|Year : 2016 | Volume
| Issue : 1 | Page : 40-42
Dual static encephalopathies - cerebral palsy with arrested hydrocephalus: A challenging spasticity management
Pavan Kumar Pelluru, Aniruddh Kumar Purohit
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Web Publication||10-Aug-2016|
Aniruddh Kumar Purohit
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500 018, Telangana
Source of Support: None, Conflict of Interest: None
Cerebral palsy associated with arrested hydrocephalus is an uncommon occurrence posing management challenge in the treatment of spasticity. We present a 14 year old male who was having spastic diplegia and arrested hydrocephalus due to congenital aqueductal stenosis. He was having spasticity of MAS 1-3 in the lower limbs. We haven't found such a type of dual static encephalopathies in the literature. In such a challenging scenario Selective Posterior Rhizotomy (SPR) may lead to alteration in the CSF dynamics, so Selective Motor Fasciculotomies (SMF) were performed to relieve spasticity.
Keywords: Arrested hydrocephalus, selective motor fasciculotomies, spastic diplegia
|How to cite this article:|
Pelluru PK, Purohit AK. Dual static encephalopathies - cerebral palsy with arrested hydrocephalus: A challenging spasticity management. Indian J Cereb Palsy 2016;2:40-2
|How to cite this URL:|
Pelluru PK, Purohit AK. Dual static encephalopathies - cerebral palsy with arrested hydrocephalus: A challenging spasticity management. Indian J Cereb Palsy [serial online] 2016 [cited 2017 May 26];2:40-2. Available from: http://www.ijcpjournal.org/text.asp?2016/2/1/40/188160
| Introduction|| |
Hydrocephalus secondary to aqueductal stenosis may present at any age. Clinical course may vary according to the patient's age and the anatomical deformation of the ventricular system. Aqueductal stenosis may be diagnosed in utero and is responsible for about 20% of cases of congenital hydrocephalus.  The arrest in progression of hydrocephalus may be due to the resumption of the proper balance between the production and absorption of cerebrospinal fluid (CSF) due to the development of a minor CSF pathway and spontaneous termination occurring in 10-15% individuals. 
| Case report|| |
A 14 - year - old boy presented to our hospital with a complain of difficulty in walking. He, the 3 rd sibling of nonconsanguineous parentage was born at full term through normal vaginal delivery. He had delayed birth cry. The birth weight was normal. He had delayed developmental motor milestones and gradually increasing head circumference till the age of 1 year. He had normal mental milestones. Now he presented with tightness in both the lower limbs and equinus gait toes (more on the right side) with both the knees bent. There was no history of headache. On examination, there was frontal bossing; extraocualr movements were normal. He had diffuse spasiticity in both the lower limbs; spasticity grades the MAS (Modified Ashworth Scale) in the hip adductors and knee flexors were 1 and 2, respectively on both the sides; the right ankle plantar flexors had more spasticity compared to the left 3 and 2 respectively. There was bilateral ankle clonus. The selective voluntary control grade was 4, 4 and 2 at hips, knees and ankles respectively. He had scissoring with equinus gait [Figure 1]. There was no spinal deformity.
|Figure 1: Pre- and post-operative images of individual with congenital aqueductal stenosis following bilateral tibial and obturator selective motor fasciculotomy for bilateral ankle plantar flexor and hip adductor spasticity|
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Magnetic resonance imaging of the brain (plain) showed gross dilatation of the lateral and third ventricles with thinned out cerebral cortex and collapsed fourth ventricle suggestive of congenital aqueductal stenosis with obstructive hydrocephalus. The child was evaluated and followed up. Clinico-imageologically, he was found to have arrested hydrocephalus. The selective posterior rhizotomy (SPR) was considered for relief of lower limb spasiticty. But, it could have disturbed the stable CSF dynamics. Therefore, alternative limb spasticity relief surgery SMF (Selective Motor Fasiculotomy) was planned.
He underwent Bilateral tibial selective motor fasciculotomies (SMF) (50% of fascicles supplying bilaterally the lateral and medial gastrocnemei and soleus muscles were ablated) and TA Z-plasty on the right side was performed to relieve contracture; in the second stage, he underwent bilateral obturator SMF (bilaterally, 50% of anterior division fascicles supplying the adductor muscles were clipped and ablated) was performed to relieve scissoring [Figure 1].
| Discussion|| |
The combination of two different static encephalopathies such as arrested hydrocephalus due to aqueductal stenosis and spastic diplegia due to cerebral palsy (CP) pose a management challenge, especially in treating diffuse lower limb spasticity. We have not found in the literature showing combination of arrested hydrocephalus due to aqueductal stenosis and spastic diplegic CP undergoing SMF instead of SPR.
Obstructive hydrocephalus due to aqueductal stenosis may be of varied etiology congenital and acquired; in our case, it is a congenital type as suggested by the history of gradually progressive increase in head circumference followed by stabilization without any intervention [Figure 2]. Villani et al. studied 78 patients with aqueductal stenosis and were followed for up to 5-25 years with detailed neurodevelopmental assessment, and they found that 68% of the patients remained categorically normal with mild neurological deficits requiring no further treatment. 
|Figure 2: (a and b) Axial and coronal TW2 magnetic resonance imaging showing dilatation of supratentorial ventricular system due to aqueductal stenosis|
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Even though in the individuals there was diffuse spasticity and selective posterior rhizotomy was appropriate (which involves opening of the thecal sac which may lead to alteration in the already compensated CSF dynamics) to relieve spasticity in the lower limbs, an alternative procedure multilevel SMF, was performed.
| Conclusion|| |
In dual static encephalopathy where the individual has arrested hydrocephalus, SPR can be avoided and the benefit can be given by alternative spasticity relieving surgery the SMF.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Levitsky DB, Mack LA, Nyberg DA, Shurtleff DB, Shields LA, Nghiem HV, et al
. Fetal aqueductal stenosis diagnosed sonographically: How grave is the prognosis? AJR Am J Roentgenol 1995;164:725-30.
Nonaka Y, Miyajima M, Ogino I, Nakajima M, Arai H. Analysis of neuronal cell death in the cerebral cortex of H-Tx rats with compensated hydrocephalus. J Neurosurg Pediatr 2008;1:68-74.
Villani R, Tomei G, Gaini SM, Grimoldi N, Spagnoli D, Bello L. Long-term outcome in aqueductal stenosis. Childs Nerv Syst 1995;11:180-5.
[Figure 1], [Figure 2]