Jean-Pierre Farmer

Neurocysticercosis: surgical and medical management with praziquantel.

Eight cases of neurocysticercosis were encountered at the two major neurological centers affiliated with McGill University in Montreal in 4 years. All patients were immigrants who had been in Canada for a period of 1 to 18 years and came from South America (three cases), Haiti (three cases), and Greece (two cases). Five patients had parenchymal cysts and presented with epilepsy; three had the racemose form and presented with hydrocephalus. Four patients with parenchymal cysts were treated by removal of the cysticercus and did well. One patient with numerous cysts was treated with praziquantel with good clinical and radiological results. Two patients with the racemose form were treated by cyst aspiration, cerebrospinal fluid (CSF) diversion, and praziquantel and did well. Another patient with the racemose form, seen before the advent of praziquantel, died of cysticercosis infection. Neurocysticercosis is not uncommon in the immigrant population of large North American cities. Single symptomatic cysts can be evacuated surgically with good results; multiple parenchymatous cysts can be treated with praziquantel. Patients with the racemose form who are treated with CSF diversion and praziquantel do well, although those who are only treated surgically die of their disease.

BLADDER FUNCTION BEFORE AND AFTER SELECTIVE DORSAL RHIZOTOMY IN CHILDREN WITH CEREBRAL PALSY

PURPOSE Approximately a third of children with cerebral palsy are expected to present with dysfunctional voiding symptoms. Selective dorsal rhizotomy, which is indicated for managing lower extremity spasticity in children with cerebral palsy, also has the potential of affecting bladder function. We evaluate the impact of selective dorsal rhizotomy on bladder function by comparing preoperative and postoperative symptoms, and urodynamic parameters in children undergoing selective dorsal rhizotomy for spasticity. MATERIALS AND METHODS We reviewed urodynamic studies in 25 boys and 15 girls with a mean age plus or minus standard deviation of 5.43+/-2.1 years who underwent selective dorsal rhizotomy for spasticity at our institution between January 1992 and September 1995. Urodynamics were performed preoperatively only in 22 patients, preoperative and postoperatively in 13, and postoperatively only in 5. Preoperative urodynamic studies were done within 2 weeks of surgery and postoperative studies were done at least 6 months after surgery (mean 1.32+/-0.65 years). RESULTS Of the 35 patients with preoperative urodynamic studies total bladder capacity, pressure specific volumes and full resting pressure were abnormal for age in 23 (65.7%). In addition, 17 of the 23 children (74%) were completely asymptomatic. In the group that underwent preoperative and postoperative urodynamic studies there was significant improvement in total bladder capacity (p <0.005) and pressure specific volumes (p <0.005) using the paired Student t test. All children had neurological improvement postoperatively, 5 of 7 (71%) who were incontinent preoperatively became continent and none had deterioration on urodynamics. CONCLUSIONS At least half of the children with spastic cerebral palsy have clinically silent bladder dysfunction. Selective dorsal rhizotomy improves spasticity and significantly improves bladder storage characteristics. We propose that urodynamic studies be included in the evaluation of children with spastic cerebral palsy who are possible candidates for selective dorsal rhizotomy to treat lower limb spasticity.

Lesionectomy of MRI detected lesions in children with epilepsy.

The results of complete excision of cerebral lesions detected by MRI in 18 children presenting with epilepsy were analyzed. There were 14 boys and 4 girls with a mean age of 9.2 years. The average age of onset of seizures was 6.8 years. The mean time from onset of seizures to surgery was 2.3 years. Often, CT scans suggested that the lesions were indolent. MRI was better in differentiating neoplastic from developmental lesions. Angiography was non-contributory in this series. Interictal EEGs showed epileptiform activity correlating with imaging studies in 54% of children. The lesion was completely surgically excised in all patients. This was confirmed by intra-operative ultrasound and postoperative imaging. Electrocorticography was performed prior to and after the resection, but residual spiking did not lead to further resection. The average postoperative follow-up was 5.7 years. Five patients had low grade astrocytomas, 4 had gangliogliomas, 1 a mixed astrocytoma-oligodendroglioma, 3 had cortical dysplasia, 2 infantile desmoplastic gangliogliomas, 2 hamartomata, and 1 cavernous angioma. Sixteen patients have been seizure-free since surgery. Only 2 have partial seizures. Thus, all patients benefited from the resection, with respect to seizure control. In those with temporal lobe lesions, improvement in IQ was seen postoperatively. Early consideration of surgery in patients with epilepsy and lesions demonstrated by MRI is suggested.

