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Chiari I malformation 23

econdary to Chiari I malformation, the therapeutic responseo phenobarbital strongly supports the diagnosis of epilepsy.o our knowledge, this is the first case report of seizures withroximal hypotonia as a predominant clinical manifestation.

eferences1. Prilipko O, Dehdashti AR, Zaim S, et al: Orthostatic intolerance and

syncope associated with Chiari type I malformation. J Neurol Neuro-surg Psychiatry 76:1034-1036, 2005

2. Hood BM, Harbord MG: Paediatric narcolepsy: Complexities of diag-nosis. J Paediatr Child Health 38:618-621, 2002

3. Sharp SJ, D’Cruz OF: Narcolepsy in a 12 month old-boy. J Child Neurol16:145-146, 2001

4. Nishino S: Clinical and neurobiological aspects of narcolepsy. SleepMed 8:373-399, 2007

5. Crow YJ, Zuberi SM, McWilliam R, et al: “Cataplexy” and muscle ultra-sound abnormalities in Coffin-Lowry syndrome. J Med Genet 35:94-98, 1998

6. Vossler DG, Wyler AR, Wilkus RJ, et al: Cataplexy and monoamineoxidase deficiency in Norrie disease. Neurology 46:1258-1261, 1996

7. Lehmann-Horn F, Rudel R: Channelopathies: The nondystrophic myo-tonias and periodic paralyses. Semin Pediatr Neurol 3:122-139, 1996

8. Ryan MM, Taylor P, Donald JA, et al: A novel syndrome of episodicmuscle weakness maps to xp22.3. Am J Hum Genet 65:1104-1113,1999

9. Alirol E, Martinou JC: Mitochondria and cancer: Is there a morpholog-ical connection? Oncogene 25:4706-4716, 2006

0. Dimauro S, Davidzon G: Mitochondrial DNA and disease. Ann Med37:222-232, 2005

1. DiMauro S, Andreu AL, De Vivo DC: Mitochondrial disorders. J ChildNeurol 17:3S35–3S45, 2002; discussion 3S36-3S37 (suppl 3)

2. Carrozzo R, Piemonte F, Tessa A, et al: Infantile mitochondrial disor-

ders. Biosci Rep 27:105-112, 2007

EDITORIAL CO

ified and are often of unclear clinical significance. The mag-

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3. Tubbs RS, Lyerly MJ, Loukas M, et al: The pediatric Chiari I malforma-tion: A review. Childs Nerv Syst 23:1239-1250, 2007

4. Nohria V, Oakes WJ: Chiari I malformation: A review of 43 patients.Pediatr Neurosurg 16:222-227, 1990

5. Listernick R, Tomita T: Persistent crying in infancy as a presentation ofChiari type I malformation. J Pediatr 118:567-569, 1991

6. Schaul N: The fundamental neural mechanisms of electroencephalog-raphy. Electroencephalogr Clin Neurophysiol 106:101-107, 1998

7. Schaul N, Gloor P, Gotman J: The EEG in deep midline lesions. Neu-rology 31:157-167, 1981

8. Pandey A, Robinson S, Cohen AR: Cerebellar fits in children withChiari I malformation. Neurosurg Focus 11:E4, 2001

9. Korff CM, Nordli DR Jr: The clinical-electrographic expression of in-fantile seizures. Epilepsy Res 70:S116-S131, 2006 (suppl 1)

0. Hamer HM, Wyllie E, Luders HO, et al: Symptomatology of epilepticseizures in the first three years of life. Epilepsia 40:837-844, 1999

1. Fogarasi A, Jokeit H, Faveret E, et al: The effect of age on seizuresemiology in childhood temporal lobe epilepsy. Epilepsia 43:638-643,2002

2. Fogarasi A, Boesebeck F, Tuxhorn I: A detailed analysis of symptomaticposterior cortex seizure semiology in children younger than sevenyears. Epilepsia 44:89-96, 2003

3. Iannetti P, Spalice A, De Felice Ciccoli C, et al: Seizures in paediatricChiari type I malformation: The role of single-photon emission com-puted tomography. Acta Paediatr 91:313-317, 2002

4. Elia M, Biondi R, Sofia V, et al: Seizures in Chiari I malformation: Aclinical and electroencephalographic study. J Child Neurol 14:446-450, 1999

5. Brill CB, Gutierrez J, Mishkin MM: Chiari I malformation: Associationwith seizures and developmental disabilities. J Child Neurol 12:101-106, 1997

6. Buoni S, Zannolli R, di Bartolo RM, et al: Surgery removes EEG abnor-malities in patients with Chiari type I malformation and poor CSF flow.

Clin Neurophysiol 117:959-963, 2006

MMENT

oincidence or Causation?ishman et al present an interesting case that again raisesthe age-old question of whether 2 relatively common

iagnoses are related or coincidental. Their patient was notedo have events that were concerning for clinical seizures alongith a Chiari I malformation with associated cervical cord T2

ignal change. Given the myriad of clinical symptoms re-orted with Chiari malformations, including drop attacks,he question arises whether the clinical events were related tohe Chiari or a coincidence. The stereotyped nature of thearoxysmal events suggested that the events were epileptic,hich was supported by video-encephalographic monitor-

ng. Her response to therapy with phenobarbital and recur-ence of events after the discontinuation of the medicationlso strongly support that the events are epileptic.

Other studies have shown the coexistence of epilepsy andhiari malformation with some noting improvement in thencephalogram after decompression. However, this casegain reinforces that the relatively high incidence of Chiari Ialformations may cause us to suspect a link when one mayot be real. With more children undergoing magnetic reso-ance neuroimaging, it is apparent that more cases are iden-

etic resonance imaging T2 signal change seen in the cervicalord in this patient certainly supports the choice of surgicalecompression. However, the return of her events after sur-ical correction support that the Chiari is likely a “red her-ing” and not related to her events. In hindsight, if neuroim-ging had not been performed and the Chiari had not beenound, the patient may have benefited from a therapeutic trialf antiepileptic drugs (AED) and avoided surgery at that time.ore research into a possible link between Chiari malforma-

ions and seizures seems justified based on the few observa-ional studies.

The authors note that this is the first time that hypotoniaas been identified as a semiologic finding associated witheizures in infants. The differential for hypotonic events innfants can be protean, with many nonepileptic events havingimilar clinical characteristics. Often when faced with thisiagnostic uncertainty, many tests are performed that mayail to clearly identify a cause and tend to vary depending onhich specialist you consult. The authors approached theatient appropriately by striving to document if the eventsere in fact epileptic before starting AED therapy. Although

tonic seizures are described in infants, the described patientad a more gradual and prolonged hypotonic phase with

lteration of awareness. This highlights the difficulty in clas-

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24 K. Chapman

ifying seizures in infants and has led to the term of hypomo-or seizures for those times when patients have clear motorrrest but lack automatisms and identifying alteration ofwareness can be challenging. Much of this likely relates tohe age-dependent semiology that infants and young childrenanifest that makes typical adult features less likely. The

ypotonia exhibited in this patient, although unusual in

dults, may be more common in children but underrecog-ized as a semiologic feature.

Kevin Chapman, MDBarrow Neurological Institute

St. Joseph’s Hospital and Medical Center

Phoenix, AZ