Electromyographic findings in primary lateral sclerosis during disease progression

Silva, Cláudia Santos; Santos, Miguel Oliveira; Gromicho, Marta; Pinto, Susana; Swash, Michael; Carvalho, Mamede de

doi:10.1016/j.clinph.2021.08.022

Cited by 8 publications

(6 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Patients underwent regular EMG in one-to-two-year intervals, and although in some patients it showed stable minor changes, none of them fulfilled electrodiagnostic criteria for ALS. 4,6 All patients had a normal cerebrospinal fluid analysis and brain and spinal cord magnetic resonance imaging.…”

Section: Methodsmentioning

confidence: 99%

“…that is, age > 25 years; symptoms of progressive UMN dysfunction (in at least two of three regions) for at least 4 years; absence of relevant LMN signs for ≥4 years from symptom onset, and absence of sensory, ocular, cerebellar, or cognitive symptoms/signs. Patients underwent regular EMG in one‐to‐two‐year intervals, and although in some patients it showed stable minor changes, none of them fulfilled electrodiagnostic criteria for ALS 4,6 . All patients had a normal cerebrospinal fluid analysis and brain and spinal cord magnetic resonance imaging.…”

Section: Methodsmentioning

confidence: 99%

“…[1][2][3] Although the absence of LMN involvement is the core feature to differentiate PLS from ALS, minor and stable electromyographic (EMG) abnormalities may be present in PLS patients. 4 The differentiation between PLS and HSP can be much more challenging, as they can both be UMN lower limb onset syndromes. This is especially true of pure forms of HSP or in the absence of a family history.…”

Section: Introductionmentioning

confidence: 99%

“…The mean onset age is around 50 years old, which is approximately a decade earlier than ALS, and a decade later than HSP 1–3 . Although the absence of LMN involvement is the core feature to differentiate PLS from ALS, minor and stable electromyographic (EMG) abnormalities may be present in PLS patients 4 . The differentiation between PLS and HSP can be much more challenging, as they can both be UMN lower limb onset syndromes.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Upper motor neuron signs in primary lateral sclerosis and hereditary spastic paraplegia

Santos Silva,

Correia Rodrigues,

Fortuna Baptista

et al. 2024

Muscle and Nerve

Self Cite

View full text Add to dashboard Cite

Introduction/AimsThe frequency and distribution of upper motor neuron (UMN) signs in primary lateral sclerosis (PLS) are unknown. We aimed to study the spectrum of UMN signs in PLS and compare it with hereditary spastic paraplegia (HSP).MethodsWe retrospectively analyzed the frequency of different UMN signs, including hyperreflexia (limbs and jaw), limb and tongue spasticity, Babinski, and Hoffman signs, in PLS patients at first observation and compared this respect to onset region and symptom duration. We also compared PLS versus HSP patients.ResultsWe included 34 PLS and 20 HSP patients, with a median symptom duration at first visit of 3.0 (interquartile range, IQR = 4.0) and 19.0 (IQR = 22.0) years, respectively. In PLS patients, hyperreflexia of upper (UL) (88.2%) and lower (LL) (91.2%) limbs, and LL spasticity (79.4%) were the most common findings. Spasticity of LL was significantly (p = .012) more frequent in LL‐spinal onset subgroup, tongue spasticity in bulbar‐onset subgroup (p = .021), and Hoffman sign in UL‐spinal onset subgroup (p = .024). The PLS subgroup with shorter disease duration had a higher frequency of abnormal jaw jerk reflex (p = .037). Compared with HSP, PLS patients had a higher frequency of UL hyperreflexia (88.2% vs. 42.1%, p < .001) and UL spasticity (44.1% vs. 0.0%, p < .001). Asymmetric distribution of UMN signs was present in PLS and not in HSP.DiscussionIn PLS, UL UMN signs are nearly always present and UMN sign distribution appears to be associated with onset region. At first observation, bulbar involvement, asymmetrical distribution of UMN signs and UL spasticity may indicate PLS versus HSP.

show abstract

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Upper motor neuron signs in primary lateral sclerosis and hereditary spastic paraplegia

Santos Silva,

Correia Rodrigues,

Fortuna Baptista

et al. 2024

Muscle and Nerve

Self Cite

View full text Add to dashboard Cite

show abstract

“…A longitudinal study included 22 patients with PLS who received 2-3 EMG studies over a median time of ;50 months, for an extreme interval of 24 years between symptom onset and the last study. 8 The EMG results included abnormal spontaneous activity and subjective assessment of the full interference pattern. Fasciculation potentials were common, but positive waves and fibrillation potentials were rare, and some patients had scattered motor unit changes in a limited number of muscles, but no abnormality increased over subsequent studies in the setting of progressive loss of patient function, and no patient progressed to ALS.…”

Section: Primary Lateral Sclerosismentioning

confidence: 99%

What Is in the Literature

Bromberg

2022

Journal of Clinical Neuromuscular Disease

View full text Add to dashboard Cite

This issue of What Is in the Literature focuses on articles on amyotrophic lateral sclerosis over the past year. Amyotrophic lateral sclerosis remains a challenging disorder with progression to death. Within the past year, a phase 2 trial of a drug combination showed slowing in the rate of progression. While awaiting a phase 3 trial or approval by the Food and Drug Administration, selected articles that aid the diagnosis, contribute to care, or add to general knowledge about the disease are reviewed.

show abstract