134 Anna Sans-Fitó, MD Pseudomigraine With Prolonged Aphasia in a Child Jaime Campistol-Plana, MD, PhD With Cranial Irradiation for Medulloblastoma Maria José Mas-Salguero, MD Pilar Póo-Argüelles, MD, PhD ABSTRACT Emilio Fernández-Álvarez, MD, PhD Servicio de Neurologia We report a 12-year-old child with episodes of migraine-like Unitat Integrada Hospital Clinic-Sant Joan de Déu headaches with visual and motor auras a year after the surgical resec- Universitat de Barcelona, Barcelona, Spain tion and radiation therapy for medulloblastoma. The patient pre- Received May 10, 2001. Received revised Oct 23, 2001. Accepted for publication Nov 5, 2001. Address correspondence to Dr Anna Sans-Fit6, Servicio de Neurologia, Passeig Sant Joan de Deu, 2-08950 Esplugues, Barcelona, Spain. Tel: 93 253 21 53; fax: 93 203 39 59; e-mail: annasans@hsjdbcn.org. References 1. Barth PG: Pontocerebellar hypoplasias: An overview of a group of inherited neurodegenerative disorders with fetal onset. Brain Dev 1993; 15:411-422. 2. Barth PG, Blennow G, Lenard HG, et al: The syndrome of autosomal recessive pontocerebellar hypoplasia, microcephaly, and extrapyramidal dyskinesia (pontocerebellar hypoplasia type 2): Compiled data from 10 pedigrees. Neurology 1995;45:311-317. 3. Barth PG, Vrensen GFJM, Uylings HBM, et al: Inherited syndrome of microcephaly, dyskinesia and pontocerebellar hypoplasia: A systemic atrophy with early onset. J Neurol Sci 1990;97:25-42. 4. Muntoni F, Goodwin F, Sewry C, et al: Clinic spectrum and diagnosis difficulties of infant pontocerebellar hypoplasia type 1. Neuropediatrics 5. Aicardi J: Diseases of the Nervous System in Childhood, 2nd ed. London, Mac Keith Press, 1998. 6. Uhl M, Pawlik H, Laubenberger J, et al: MR findings in pontocerebellar hypoplasia. Pediatr Radiol 1998;28:547-551. 7. Chaves-Vischer V, Pizzolato GP, Hanquinet S, et al: Early fatal pontocerebellar hypoplasia in premature twin sisters. Eur J Paediatr Neu- 1999;30:243-248. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. rol 2000;4:171-176. Coppola G, Muras I, Pascotto A: Pontocerebellar hypoplasia type 2 (PCH2): Report of two siblings. Brain Dev 2000;22:188-192. Barth PG: Pontocerebellar hypoplasia—How many types? Eur J Paediatr Neurol 2000;4:161-162. Simonati A, Dalla Bemardina B, Colombari R, Rizzuto N: Pontocerebellar hypoplasia with dystonia: Clinico-pathological findings in a sporadic case. Childs Nerv Syst 1997;13.642-647. Barbot C, Carneiro G, Melo J: Pontocerebellar hypoplasia with microcephaly and dyskinesia: Report of two cases. Dev Med Child Neurol 1997;39:554-557. Grellner W, Rohde K, Wilske J: Fatal outcome in a case of pontocerebellar hypoplasia type 2. Forensic Sci Int 2000;113:165-172. Horlsen SP, Clayton PT, Hardin BN, et al: Olivopontocerebellar atrophy of neonatal onset and disialotransferrin developmental deficiency syndrome. Arch Dis Child 1991;66:1027-1032. Stromme P, Maehlen J, Strim EH, Torvik A: Postmortem findings in two patients with the carbohydrate-deficient glycoprotein syndrome. Acta Paediatr Scand 1991;375(Suppl):55-62. Dobyns WB, Kirkpatrick JB, Hittner HM, et al: Syndromes with lissencephaly II: Walker-Warburg and cerebro-oculo-muscular syndromes and a new syndrome with type II lissencephaly. Am J Med Genet 1985;22:157-195. Bawle EV, Kupsky WJ, D’Amato CJ, et al: Familial infantile olivopontocerebellar atrophy. 1995;13:14-18. Pediair Neurol Lincke CR, van den Bogert C, Nijtmans LGJ, et al: Cerebellar hypoplasia in respiratory chain dysfunction. Neuropediatrics 1996;27:216-218. de Koning TJ, de Vries LS, Groenendaal F, et al: Pontocerebellar hypoplasia associated with respiratory-chain defects. Neuropediatrics 1998;30:93-95. 19. Balistreri WF: Reye’s syndrome and Reye-like disorders, in Behrman RE, Kliegman RM, Jenson HB (eds): Nelson. Textbook of Pediatrics, 16th ed. Philadelphia, WB Saunders, 2000,1331-1332. sented with an episode of headache, prolonged aphasia, right hemiparesis, status epilepticus, and salt wasting. There was no evidence of a structural lesion. The neurologic deficits resolved over a period of 6 weeks. Because of the progressive deterioration in neurologic deficits, the patient underwent an extensive battery of laboratory tests and multiple neuroimages, all of which were normal. The unusually prolonged neurologic deficit in this patient without demonstrable structural lesions and his eventual complete recovery were most likely caused by ischemia in the left hemisphere secondary to vasospasm. This presentation mimics migraine headache. Evidence suggesting that this represents a long-term complication of treatment of children with central nervous system neoplasia is presented. (J Child Neurol 2002;17:134-138). Cranial irradiation for brain tumors is associated with significant neurologic effects. 