Teaching Neuro Image : Tremor in aqueductal stenosis and response to endoscopic third ventriculostomy

Abstract

Idiopathic aqueductal stenosis (AS) may account for up to 59% of cases presenting with triventricular noncommunicating hydrocephalus. 1 The clinical presentations associated with triventricular hydrocephalus differ depending on age at onset and the acuity of obstruction. Acute syndromes include Parinaud's syndrome (vertical gaze restriction, lid retraction, and pupillary abnormalities), the rostral midbrain syndrome (upward gaze palsy, retraction nystagmus, pyramidal and extrapyramidal signs), and deficits in arousal. In the very young, chronic diencephalic compression can produce the bobble-head doll syndrome (high frequency head movements, limb ataxia, tremor, and cognitive deficits). Resolution of transtentorial pressure gradients by CSF diversion typically produces rapid improvement. 2 We describe an adult patient with upper extremity tremor due to decompensated hydrocephalus from AS, who demonstrated improvement following endoscopic third ventriculostomy.CASE REPORT An 18-year-old right-handed African American man presented with chronic, progressive tremor of the right hand. He was the product of an uncomplicated pregnancy and satisfied normative criteria for both growth and developmental milestones including head circumference. There was no history of head injury or encephalitis. At age 9 he developed action tremor in his dominant hand, which began in the distal upper extremity and progressed over 6 months to include the proximal arm and right leg. These symptoms were aggravated by stress, and interfered with activities of daily living. While he was considered less agile than his peers, he had no functional impairment or problems with falls. Rest tremor was not appreciated. By age 13, he was diagnosed with essential tremor, and trials of primidone and propranolol were unsuccessful.At presentation the tremor was unchanged, but his academic performance had declined and he had frequent headaches. He denied nausea, visual disturbances, weakness, or problems with bladder control. On examination, head circumference measured 62 cm and mild frontal bossing and hypomimia were present. Recall at 5 minutes was impaired (one of three objects), he could not complete serial sevens, and he was unable to provide the date. Language function was intact. Funduscopy was benign, pupils were 3 mm and reactive, and versions were preserved without nystagmus. Strength was full throughout with a spastic catch elicited on elbow extension bilaterally. Neither postural nor rest tremor was elicited, but finger-to-nose testing elicited a bilateral, 4 Hz action tremor, which was worse on the right (see video 1 on the Neurology Web site at www.neurology.org). A postural tremor was present in the right leg with standing. Stride length, arm swing, tandem gait, and turns were preserved.MRI of the brain demonstrated expanded lateral ventricles, midbrain compression, low lying cerebellar tonsils, and transependymal interstitial edema (figure 1). The cortical mantle was thinned and the fourth ventricle was preserved. While CSF flow studies demo...

Aqueductal Stenosis Presenting as Isolated Tremor: Case Report and Review of the Literature

“…A similar case was reported by Halterman et al [10 ]in which an 18-year-old male presented with an action tremor of the right hand, which progressed over a period of a few months to include the right arm and leg. Although this patient also presented with many other symptoms, such as headache and cognitive decline, he is similar to this patient in that they both possessed action tremor from hydrocephalus due to aqueductal stenosis, and both patients had rapid improvement after ETV.…”

“…A variety of mechanisms has been suggested involving injury to the red nuclei, substantia nigra and cerebellothalamic tracts amongst other areas [7,8,9,10,11,12]. Reports linking tremor to hydrocephalus are infrequent.…”

Headache and Tremor: Co-occurrences and Possible Associations

“…Tremor resembling ET has been noted in obstructive hydrocephalus. Halterman et al35 describe an 18-year-old African American male who developed action tremor in his dominant right hand and his right leg at the age of 9 years. At presentation, his academic performance had declined and he experienced frequent headaches.…”

Headache and Tremor: Co-occurrences and Possible Associations

“…Halterman et al 35 describe an 18-year-old African American male who developed action tremor in his dominant right hand and his right leg at the age of 9 years. At presentation, his academic performance had declined and he experienced frequent headaches.…”