You are here

Article Text

Article menu

Download PDFPDF

Developmental venous anomaly: a rare cause of cerebellar ataxia
Free
  1. Ashley Griswold
  1. Nova Southeastern University, Davie, Florida, USA
  1. Correspondence to Ashley Griswold, ag1749{at}nova.edu

https://doi.org/10.1136/bcr-2016-215004

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    Description

    The most common causes of acute cerebellar ataxia are vascular disorders resulting in cerebellar ischaemia or haemorrhage. Most cerebellar haemorrhages are midline and therefore present with gait ataxia, truncal ataxia, dysmetria of lower extremities, saccadic intrusions and vertigo. Symptoms for cerebellar hemisphere lesions include dysdiadochokinesis, dysmetria of upper extremities, limb ataxia, intention tremor and scanning speech. Chronic progressive ataxias are most commonly degenerative and frequently hereditary in origin; they account for half the cases of adult onset ataxia.1

    Developmental venous anomalies are commonly encountered congenital anatomical variations of the cerebral vasculature—a malformation of medullary veins separated by normal brain parenchyma.2 They are considered benign lesions that may uncommonly present symptomatically.3

    We present a case of a 13-year history of acute onset, progressively worsening cerebellar ataxia with a developmental venous anomaly presenting in a 62-year-old man. He was admitted for cerebellar ataxia. He reported a medical history of acute onset of cerebellar ataxia in 2003 as well as a 45-pack year tobacco use disorder. In the past month, the patient reported repeated falls as well as a low velocity, frontal impact motor vehicle collision requiring extrication, 1 week prior to presenting to the emergency room. He reported worsening dysphagia and progressive weight loss. He denied any recent illness or malaise.

    On physical examination, he appeared cachectic (body mass index 16.4 kg/m2) alert, oriented and in no acute distress (video 1). He presented with an intention tremor, dysdiadochokinesis (video 2), dysmetria of the upper extremity (video 3), dysphagia, broad-based gait and ataxic dysarthria (video 1).

    Video 1

    Ataxic dysarthria with normal mentation.

    Video 2

    Dysdiadochokinesis.

    Video 3

    Dysmetria of upper extremities.

    During his admission, CT angiography of the brain (figures 1⇓–3) and MRI of the brain (figures 4⇓–6) were performed, revealing a developmental venous anomaly in the cerebellar vermis, cerebral and cerebellar atrophy and chronic deep white matter ischaemic changes. The combined cerebellar atrophy and developmental venous anomaly is believed to have resulted in the cerebellar ataxia. Neoplastic and paraneoplastic disease was not excluded.

    Figure 1
    Figure 1

    Cerebellar developmental venous anomaly revealed on CT angiography of the brain, axial view.

    Figure 2
    Figure 2

    Cerebellar developmental venous anomaly revealed on CT angiography of the brain, sagittal view.

    Figure 3
    Figure 3

    Cerebellar developmental venous anomaly revealed on CT angiography of the brain, coronal view.

    Figure 4
    Figure 4

    Cerebellar developmental venous anomaly and cerebellar atrophy revealed on MRI, T2* gradient recalled echo weighted.

    Figure 5
    Figure 5

    Cerebellar developmental venous anomaly, cerebellar atrophy and cerebral atrophy revealed on MRI, T1 weighted, sagittal and axial views.

    Figure 6
    Figure 6

    Cerebellar developmental venous anomaly and cerebellar atrophy revealed on MRI, fluid-attenuated inversion recovery weighted.

    Learning points

    • Adult-onset progressive cerebellar ataxia points to a broad set of differential diagnoses.

    • Not all developmental venous anomalies will remain asymptomatic.

    • Physical, especially neurological examination, is paramount in identifying and treating cerebellar ataxia.

    References

    1. ↵
      1. Wardle M,
      2. Robertson N
      . Progressive late-onset cerebellar ataxia. Adv Clin Neurosci 2007;7:2.
      OpenUrl
    2. ↵
      1. Ruíz DS,
      2. Yilmaz H,
      3. Gailloud P
      . Cerebral developmental venous anomalies: current concepts. Ann Neurol 2009;66:271–83. doi:10.1002/ana.21754
      OpenUrlCrossRefPubMedWeb of Science
    3. ↵
      1. Pereira VM,
      2. Geibprasert S,
      3. Krings T, et al
      . Pathomechanisms of symptomatic developmental venous anomalies. Stroke 2008;39:3205–15. doi:10.1161/STROKEAHA.108.521799
      OpenUrl
    View Abstract

    Footnotes

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.