Encephaloduroarteriosynangiosis (EDAS) for the treatment of childhood moyamoya disease

Moyamoya disease is defined by the angiographic demonstration of stenosis or occlusion of the vessels of the anterior circulation at the base of the brain and the concomitant development of collateral blood supply. Untreated, the disease is often clinically progressive, resulting in significant neurologic sequelae. Encephaloduroarteriosynangiosis (EDAS), which involves the transposition of a segment of a scalp artery onto the surface of the brain, is a surgical treatment aimed at improving collateral blood flow. Six children underwent 8 EDAS procedures and were followed from 6 months to 9 years after surgery. No patient experienced further deterioration in neurologic status. Postoperative angiography demonstrated cerebral revascularization from the donor scalp artery on 3 of the 6 EDASs that were studied. The 2 patients who did not revascularize after EDAS demonstrated angiographic regression of their disease. The data suggest that EDAS is a safe procedure for the treatment of childhood moyamoya disease. Given the potential severity of the sequelae, early operative intervention is recommended in all children with this disease.

Posterior Quadrantic Epilepsy Surgery: Technical Variants, Surgical Anatomy, and Case Series

Summary:  Objective: Patients with intractable epilepsy due to extensive lesions involving the posterior quadrant (temporal, parietal, and occipital lobes) form a small subset of epilepsy surgery. This study was done with a view to analyze our experience with this group of patients and to define the changes in the surgical technique over the last 15 years. We also describe the microsurgical technique of the different surgical variants used, along with their functional neuroanatomy.

Magnetic resonance imaging in children with voiding dysfunction : Is it indicated?

PURPOSE We evaluated the role of magnetic resonance imaging (MRI) of the lumbosacral spinal cord in children with complicated voiding dysfunction and normal neuro-orthopedic examination. MATERIALS AND METHODS We reviewed the records of 32 consecutive children with complicated enuresis who were referred for neurosurgical evaluation, including those with a history of refractory voiding dysfunction or incontinence associated with persistent vesicoureteral reflux, encopresis, or associated leg or back pain. Nine patients were excluded from study because of urethral or anorectal anomalies, or failure to meet the inclusion criteria. Eligible for study inclusion were 23 children with a mean age of 8.9 years. Complete neurological and orthopedic examinations were normal in all patients except 1 with mild scoliosis and 1 with congenital facial palsy. RESULTS Urodynamic studies revealed instability in 14 cases, hypertonia in 7, hyporeflexia in 2 and detrusor-sphincter dyssynergia in 4. Skeletal abnormalities, mostly spina bifida occulta, were detected in 16 of the 23 children (70%). Spinal MRI was normal in 21 patients (91.3%), including 1 with a tethered cord and lipoma associated with a complex skeletal abnormality, and 1 with a nonprogressive, nonsurgical T7 to T9 syrinx. Only the case of lipoma required neurosurgical intervention. CONCLUSIONS The value of MRI is limited in children with voiding dysfunction and a normal neuro-orthopedic assessment. This study should be reserved for patients with associated neuroorthopedic findings or complex skeletal deformity on plain x-ray.

