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Tremor in Aqueductal Stenosis and Response to Endoscopic Third Ventriculostomy Marc W

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Tremor in Aqueductal Stenosis and Response to Endoscopic Third Ventriculostomy Marc W RESIDENT AND FELLOW SECTION Teaching NeuroImage: Section Editor Tremor in aqueductal stenosis and Mitchell S.V. Elkind, MD, MS response to endoscopic third ventriculostomy Marc W. Halterman, Idiopathic aqueductal stenosis (AS) may account for 62 cm and mild frontal bossing and hypomimia MD, PhD up to 59% of cases presenting with triventricular were present. Recall at 5 minutes was impaired (one G. Edward Vates, MD, noncommunicating hydrocephalus.1 The clinical of three objects), he could not complete serial sev- PhD presentations associated with triventricular hydro- ens, and he was unable to provide the date. Lan- Garrett Riggs, MD, cephalus differ depending on age at onset and the guage function was intact. Funduscopy was benign, PhD acuity of obstruction. Acute syndromes include pupils were 3 mm and reactive, and versions were Parinaud’s syndrome (vertical gaze restriction, lid re- preserved without nystagmus. Strength was full traction, and pupillary abnormalities), the rostral mid- throughout with a spastic catch elicited on elbow Address correspondence and brain syndrome (upward gaze palsy, retraction extension bilaterally. Neither postural nor rest reprint requests to Dr. Marc W. nystagmus, pyramidal and extrapyramidal signs), and tremor was elicited, but finger-to-nose testing elic- Halterman, Department of deficits in arousal. In the very young, chronic dience- ited a bilateral, 4 Hz action tremor, which was Neurology, University of Rochester School of Medicine phalic compression can produce the bobble-head doll worse on the right (see video 1 on the Neurology and Dentistry, 601 Elmwood syndrome (high frequency head movements, limb Web site at www.neurology.org). A postural tremor Avenue, Box 673, Rochester, NY 14642 ataxia, tremor, and cognitive deficits). Resolution of was present in the right leg with standing. Stride Marc_Halterman@urmc. transtentorial pressure gradients by CSF diversion typ- length, arm swing, tandem gait, and turns were rochester.edu ically produces rapid improvement.2 preserved. We describe an adult patient with upper extrem- MRI of the brain demonstrated expanded lateral ity tremor due to decompensated hydrocephalus ventricles, midbrain compression, low lying cerebel- from AS, who demonstrated improvement follow- lar tonsils, and transependymal interstitial edema ing endoscopic third ventriculostomy. (figure 1). The cortical mantle was thinned and the fourth ventricle was preserved. While CSF flow CASE REPORT An 18-year-old right-handed Afri- studies demonstrated flow across the foramen mag- can American man presented with chronic, progres- num along both anterior and posterior aspects of sive tremor of the right hand. He was the product of the cranio-cervical junction (figure 2A), cross sec- an uncomplicated pregnancy and satisfied norma- tional phase contrast MRI documented absent flow tive criteria for both growth and developmental in the aqueductal canal, supported by the appear- milestones including head circumference. There was ance on sagittal high resolution gradient echo imag- no history of head injury or encephalitis. At age 9 he ing. There were no areas of abnormal gadolinium developed action tremor in his dominant hand, enhancement. These features were consistent with which began in the distal upper extremity and pro- the diagnosis of AS.3 gressed over 6 months to include the proximal arm Endoscopic third ventriculostomy was per- and right leg. These symptoms were aggravated by formed to arrest progression of the hydrocephalus.4 stress, and interfered with activities of daily living. Ventriculographic guidance provided visualization While he was considered less agile than his peers, he of the distended architecture of the lateral ventricle had no functional impairment or problems with and vascular structures (figure 1D). Six months falls. Rest tremor was not appreciated. By age 13, he postoperatively, the patient could hold a drinking was diagnosed with essential tremor, and trials of glass without spilling, and his headaches had re- primidone and propranolol were unsuccessful. solved. Follow-up MRI studies demonstrated an At presentation the tremor was unchanged, but open ventriculostomy site (figure 2B), and decom- his academic performance had declined and he had pression of the lateral ventricles with expansion of frequent headaches. He denied nausea, visual dis- the cortical mantle (figure 2, C and D). On examina- turbances, weakness, or problems with bladder con- tion, the patient’s free-recall and dysmetria (video 2) trol. On examination, head circumference measured had improved. From the Departments of Neurology (M.W.H., G.R.) and Neurosurgery (G.E.V.), University of Rochester School of Medicine and Dentistry, NY. Disclosure: The authors report no conflicts ofinterest. Copyright © 2007 by AAN Enterprises, Inc. E29 Preoperative axial FLAIR (A, patients (age 1 month to 18 years) with obstructive Figure 1 Axial FLAIR and T2-weighted MR images B) and sagittal (panel C) T2- hydrocephalus found headache, vomiting, psy- weighted MR images. Note the prominent chomotor retardation, and gait disturbances to be ventriculomegaly involving the most common presenting symptoms.1 Patients the lateral and third under the age of 30 with chronic AS (present for ventricles with transependymal edema and greater than 6 months) most often complain of preserved architecture of headache, while patients over age 63 exhibit demen- both the fourth ventricle and tia, gait disturbance, and urinary incontinence.6 Of cerebellum. (D) Endoscopic note, tremor was present in only 12.5% (3/25) of appearance of dilated right ventricle with distended cases and was seen later in the disease course. Also, vascular and connective patients initially diagnosed and treated for triven- tissue elements. tricular hydrocephalus can present with parkinson- ism without tremor, which responds to dopaminergic therapy and shunt revision.7 Surgical options for AS include shunting, cho- roid plexectomy, third ventriculostomy, and aque- ductoplasty. Intervention may be warranted if a pattern of gradual clinical decline and transependy- mal edema on imaging are present. Endoscopic DISCUSSION Tremor is an uncommon presenting third ventriculostomy (ETV) creates a communica- symptom of occult hydrocephalus. Essential tremor tion between the third ventricle and the subarach- (ET) is the most common cause of tremor in child- noid space, and is an option in patients older than 6 hood and presents most commonly in the second months of age.8 In a retrospective analysis with 42 and sixth decades.5 While ET has a predilection for months of follow-up, investigators demonstrated hand and arm involvement, the lack of family his- that ETV provided durable patency in 90% of pa- tory (elicited in greater than 75% of cases) or re- tients with AS, excluding cases due to cysts, tumors, sponse to medication trials, along with the or post-hemorrhagic ventriculitis.9 Third ventricu- associated symptoms of headache and cognitive de- lostomy can be performed with success rates be- cline, were features prompting consideration of an tween 80 and 100%.10 In cases of late onset AS (n ϭ alternate diagnosis. 31) treated with ETV, improvement occurred in For all causes of obstructive hydrocephalus, age 84% of patients after 26 months.6 at the time of diagnosis also exhibits a bimodal dis- While stereotactic approaches have significantly tribution with an early peak between months 1 and reduced the procedure-related mortality associated 4 (23% of cases) with a broader distribution span- with open third ventriculostomy (5% vs 27%), cur- ning ages 8 to 17. A retrospective analysis of 213 rent techniques are associated with rare but fatal vascular injuries.4,11,12 While decompression of the Figure 2 Cardiac-gated cine-MRI and axial FLAIR- third ventricle, upward deviation of the brainstem, weighted images and reduction in the size of the lateral ventricles are predictable changes postoperatively,13 clinical im- provement can precede demonstrable radiographic Pre- and postoperative improvement. Improved posture, gait, and cogni- cardiac-gated cine-MRI tion were achieved in greater than 50% of patients images (A vs B, respectively) (32/58) within 2 weeks postoperatively when imag- demonstrate the absence of 14 CSF flow across the ing data were largely unchanged. aqueduct of Sylvius and Our experience suggests that late onset AS turbulent flow in the third should be considered in the differential diagnosis ventricle following for new onset tremor in adolescence. Furthermore, ventriculostomy. Axial FLAIR- weighted images obtained 5 this case supports a role for endoscopic third ven- months postoperatively triculostomy in the treatment of AS. demonstrate expansion of the cortical mantle and partial reversal of REFERENCES transependymal edema (C, D); the fluid collection adjacent 1. Cinalli G, Sainte-Rose C, Chumas P, et al. Failure of third to the right frontal ventriculostomy in the treatment of aqueductal stenosis in craniectomy site can be seen. children. J Neurosurg 1999;90:448–454. E30 Neurology 68 May 8, 2007 2. Russman BS, Tucker SH, Schut L. Slow tremor and mac- 9. Feng H, Huang G, Liao X, et al. Endoscopic third ventric- rocephaly: expanded version of the bobble-head doll syn- ulostomy in the management of obstructive hydrocepha- drome. J Pediatr 1975;87:63–66. lus: an outcome analysis. J Neurosurg 2004;100:626–633. 3. Lev S, Bhadelia RA, Estin D, Heilman CB, Wolpert SM. 10. Kelly PJ. Stereotactic third ventriculostomy in patients Functional analysis of
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