1 The radiation effect may be divided into three stages: an acute effect generally related to brain edema; a subacute effect occurring up to 3 months after radiation causing demyelination, which typically causes a somnolence syndrome; and a late effect, which appears months to years after radiation, resulting in vascular injury and tissue necrosis. Acute and subacute effects are generally self-limiting; however, the late effect of vascular injury may progressively get worse or remain stable and may produce focal neurologic deficits, seizures, neurocognitive and developmental disorders, and endocrinologic deficiencies. Headache may be a late sequel of radiation injury and may resemble migraine with or without an aura. We report here a child with surgical resection of medulloblastoma and radiotherapy presenting with migraine-like headaches with sensory and visual aura. He suffered a prolonged right hemiparesis with global aphasia with eventual complete resolution of the deficits in 6 weeks. During his course, he had an episode of status epilepticus and associated cerebral salt wasting. Case History A 12-year-old white boy was admitted to the Children’s Hospital of Buffalo with a history of left-sided headache, confusion, inability to speak, and inability to adequately comprehend spoken and written words since a few hours prior to admission. He had a near-drowning accident 21/2 years ago, when an incidental cranial computed tomography (CT) scan revealed a medulloblastoma. The medulloblastoma was completely resected. Recovery was complicated by pseudomonas ventriculitis and hydrocephalus, for which he received prolonged intravenous antibiotics and a ventriculoperitoneal shunt. He had complete recovery, with no motor or intellectual deficits. He subsequently received craniospinal radiation with a boost to the posterior fossae. A total radiation dose of 3600 cGy in 180-cGy fractions was given. An additional boost of 1800 cGy resulted in the posterior fossae receiving a total of 5400 cGy. He had no acute adverse effects from radiation therapy. Follow-up magnetic resonance imaging (MRI) scans of his head and spine had not shown Downloaded from jcn.sagepub.com at UNIV OF CONNECTICUT on May 10, 2015 135 Figure 1. Electroencephalogram shows marked slowing in the left hemisphere. any evidence of tumor recurrence. The latest MRI was a month prior to the current admission. Twelve months following completion of therapy, he developed paroxysmal left-sided headaches, thought to be migrainous. He did not have significant headaches prior to surgery. The headaches were always left sided, occasionally associated with blurry vision, right hemianopia, nausea/vomiting, and sometimes with brief periods of confusion and word finding difficulties. However, he had not reported a hemiparesis, and visual deficits had not lasted more than a few minutes. He did not previously have seizures. He would have headaches an average of two times per month. He was given cyproheptadine hydrochloride as a prophylaxis for his headache, with some benefit. Two days prior to the admission, he developed another headache and was given clorazepate dipotassium in addition to acetaminophen. Prior to arrival, he was his usual self, talking fluently and functioning like any other intelligent 7th-grade honor roll student. His family history was significant for maternal migraine of many years, with sensory aura characterized by fluctuating tingling numbness of one side of the body lasting 15 minutes to half an hour prior to the migraine. Headaches were variable, alternating from side to side, and not associated with motor, visual, or speech deficits. Two sisters, 11 years and 7 years of age, are well and did not have migraine. Physical examination revealed a well-nourished child with a temperature of 39.5°C. Abdominal, lung, and cardiovascular system examinations were normal. He had sparse scalp hair, the consequence of radiation. Neurologic examination showed paraphasia, word finding difficulties, and anomia with isolated words numbering two to three. He was unable to name, repeat, read, and write. He understood gestures slightly better. He had a dense right homonymous hemianopia with mild right facial droop. Fundus examination was normal. Right-sided hemiparesis involved the upper extremities more than