of instrumental and vocal music. The hallucinations are disturbing to the patients only by their monotony and persistence [ 5 , 61, and there is no evidence of psychosis. As in other cases, our patient was able to “change the tunes” voluntarily [2, 51. In addition, she could “teach the quartet” new songs, which were incorporated into the repertoire and subsequently heard spontaneously and reproduced without conscious effort. This patient also reaffirms the statement of Ross et a1 [5] that “the origins of a hallucinatory process need not necessarily be in the same sensory system as the hallucination,” because she could change the content of the auditory hallucinations by visual stimulus (leafing through a hymnal). Our patient also experienced nonmusical auditory hallucinations, an unusual occurrence in patients experiencing this phenomenon. The similarities between the nonmusical and musical hallucinatory forms are striking: both are recollections of childhood experiences and are nonthreatening and nonpsychotic in character. Early writers supposed a relationship with atherosclerosis [4]. However, despite the advanced age of most patients, hearing loss rather than evidence of symptomatic cerebrovascular disease is the common denominator in such nonpsychotic auditory hallucinations. Similarities between this form of hallucinosis and that reported by rare patients experiencing slowly progressive blindness seems to emphasize the role of sensory deprivation in this syndrome [ l , 51. West [7] has advocated a paradigm, founded largely on Hughlings-Jackson’s concept of disinhibition, whereby sensory input suppresses myriads of nonessential information, including previously acquired memories. The loss of this input permits “perceptual traces . . . [to] be ‘released’ and reexperienced, either in familiar or n e w - e v e n bizarre-ombinations”; West cites the case of an individual with cataracts and otosclerosis who experienced both visual and auditory hallucinations, further implicating the role of sensory deprivation.
References 1. Bartlett JEA: A case of organized visual hallucinations in an old man with cataracts and their relation to the phenomenon of phantom limb. Brain 74:363-373, 195 1 2. Clovis W L They hear music (letter). Am J Psychiatry 133:1096, 1976 3. Coleman WS: Hallucinations in the sane associated with local organic disease of the sensory organs, etc. Br Med J 1:10151017, 1894 4. Rhein JHW: Hallucinations of hearing and disease of the ear. N Y Med J 97:1236-1238, 1913 5 . Ross ED, Jossman PB, Bell B, et al: Musical hallucinations in deafness. JAMA 231:620-622, 1975 6 . Rozanski J, Rosen H : Musical hallucinosis in otosclerosis. Confin Neurol 12:49-54, 1952
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7. West LJW: A clinical and theoretical overview of hallucinatory phenomena, in Siege1 RK, West LJ (eds): Hallucinations: Behavior, Experience, and Theory. New York, Wiley, 1975, pp 300-301
Choroid Plexus Patdoma and Infantile Spasms Clinton E. Branch, MD, and Paul R. Dyken, M D
A 7-month-old infant had the infantile spasm syndrome, consisting of flexor and extensor spasms, developmental delay, and hypsarrhythmia. Corticotropin produced improvement in the clinical symptoms and reverted the generalized electroencephalographic abnormalities to more focal ones. Removal of a choroid plexus papilloma of the left lateral ventricle was followed by clinical recovery. One year later the child was normal developmentally and neurologically and was seizure free on anticonvulsant therapy. Branch CE, Dyken PR: Choroid plexus papilloma and infantile spasms. Ann Neurol 5:302-304, 1979
Infantile spasms are a relatively common type of seizure during infancy which nevertheless have not been reported with treatable structural lesions of the brain [ l , 3, 4, 71. Since the prognosis is poor even with the use of corticosteroids and corticotropic agents, some authorities have tended to be unaggressive in evaluating these infants. We report a child with infantile spasms, developmental delay, and hypsarrhythmic electroencephalographic pattern who was apparently cured after removal of a choroid plexus papilloma. A 7-month-old boy had had recurrent infantile spasms for one month; they were described at first as pure flexor spasms and later as having extensor spasms intermixed. They were sudden and brief, seldom lasted over 3 seconds, and were associated with a simulraneous myoclonic-tonic jerk of most major muscle groups of the trunk and extremities. The seizures sometimes occurred singly but more often in clusters of spasms repeated for several minutes. Many clusters and single spasms were occurring daily by the time he was seen. The prenatal course had been uneventful. He was the first born of young parents and was delivered by cesarean
From the Departments of Neurology and Pediatrics (Pediatric Neurology), Medical College of Georgia, Augusta, GA. Accepted for publication Aug 11, 1978. Address reprint requests to Dr Dyken, Section of Pediatric Neurology, Medical College of Georgia, Augusta, G A 30902.
0364-5134/791030302-03$01.25 @ 1978 by Paul R. Dyken
F i g I . Prrtrratnrent EEG .rboii'inK l~.yp.rarrhythtnicpattern coii.ii.(I i ti R oJ' hi &am plitit de. dilo rgunized bai-kKroil nd actirr t y atrd .iiiperinlposedniiilti/ocal .rpike.r. Sposnis occii rred ut point of. teiAtriciati'J tiote ':jerk," I n tl9i.c portinn qf'trace. relatii,e
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1-iq 3. CT icari zi ith iontratt. ?20 rtiutrtu. Obrerie inarkedlt dilated lejt lateral reritrrclr ii ith mild h j t of he niidltne cotitentc t o the rrqht One can ree a mrmal ciinrordpleuiir o/ t h e lateral i etitrii Ie o n t h e right and a deformed atid enKorged chorord plruiii o n the left.