Motor Development of Infants with Positional Plagiocephaly

Concurrent with recommendations to place infants to sleep in supine, there has been a dramatic increase in the number of infants with positional plagiocephaly (PP). Recent evidence suggests that infants who have decreased exposure to prone position may have a higher incidence of PP and may be at risk for a delay in the acquisition of certain motor skills. The purpose of this study was to compare motor development between infants with PP and matched peers without PP. We also examined differences in infant positioning practices when asleep and awake between the two groups. Twenty-seven infants with PP, 3 to 8 months of age, were matched by age, gender, and race to infants without PP. Motor performance was evaluated using the Alberta Infant Motor Scale (AIMS) and the Peabody Developmental Motor Scales (PDMS). Parents completed a diary that recorded infant positioning over a 3-day period. Mean AIMS percentile score for infants with PP was 31.1 ± 21.6 as compared with 42.7 ± 20.2 in infants without PP (p =. 06). Better performance on the AIMS was positively correlated with the amount of time in prone position when awake, for both groups of children (PP r =. 52, no PP r =. 44, p <. 05). Therapists should be aware of a risk of a motor delay when evaluating infants with PP. It is also important for parents to be informed about the importance of supervised prone playtime to enhance the development of early motor skills.

Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease

We report a black girl with sickle cell anemia. On prophylactic exchange transfusion protocol, she experienced cerebrovascular accidents at 3 and 3.5 years of age, both associated with transient right hemiparesis. At 7.5 years of age, she presented with a partial motor seizure and a left hemiparesis. A cerebral angiogram demonstrated stenosis at the origins of both middle and anterior cerebral arteries bilaterally with extensive basal collateralization. She underwent uncomplicated bilateral encephaloduroarteriosynangiosis (EDAS) procedures using both superficial temporal arteries. At age 9 years, the patient presented with a severe headache and tunnel vision secondary to a stenosis of both posterior cerebral arteries. She underwent bilateral EDAS procedures using both occipital arteries. No complication was encountered. Postoperative cerebral angiogram demonstrated impressive neovascularity at the sites of all four EDAS procedures. Different treatment options of moyamoya disease are discussed.

Use of the Prone Position in the MRI Evaluation of Spinal Cord Retethering

In order to determine the impact of magnetic resonance imaging (MRI) in the management of spinal cord retethering, we retrospectively reviewed case and imaging records of 51 patients who underwent MRI examination in supine and prone positions. Group 1 included 8 control patients without cord tethering. They exhibited a normal level of the conus medullaris with normal surrounding subarachnoid space, and consistent anterior migration of the conus within the dural sac on MRI in prone position. Group 2 included 17 patients with tethered cord secondary to occult spinal dysraphism (spinal cord lipoma in 6 patients, thick filum terminale in 4, diastematomyelia in 4, myelomeningocele manqué in 2, and dermoid tumour in 1). Supine and prone MRI performed at a median period of time of 6 months after untethering showed resolution of posterior tethering in 5 out of the 7 patients who exhibited pre-operatively dorsal attachment of the spinal cord to the dura. Anterior migration of the conus or of the cord/filum complex in prone position was observed in only 24% of the cases. Group 3 included 26 patients with secondary tethered cord following prior myelomeningocele closure. Their MRI performed at a median interval of time of 11 months following untethering demonstrated resolution of the posterior cord tethering in only 8 out of the 24 patients who exhibited this feature pre-operatively. Anterior migration within the expanded dural sac was never noted in this group. We conclude that spine MRI is of limited value and that prone-positioned MRI is of no additional use in the evaluation of spinal cord retethering.

Continuous Unilateral Epileptiform Discharge and Language Delay: Effect of Functional Hemispherectomy on Language Acquisition

Summary: Purpose: To assess the efficacy of functional hemispherectomy in promoting language acquisition in a child with severe language delay and continuous left hemispheric epileptiform discharge in the absence of clinical seizures. We report a 6‐month‐old girl who initially presented with a right hemiparesis secondary to a lesion of probable prenatal origin in the distribution of the left middle cerebral artery. Weeks later, she developed infantile spasms. At 1.5 years of age, because of intractable seizures, the patient had fenestration of the left porencephalic cyst and anterior temporal lobectomy. The seizures ceased; however, language development remained limited to 35 words at 3.5 years of age. The EEG showed almost continuous epileptiform activity over the left hemisphere with no independent epileptiform potentials contralaterally. She underwent a functional left hemispherectomy.