clinical deficits resolved, then the interpretation of the scans
is in doubt and the patients should not have been included in
the stroke group.
Oregon Comprehensive Epilepsy Program
Good Samaritan Medical Center
Portland, OR
scans 7 months apart; both showed left middle cerebral artery distribution and right posterior watershed infarcts. The
third patient presented with a seizure and had a normal
neurological examination. This patient had 2 MRI scans 1
year apart; both showed left basal ganglia and left deep watershed infarcts. The 2 patients who had repeat scans showed
persistent lesions. W e feel that the 3 patients belong in the
stroke group.
We agree with D r Gamer’s premise that one should be
cautious in making a diagnosis of infarction on the basis of
either an MRI abnormality or focal neurological findings that
may be transient. Transient abnormalities have been seen
on cerebral angiography, brain scans, and computed tomographic/MRI scans in patients with seizures 12, 31. The
pathophysiology of such abnormalities is open to debate.
Are these small strokes with seizures or seizures with secondary cerebrovascular or metabolic alterations?
For the present, we agree with D r Kramer that, to be
conservative, the diagnosis of stroke in a patient with a convulsive onset and MRI abnormality should await serial scans
that show persistent radiological abnormality. To extend the
question, is the presence of persistent MRI abnormality necessary to define stroke; can patients with either acute convulsive or nonconvulsive onset, persistent focal neurological
deficit, and normal MRI have stroke?
Departments of Neurology, Pediutritr Radiology. and Piychidtq
College of Physicians and SurgeonJ
Columbia University
New York, NY
References
References
1. Pavlakis SG, Bello J, Prohovnik I, et al. Brain infarction in sicklecell anemia: magnetic resonance imaging correlates. Ann Neurol
1988;23:125-130
2. Kramer RE, Luders H, Lesser RL, et al. Transient focal abnormalities of neuroimaging studies during focal status epilepticus.
Epilepsia 1987;2 8(5 ): 52 8-5 3 2
3. Dillon W, Brant-Zawadzki M, Sherry RG. Transient computed
tomographic abnormalities after focal seizures. AJNR 1984;5:
1. Pavlakis SG, Bello J, Prohovnik I , et al. Brain 1nCdrction in sicklecell anemia: magnetic resonance imaging correlates. Ann Ncurol
1988;23:125-130
2. Prensky AL, Swisher SCN, De Vivo DC. Positive brain scans in
children with idiopathic focal epileptic seizures. Neurology 10 7 3,
23(8):798-807
3. Kramer RE, Luders H, Lesser RL, et at. Transienr Jotal ahnormdities of neuroimaging studies during focal status epilepticus.
Epilepsia 1987;28(5):528-532
107-109
4. Sammaritano M, Andermann F, Melanson D, et al. Prolonged
focal cerebral edema associated with partial status epilepticus.
Epilepsia 1985;26(4):334-338
5. Goulatia RK, Verma A, Mishna WK, Ahuja GK. Disappearing
CT lesions in epilepsy. Epilepsia 1987;28(5):523-527
Reply
Steven G. Pavlakis, MD, Jacqueline Bello, MD,
Isak Prohovnik, PhD, Millicent Sutton, MD,
Carol Ince, RN, Jay P. Mohr, MD, Sergio Piomelli, MD,
Sadek Hilal, MD, PhD, and Darryl C. De Vivo, M D
We thank D r Kramer for his letter concerning our paper [l].
In regard to his question, 3 of our patients who were defined
as being in the stroke group had presumed convulsive onsets. The first had persistent right hemiparesis 3 years after
her presentation which, as noted in the paper, was not well
documented. H e r seizure probably occurred sometime after
the onset of hemiparesis. The second, who also had a right
hemiparesis, had two magnetic resonance imaging (MRI)
586 AnnaIs of Neurology
Vol 2 4
No 4
October 1988
Sneddon Syndrome:
Another Mendelian
Etiology of Stroke
Jose Berciano, M D
I read with interest the paper by Natowicz and Kelley { 1 1
concerning the genetic disorders associated with an increased
risk for stroke. Sneddon’s syndrome should be addect t o the
list of mendelian etiologies of stroke E2, 31. It is an entity
characterized by idiopathic livedo reticularis and cerebrovascular accidents. The basic abnormality appears to be a progressive noninflammatory occlusive arteriopathy of mediurnsize vessels. This syndrome accounted for O.26${ of the total
cases of cerebrovascular disease in our hospital 121. Fortynine cases had been published up to 1987 [ 4 ] .I n 4 cases the
disorder was familial, with a definite autosomal dominant