Fig 2. EEG ten da..rs laler. after/ire day.( oJ'ACTH tberapj. N0tii.r iniproiunient and lrteralizutiorz ojrloii: 1iwre.r and .rharp actit.ity t o the left pmterior henii-rpbere.
section because of cephalopelvic disproportion. The neonatal period was normal. Examination at birth was unremarkable except for a grade 216 soft decrescendo systolic heart murmur. Early development was considered normal until the onset of the spasms. During the month before o u r examination his developmental progress had ceased. No localizing or lateralizing signs were uncovered. T h e head size and other measurements were normal, as was the neurological examination except for mild diffuse hyperreflcxia. The child was judged to be at a developmental level of about 5 months. Routine laboratory tests were unremarkable. An initial EEG showed a hypsarrhythmic pattern (Fig 1). Corticotropins were started ( A C T H , 80 units per day), and some improvement in seizure frequency occurred over a week; an EEG (Fig 2 ) showed epileptogenic abnormalities lateralized to the left hemisphere. A C T scan showed dilation of the left lateral ventricles due to an apparent obstruction at the level of the left foramen of Monro (Fig
F I X 4 E E G nitre day\ later. erKht da, I after lejt i rutzrotoai) utid remot at o/ chornrdplimiiI pupillntna. Obrerre extenirie hi~h-unifiliti/de rundom und rytiii~rnmii\~ l o i icrctrr i t ) po\terrnrlp. greater ow the right, ti rth $harpand rpike uaie L i i t r i ItJ.
3 ) . Pneumoencephalography showed a mass arising near t h e left foramen of Monro. A choroid plexus papilloma was totally removed through a left frontoparietal craniotomy. The child was maintained postoperatively on anticonvulsants and corticotropins and T h e patient has been followed no seizurcs occurred (Fig 4). for over one year postoperatively. H e has heen readmitted on several occasions because o f reaccumulation of subdural fluid at the operative site. A subdural-peritoneal shunt was performed when he was 14 months old. Throughout follow-up, he has continued t o progress developmentally at a normal or near normal rate. A t LO months of age, neurological examination was normal except for InoJerate mac-
Case Report: Branch and Dyken: C h o r d Plexus Papilloma
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F I R 5 . EEG ten months later. three months after left sirbdrcral-peritoneal shunt. At this time the patient was seizure jree clinically biit u w on anticonr,ulsants. Obseme the highamplitude spikes localized to the lefi posterior leads associated with some slowing and spread to neighboring areas. Sharp actwity is also seen in the right hemisphere.
rocephaly. A n EEG performed ten months after surgery continued to show a left frontocerebral abnormality (Fig 5).
Discussion Infantile spasms carry a poor prognosis, for they are usually associated with developmental delay or signs of neurological deterioration. It has been suggested that the infantile spasm syndrome consists of three necessary ingredients, the spasms themselves, developmental delay or neurological deterioration, and a disorganized EEG with superimposed diffuse, multifocal epileptogenic phenomena. When this triad is closely adhered to for diagnosis, about 75% of the cases are associated with some metabolic or structural cause. Rarely have central nervous system neoplasms been associated with the infantile spasm syndrome, and when this has been reported, the tumor has been a dioma [l, 71. Neither in Jeavons and Bowers’ often quoted monograph on infantile spasms [3] nor in a more recent review [4] are primary tumors of the central nervous system listed as possible causes.
304 Annals of N e u r o l o g y Vol 5 No 3 March 1979
Choroid plexus papilloma may occur at any age. Although Cushing [2] reported the overall incidence at 0.6%’, it is higher in childhood. Matson [ 5 ] estimates the incidence in the pediatric age group to be 3%. Most choroid plexus papillomas are encountered before the patient is 3 years old, and 20% are diagnosed in infancy. This tumor probably produces symptoms earlier in life than any other intracranial neoplasm [81, but the symptoms and signs are usually those of hydrocephalus [6].In Matson’s series, only 4 of 23 children with choroid plexus papilloma had seizures, and none had infantile spasms. We believe our patient is the first with choroid plexus papilloma presenting as infantile spasms. The case is of further interest because the other two necessary symptoms and signs of the infantile spasm syndrome were also present. Hydrocephalus, the most common initial symptom of choroid plexus papilloma, was not recognized until C T scanning and pneumoencephalography were carried out. The EEG first showed findings of a diffuse encephalopathy; the lateralized process was not obvious until spasm frequency and severity were altered by corticotropic agents. This patient’s course illustrates that a thorough diagnostic evaluation should be undertaken of all cases of infantile spasm, since curative treatments sometimes may be possible.
References 1. Charlton MH: Infantile spasms, in Myodonic Seizures.
Amsterdam, Excerpta Medica, 1975 2. Cushing H: Intracranial Tumors: Notes upon a Series of 2000 Verified Cases with Surgical Mortality Percentages Pertaining thereto. Springlield, IL, Thomas, 1932 3. Jeavons PM, Bowers BD: Clinics in Developmental Medicine, No. 15: Infantile Spasms. London, Heinemann, 1964 4 . Lacy JR, Penry JK: Infantile Spasms. New York, Raven, 1976, p 169 5. Matson DD: Neurosurgery of Infancy and Childhood. Second edition. Springfield, IL, Thomas, 1969, pp 581-583 6. Milhorat TH: Pediatric Neurosurgery. Philadelphia, Davis, 1978, pp 247-251 7. Van der Berg BJ, Yerushalmy J: Studies o n convulsive disorders in young children. Pediatr Res 3:298-304, 1969 8. Zulch KJ: Brain Tumors: Their Biology and Pathology. New York, Springer, 1957