the lower extremities. He had upgoing plantars on the right. Cerebellar signs were absent. His gait was wide based, and he had difficulty with tandem walking. Because of aphasia, a sensory examination could not be done accurately. Laboratory and Radiologic Studies on Admission Laboratory evaluation was initially directed at excluding infective, vascular, inflammatory, and neoplastic etiologies. Complete blood count showed that white blood cells were 7700/tJbL, hemoglobin was 14.7 g/dL, platelets Figure 2. Magnetic resonance image showing enhancement of the cortical ribbon in the left hemisphere during the prolonged focal seizure reported in the text. 109/L, S was 62%, B was 12, L was 19, and erythrocyte sedimentation rate was 15 mm/h. Electrolytes, blood urea nitrogen, serum cre- were 283 x atinine, and glucose were normal. Computed tomography of the head with contrast and shunt series showed no new abnormalities other than the previous findings. The cerebrospinal fluid from the shunt fluid showed 1 to 2 white blood cells per f1L, protein of 20 mg/dL, and sugar of 78 mg/dL. Gram stain was negative. There were no red blood cells. A lumbar puncture revealed a pressure of 8 cm H 20, no white and red blood cells, and protein of 22 mg/dL. The cytology was negative for neoplastic cells. Polymerase chain reaction for herpes, enteroviruses, and Epstein-Barr virus was negative. Antinuclear antibodies and lupus screen were negative. Antiphospholipid antibodies, protein C, and protein S were negative. An echocardiogram was normal. Electroencephalography (EEG) revealed marked slowing in the left hemisphere without epileptiform activity (Figure 1), suggesting structural disease. Clinical Course He was treated with ceftriaxone and acyclovir. The hemiparesis became more pronounced over the first week of admission. The temperature fluctuated up to 39.5°C. Magnetic resonance angiography and two MRIs obtained over a 7day period failed to show new findings. On the 7th hospital day, he developed right-sided focal seizure, which progressed to generalized status epilepticus lasting up to an hour and requiring intubation and ventilation for 2 days. He was treated with lorazepam, phosphenytoin, and phenobarbital. He had hyponatremia (serum sodium, 124 mmol/L) with salt wasting (urine sodium 200 mmol/L) and profuse diuresis of up to 15 L of urine per day following the seizure requiring hypertonic saline and fluid replacement. He had developed a rash following intravenous phenytoin. His rash did not resolve despite discontinuing phenytoin. Acyclovir and phenobarbital were also discontinued. Repeat lumbar puncture following the status epilepticus was negative. Magnetic resonance imaging showed enhancement of the cortical ribbon of the left hemisphere (Figure 2). The repeat magnetic resonance angiography showed questionable beading of the left distal middle cerebral artery, Downloaded from jcn.sagepub.com at UNIV OF CONNECTICUT on May 10, 2015 136 suggesting the possibility of a vasculitis. He was started on methylprednisolone 1 g/day for 5 days for presumed vasculitis. A cerebral angiogram done the following day was normal, without evidence of vasculitis or complicated migraine with a prolonged aura. How- they are called migrainous infarctions. Topically, these patients show evidence of focal brain infarctions or atrophy. In the adult population, migraine headaches are known to be associated with brain infarctions. In children, reports of migrainous infarctions are infrequent ; only a handful of patients are reported in the literature. For the diagnosis of migrainous infarction, the International Headache Society stipulates that the patients should have a past history of migraine with aura. The present attack should be typical of previous attacks, and the neurologic deficits should not reverse within 7 days. Neuroimaging may demonstrate ischemic infarctions in the relevant area. Other causes of infarction must be ruled out by relevant investigations. Our patient does not fully fulfill the International Headache Society criteria for migraine as his headaches were always one sided and the prolonged deficits were not asso- ever, his complicated past medical history and the worsening ciated with a structural lesion. This classification system also man- deficits initiated a broad range of investigations. The diagnoses con- dates that the headache not be associated with specific vascular sidered were encephalitis/meningitis with stroke, radiation-induced vasculitis, stenosis/dissection of the left internal carotid