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Small spiral, big mass
  1. Nirosen Vijiaratnam1,
  2. Andrew John Lees2,
  3. Huw R Morris1,2,3
  1. 1 Department of Neurology, Royal Free Hospital, London, UK
  2. 2 Department of Molecular Neuroscience, Institute of Neurology, University College London, London, UK
  3. 3 Department of Clinical Neurosciences, Royal Free Campus Institute of Neurology, University College London, London, UK
  1. Correspondence to Dr Nirosen Vijiaratnam, Department of Neurology, Royal Free Hospital NHS Trust, London NW3 2QG, UK; nirosenv{at}gmail.com

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A right-handed 44-year-old woman presented with a 4-month history of sudden-onset right leg weakness and a 4-week history of small handwriting. On examination, facial expression, blink rate and spontaneous and associative movements were normal. Power in her right leg was mildly reduced proximally with a collapsing, variable nature and she was unable to perform ankle dorsiflexion. Hoover’s sign was positive. She had slight impairment of dexterity in the right arm, but there was no bradykinesia. The stretch reflexes were normal and symmetrical and the plantar responses down going. On pen-and-paper tasks, she had right-sided small script but there was no size decrement, and the Archimedes spiral was small in size with consistent spiral turn spacing (figure 1A, B). MRI brain showed a large left parafalcine meningioma with areas of acute/subacute blood internally and intense enhancement with probable infiltration of the superior sagittal sinus at which point the mass crossed the midline. There was no basal ganglia distortion by the mass (figure 2). She was managed with a combination of surgical debulking and tapering oral steroid therapy. Her writing and spiral assessments improved over the course of treatment with an initial dramatic improvement noted shortly after the initiation of steroid (figure 1C, D).

Figure 1

Writing and Archimedes spirals before and after treatment.

Discussion

Although Pick and later Kinnier Wilson1 considered micrographia, defined as a reduction in size in a patient’s handwriting, as probably being of cortical origin, it is largely considered a sign of basal ganglia disease in contemporary practice. While there are a range of reports implicating cortical pathology including masses resulting in parkinsonism, cases resulting in isolated micrographia are rare and include frontal infarction2 and parietal demyelination.3 The dramatic improvement with corticosteroids in our patient is presumably due to a resolution in oedema and is similar to the response seen in a case reported with multiple sclerosis.3 Further support for a cortical origin of micrographia come from postoperative inadvertent ‘lesioning’ of the dominant frontoparietal region; stimulation studies implicating Brodmann area 6 and a recent functional imaging study suggesting an aberrance in the network involved in the programming and execution of motor sequences (the supplementary motor area (SMA), pre-SMA and rostral cingulate motor area) may be responsible.4

The correlation between micrographia severity in Parkinson’s disease and bradykinesia occurring on finger tapping is not strong, and it is possible that when it occurs, a disturbance in cortical motor circuitry may also be involved. However, the distinctive progressive reduction in the size of the handwriting is what distinguishes it easily from the ‘cortical’ micrographia seen in this case. The Archimedes spiral drawing in Parkinson’s disease is typically asymmetric and small, with spirals compressing further with turns, which were not seen in our patient. In right-handed patients with Parkinson’s disease, the script also tends to slope upwards, which was not the case in this patient. In patients with pathologically defined progressive supranuclear palsy and in focal pallidal disease, ‘pallidal handwriting’ is occasionally seen in which the script is extremely small but the speed of writing is normal or increased, and there is no decrement in the size of letters.

This case highlights the potential use of writing and spiral assessments in cases with functional features and few objective motor abnormalities, and the contribution of cortical pathology to micrographia.

Acknowledgments

We are grateful to Dr Rebecca Liu, and the patient for allowing us to make this report and to Associate Professor Peter Kempster for advice on the interpretation.

References

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Footnotes

  • Contributors NV: study concept and design, acquisition and interpretation of data, drafting and revision of manuscript. AJL: interpretation of data, drafting and revision of manuscript. HRM: interpretation of data, drafting and revision of manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

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

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