Primary lateral sclerosis presenting parkinsonian symptoms without nigrostriatal involvement

Mabuchi, Naoki; Watanabe, Haruo; Atsuta, Naoki; Hirayama, Masaaki; Ito, Hiroki; Fukatsu, Hiroshi; Kato, Takashi; Ito, Keiichi; Sobue, G

doi:10.1136/jnnp.2003.035212

Cited by 28 publications

(17 citation statements)

References 17 publications

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“…FDG PET scans (n = 3) showed reduced uptake in frontal areas. The authors postulated that the extrapyramidal features were likely due to frontal lobe involvement 1. Whether we can extrapolate their findings to our patients is questionable, as none of our patients demonstrated fatiguing or decrement of repeated finger/foot tapping.…”

Section: Discussionmentioning

confidence: 60%

Primary lateral sclerosis mimicking atypical parkinsonism

Bhatia

Østergaard

et al. 2007

Movement Disorders

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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.

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Section: Discussionmentioning

confidence: 60%

Primary lateral sclerosis mimicking atypical parkinsonism

Bhatia

Østergaard

et al. 2007

Movement Disorders

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“…18‐Fluorodeoxyglucose positron emission tomography (FDG‐PET) imaging studies of patients with PLS and clinical parkinsonism showed decreased uptake in the precentral gyrus, involving the prefrontal, medial frontal, and cingulate areas. 6‐[ 18 F]fluoro‐ L ‐dopa (F‐DOPA) PET was normal, arguing that degeneration of the precentral gyrus may be responsible for clinicalparkinsonism in at least some cases of PLS‐parkinsonism 19…”

Section: Discussionmentioning

confidence: 99%

Parkinsonism and motor neuron diseases: Twenty‐seven patients with diverse overlap syndromes

et al. 2010

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It has long been recognized that signs of motor neuron disease (MND) may accompany clinical evidence of parkinsonism in different neurodegenerative conditions. By using the Columbia University Division of Movement Disorders database, we reviewed data from 5,500 cases of parkinsonism and recorded the presence of upper motor neuron (UMN) dysfunction, lower motor neuron (LMN) dysfunction, or both. Among the 27 patients so identified, we counted those with autonomic dysfunction, cerebellar dysfunction, or dementia. Among the 27 cases, seven had UMN signs and LMN signs as well as parkinsonism and were diagnosed with amyotrophic lateral sclerosis (ALS)-parkinsonism (Brait-Fahn disease). Three of the seven had dementia that was not deemed to be frontotemporal dementia (FTD). Six other patients had no LMN signs but had UMN signs and parkinsonism and were classified as having primary lateral sclerosis (PLS)-parkinsonism. Four patients had both UMN and LMN signs with parkinsonism as well as the characteristic dementia of FTD; they were diagnosed with FTD-parkinsonism-ALS. Seven patients had MND, parkinsonism, and autonomic or cerebellar dysfunction, a combination compatible with multiple system atrophy (MSA). Three patients had syndromes compatible with hereditary spastic paraplegia (HSP). In sum, we found that MND occurs in association with diverse parkinsonian syndromes; some are heritable, others sporadic and causes are uncertain. Having MND may be a risk factor for parkinsonism. A prospective study may elucidate this possibility.

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“…Primary lateral sclerosis (PLS), a rare, pure (at least initially) upper motor neurone variant of motor neurone disease, is sometimes mistaken for atypical parkinsonism 1–3. The disease is gradually progressive with prominent spinal or bulbar spasticity and, often for a long duration, only minimal motor weakness 3–6.…”

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confidence: 99%