and mid- or nonvascular disorder known to cause headache. dle cerebral arteries, recurrence of medulloblastoma, other cen- syndrome genetically linked in a majority of patients to chromosome 19.1 It is characterized by recurring episodes of hemiparesis or hemiplegia during the aura phase of the migraine attack, and the deficit can typically last up to 1 hour in most of the patients but, occasionally, may last days to weeks. The family history in our patient was not suggestive of familial hemiplegic migraine. CADASIL is again a genetic syndrome linked to chromosome 19; which presents with migraines that frequently occur before the fourth decade followed by transient ischemic attack, lacunar strokes, and multi-infarct dementia.’ This is a disease of the small cerebral vessels without hypertension. Neuroimaging in these patients is usually abnormal with subcortical white-matter lesions. The normal MRI and absence of a family history in our patient is against this diagnosis. ischemia. Recovery began the 12th hospital day, and the salt wasting resolved after 1 week. No further seizures occurred, and the fever subsided over the next week. Aphasia resolved over the next few weeks. At 6 weeks post hos- pitalization, he was back to normal. He resumed 7th-grade classes, performing just below his previous level. At discharge, he was placed on cyproheptadine. However, further episodes of brief headache occurred the next 2 weeks. Cyproheptadine was discontinued, and he was started on verapamil. He has had no significant headache since. Discussion This case presented a diagnostic challenge. The initial impression was that of a tral nervous system malignancies, bland and infective emboli, other vasculitides, hypercoagulable states, mitochondrial disorders such as mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and genetic syndromes such as cerebral autosomal-dominant arteriopathy, subcortical infarcts, and leukoencephalopathy (CADASIL). The cerebrospinal fluid examination on three occasions did not show evidence of an inflammatory, infective, or neoplastic etiology. However, the patient was covered with ceftriaxone and acyclovir because of the presence of significant fever. Initial CT scan and MRI/magnetic resonance angiography did not show a change in ventricular size or focal lesions, excluding the possibility of shunt malfunction, stroke, recurrence of the tumor, and inflammatory and demyelinating disorders. The radiation-induced vasculitis was unlikely because of the normal MRI and normal cerebral angiogram. The patient was given corticosteroid pulse dosing for 5 days because of beading seen on the distal middle cerebral artery. This was later considered artifactual as the cerebral angiogram was normal. The enhancement of the left hemispheric cortical ribbon was most likely because of the prolonged focal seizure. This appeared following the seizure and had resolved on the subsequent MRI 2 days later. The marked slowing of the left hemisphere on the EEG is suggestive of a functional impairment of that hemisphere. The hemispheric slowing, however, can also be a feature of hemiplegic migraine.3 His EEG had normalized at the time of discharge 3 Familial hemiplegic migraine is a distinct autosomal-dominant MELAS is a maternally transmitted mitochondrial disease, which presents with poor growth, focal or generalized seizures, recurrent acute episodes that resemble strokes or prolonged transient ischemic attack, recurrent vomiting with lactic acidosis, and his- tologic confirmation by ragged red fibers on muscle biopsy.’ Some patients have migraine-like headaches initially Neuroimaging in these patients is usually abnormal, with subcortical lesions. Our patient did not have clinical or neuroimaging features of this condition. A syndrome of pseudomigraine with temporary neurologic deficits and lymphocytic pleocytosis has been described. 