Primary lateral sclerosis mimicking atypical parkinsonism

Norlinah Mohamed Ibrahim, Kailash P. Bhatia, Karen Østergaard, Robin Howard, Gennarina Arabia, Niall P. Quinn

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa-unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes.

Original languageEnglish
Pages (from-to)2057-2062
Number of pages6
JournalMovement Disorders
Volume22
Issue number14
DOIs
Publication statusPublished - 31 Oct 2007

Fingerprint

Motor Neuron Disease
Parkinsonian Disorders
Fingers
Foot
Babinski's Reflex
Anterior Horn Cells
Chronic Progressive Multiple Sclerosis
Abnormal Reflexes
Dopamine Plasma Membrane Transport Proteins
Levodopa
Motor Neurons
Single-Photon Emission-Computed Tomography
Jaw
Diagnostic Errors
Gait
Age of Onset
Extremities

Keywords

  • Akinesia
  • Atypical parkinsonism
  • Extrapyramidal
  • Primary lateral sclerosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Mohamed Ibrahim, N., Bhatia, K. P., Østergaard, K., Howard, R., Arabia, G., & Quinn, N. P. (2007). Primary lateral sclerosis mimicking atypical parkinsonism. Movement Disorders, 22(14), 2057-2062. https://doi.org/10.1002/mds.21645

Primary lateral sclerosis mimicking atypical parkinsonism. / Mohamed Ibrahim, Norlinah; Bhatia, Kailash P.; Østergaard, Karen; Howard, Robin; Arabia, Gennarina; Quinn, Niall P.

In: Movement Disorders, Vol. 22, No. 14, 31.10.2007, p. 2057-2062.

Research output: Contribution to journalArticle

Mohamed Ibrahim, N, Bhatia, KP, Østergaard, K, Howard, R, Arabia, G & Quinn, NP 2007, 'Primary lateral sclerosis mimicking atypical parkinsonism', Movement Disorders, vol. 22, no. 14, pp. 2057-2062. https://doi.org/10.1002/mds.21645
Mohamed Ibrahim N, Bhatia KP, Østergaard K, Howard R, Arabia G, Quinn NP. Primary lateral sclerosis mimicking atypical parkinsonism. Movement Disorders. 2007 Oct 31;22(14):2057-2062. https://doi.org/10.1002/mds.21645
Mohamed Ibrahim, Norlinah ; Bhatia, Kailash P. ; Østergaard, Karen ; Howard, Robin ; Arabia, Gennarina ; Quinn, Niall P. / Primary lateral sclerosis mimicking atypical parkinsonism. In: Movement Disorders. 2007 ; Vol. 22, No. 14. pp. 2057-2062.
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