10 In a series of 50 patients reported by Gomez-Aranda et al, a consistent feature was the presence of cerebrospinal fluid pleocytosis, which was not weeks later. present in our patient. The neurologic deficits ranging in duration Hyponatremia caused by inappropriate antidiuretic secretion is a well-known complication of many systemic illnesses.4 However, hypovolemia with salt wasting in the setting of an acute intracra- between 5 minutes and 3 days were completely reversed in these nial disease is less common and not well understood. The renal salt wasting is thought to be mediated through atrial natriuretic factors In our patient, salt wasting was observed after the patient had the first status epilepticus, 7 days after admission. The salt wasting resolved with the improvement in the patient’s condition in about 1 week’s time. According to the International Headache Society guidelines, the migraine with prolonged aura can result in reversible deficits lasting up to 1 weeks However, if the deficits last longer than 1 week, patients. None of these patients had structural lesions on neuroimaging and none had preexisting intracranial tumors or radiation therapy. Migraine-like episodes in these patients are thought to be related to an aseptic inflammation of the meninges. Several authors have reported radiation-induced vasculopain thy children with strokes and focal neurologic deficits These patients had associated vascular or parenchymatous brain lesions demonstrated on neuroimaging. Shuper et al reported migraine-like episodes with reversible neurologic deficits following cranial irradiation and chemotherapy in 1995.1:3 Theirs was the first report of a migraine-like vasospas- Downloaded from jcn.sagepub.com at UNIV OF CONNECTICUT on May 10, 2015 137 Table 1. Clinical Data in Children With Pseudomigraine *Patient presented in this report: all other patients from the Shuper et al report.7 PNET = primitive neuroectodermal tumor; PF = posterior fossa; CSRT = craniospinal radiation therapy; Local = total local radiotherapy. tic condition produced by radiation and chemotherapy without rent episode in our patient, in whom the deficit lasted several demonstrable structural lesion. They reported four children who, weeks, all of the other episodes were similar to the ones reported by Shuper et al (Table 1). This report is but another of the increasing list of treatmentrelated complications associated with the care of children with central nervous system neoplasia. Recognition of this complication and chronicling of its natural history are important to further document treatment-related problems and to help alleviate the fears in patients and health care providers that paroxysmal events with a focal signature do not necessarily imply recurrent or metastatic disease. following cranial irradiation and chemotherapy, had an onset of paroxysmal events characterized by severe headache associated with nausea, vomiting, and transient neurologic impairment lasting from a few minutes to 1 hour. Structural deficits were not noted in these patients. These patients were worked up extensively for recurrence of tumor, radiation necrosis, and vascular disease; none were found. Our patient presented with many features suggesting migraine headache. His aura consisted of right visual hemianopia followed by left-sided headache and aphasia. The headache and neurologic symptoms had been paroxysmal over a period of several months. At the time of the last admission, however, neurologic symptoms lasted over 4 weeks and appeared to be associated with structural damage. Despite many MRIS, magnetic resonance angiograms, and cerebral angiography, structural disease was not identified. Electroencephalography suggested physiologic abnormalities in the left hemisphere. The neurologic signs, the onset of status epilepticus, and an MRI suggesting a major metabolic event affecting the blood-brain barrier corroborated this. All of these, however, resolved with time without residual deficits. The lack of structural findings on MRI tended to rule out radiation necrosis, frank infarcts, and other forms of radiation damage. As in the Shuper et al report,13 we concluded that this patient presented with transient migraine-like episodes without demonstrable structural deficits. This case further documents the role of S.N. Krishna Murthy, MD Michael E. Cohen, MD Department of Neurology SUNY/Buffalo Children’s Hospital of Buffalo Buffalo, New York Received July 12, 2001. Received revised August 28, 2001. Accepted for publication Sept 12, 2001. Address correspondence to Dr Michael E. Cohen, Department of Neurology, Children’s Hospital of Buffalo, 219 Bryant Street, Buffalo, NY 14222. Tel: 716-878-7848; fax: 716-878-7326; e-mail: mecohen@acsu.buffalo.edu. References - 1. 2. Duffner PK, Cohen ME: Long-term consequences of CNS treatment for childhood cancer: Part 1-Pathological consequences and potential for oncogenesis. 1991;7:299-301. Pediatr Neurol 3. Sand T: EEG in migraine: A review of the literature. Funct Neurol 4. 1991;6(1):7-22. Oruckaptan HH, Ozisik P, Akalan N: Prolonged salt wasting syndrome radiation and adds to the original report of Shuper et al of the poten- tial development of a vasospastic-like phenomenon occurring many months after radiation. The pathophysiology of its causation and the role of radiation are yet to be elucidated. Yet we are left with the inference that radiation, perhaps because of injury to the associate with the intraventricular dissemination of brain tumors: Report of two cases and review of literature. Pediatr Neurosurg vascular endothelium, lends itself to recurring vasospastic events. The total dose of radiation to the whole head was 3600 cGy. How- 5. ever, the boost to the posterior fossae includes the posterior two- thirds of the temporal lobes. Hence, the temporal lobe received more ICD-10 guide for headaches. International Headache Classification Committee. Cephalalgia 1997;17(Suppl 19):1-82. 7. Crawford JS, Konkol RJ: Familial hemiplegic migraine with crossed cerebellar diaschisis and unilateral meningeal enhancement. Headache 8. Tournier-Lasserve E, Iba-Zizen MT, Romero N, Bousser MG: Autosomal dominant syndrome with stroke like episodes and leucoencephalopathy. Stroke 1991;22:1297-1302. involves the dominant hemisphere remains conjectural. Curiously, all of the four patients reported by Shuper et al had posterior fossa tumors, received a radiation boost to the posterior fossae, and presented with dominant hemispheric symptoms. Except for the cur- 2000;33(1):16-20. Betges MG, Koopmans RP: Hyponatremia in acute intracranial disorders : Cerebral salt wasting. Ned Tijdschr Geneeskd 2000; 144:553-556. 6. radiation than that given to the craniospinal axis. This may account for the predominantly temporal lobe symptoms. Why this mainly Cohen ME, Duffner PK: Clinical and pathologic consequences of treatment of cancer of CNS. 1991;3:157-163. Pediatr Neurol 1997;37:590-593. Downloaded from jcn.sagepub.com at UNIV OF CONNECTICUT on May 10, 2015 138 9. Pavlakis SG, Phillips PC, Dimauro S, et al: Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes: A distinctive clinical syndrome. Ann Neurol 1984;481-488. 10. Gomez-Aranda F, Canadillas F, Marti-Masso JF, et al: Pseudo migraine with temporary neurological symptoms and lyphocytic pleocytosis— A report of 50 cases. Brain 1997;120(Pt7):1105-1113. 11. Painter MJ, Chutorian AM, Hilal SK: Cerebrovasculopathy following irradiation in childhood. Neurology 1975;25:189-194. 12. Raffel C: Early vasculopathy following radiation in a child with medulloblastoma. Pediatr Neurosurg 2000;32:255-258. 13. Shuper A, Packer RJ, Vezina LG, et al: Complicated migraine like episodes in children following cranial irradiation and chemotherapy. Neurology 1995;45:1837-1840. apy.9--13 Reports on the use of botulinum toxin in upper limb spasticity cerebral palsy have been scarce. 14-10 The objective of this study was to determine the efficacy and tolerability of botulinum toxin in children with cerebral palsy in reducing spasticity and improv- ing upper limb function. Subjects and Methods Children with cerebral palsy currently followed up in the Children’s Habilitation Institute of Duchess of Kent Children’s Hospital were recruited into the study. Static function of the upper limb must have been present for 3 months before being recruited. The inclusion criteria included moderate spasticity of the upper limb with a Modified Ashworth Spasticity Scale score of at least 2, dynamic deformity, no fixed joint deformity, and no previous surgery. Children with fixed joint contractures were excluded. The cohort consisted of 11 children (7 boys, 4 girls). The mean age was 6 years, 2 months (range 2 years to 15 years, 6 months). The type of cerebral palsy was hemiplegia (n 9; 4 left, 5 right), spastic diplegia (n 1), and spastic triplegia (n 1). All children were ambulatory. The children were evaluated prior to the observation period for 3 months, postinjection (weeks 1, 4, and 16), with the same parameters. Baseline evaluation prior to botulinum toxin injection consisted of objective measurement of upper limb function with standardized tests by the physiotherapist and occupational therapist. One or more of the following = Open-Label Study of Botulinum Toxin for Upper Limb Spasticity in Cerebral Palsy = = = ABSTRACT The objective of this study was to assess the efficacy of botulinum toxin for upper limb spasticity in cerebral palsy. An open-label study was conducted in 11 children with cerebral palsy. Post-botulinum toxin assessment was conducted in weeks 1, 4, and 16, with averaged scores being analyzed. The Clinical Global Impression Scale of the mothers showed marked, moderate, and mild improvement in five, four, and two cases, respectively. The Modified Ashworth Spasticity Scale score showed a statistically significant decrease in the mean spasticity score (P < .003). Other tests were performed depending on the cognition of the child. Increase in joint motion occurred in all five children assessed using web space (P .043). For the Jebson Hand Function Test, improvement occurred in all five cases assessed (P < .03). Four of five (80%) children could perform tasks that they failed before they were given botulinum toxin. Botulinum toxin is useful in decreasing spasticity and improving the upper limb function of young children with cerebral palsy with normal cognition. Motivated families should be selected with a specific target of using botulinum toxin as an adjunct in a habilitation program. (J Child Neurol 2002;17:138-142). = Various methods have been used in children with cerebral palsy to improve spasticity. This includes oral medication (benzodi- azepines, baclofen, dantrolene)l and surgical release of tendon contractures.2 Oral antispastic medication induced a central sedative action. More recent techniques used for treating spasticity of the lower limbs include continuous intrathecal baclofen infusion.&dquo; Botulinum toxin type A is a reversible neuromuscular block- ing agent that has been used extensively in treating patients with dystonia such as blepharospasm, hemifacial spasm, cervical dystonia, or strabismus.4,5 Its use in spasticity was first tried in multiple sclerosis in 1990.6 The use of botulinum toxin was also documented in spastic mouse models in increasing the muscle length as compared to controls. The use of botulinum toxin in children with cerebral palsy was first reported in 1994.8 Subsequently, a few studies on children with the spastic or athetoid type of cerebral palsy documented its value as a short-term adjunctive ther- parameters were analyzed for each child depending on the cooperativeness, cognitive level, and functional ability. The average value in the three assessments was analyzed: 1. Spasticity (evaluated for all cases: biceps, pronater teres/pronator quad- ratus, adductor pollucis brevis). The degree of spasticity was assessed using the Modified Ashworth Spasticity Scale.21 The score was as follows: 0 = normal (no increase or decrease in tone); (1) mild (slight increase = in muscle tone, &dquo;catch&dquo; in limb movement or minimal resistance of movement through less than half of the range); (2) = moderate (marked increase in muscle tone through most of the range of motion, but the passive movement of the affected part is easily performed); (3) severe = (prominent increase in muscle tone with difficulty in passive movement) ; and (4) extreme (affected part is rigid in flexion and extension). The mean Modified Ashworth Spasticity Scale score of the muscle assessed was compared pre-botulinum toxin and post-botulinum toxin injection. 2. Measurement of range of joint motion by goniometer (n 3) (cases 3, 7, and 8) and hand map (web space) (cases 1, 3, 5, 7, and 11). The degree of active joint movement was measured after the injection (elbow extension, hand supination, and thumb abduction). The maximum space between the thumb and first finger was measured with a simple imprinting technique on a hand map (ie, web space). 3. Muscle power of the hand including palmar strength (using the Jamar hand dynamometer) and pinch strength, measured in five children. (cases 3, 5, 7, 8, and 11). 4. Jebson Hand Function Test (n 5) (cases 2, 5, 6, 8, and 9). The mean time taken to achieve seven items of hand function (after three trials) was scored. The seven items included writing, card turning, picking up small objects, stacking up checkers, stimulated feeding, lifting up of light objects, and lifting up of heavy objects. The averaged time score achieved after injection was compared to the averaged time taken for each task before the injection. The time score taken for the normal hand was also assessed to take away the learning effect. 5. Clinical photograph of hand maps and video recordings of the actual assessment procedures, taken to document any changes. = = = Evaluation for the mother included the following: 1. Report of onset, peak effect of action, and any side effect. 2. Clinical Global Impression Scale-a Likert scale of 7 with 4 = no change, 1 very much improved (>75%), 2 = much improved (50-75%), 3 = min= imally improved (25-50%), 7 very much worse (>75%), (50-75%), and 5 minimally worse (25-50%). = = Downloaded from jcn.sagepub.com at UNIV OF CONNECTICUT on May 10, 2015 6 = much worse