Mills’ syndrome: an italian case and revision of the literature

Rigamonti, Andrea; Lauria, Giuseppe; Prone, Valentina; Agostoni, Elio

doi:10.1007/s10072-012-0979-z

Cited by 6 publications

(4 citation statements)

References 5 publications

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“…Patient 3 noticed symptoms a few weeks after recovering from a low velocity road traffic accident. A similar temporal relationship to trauma was noted in a previous case report, in which paresis began developing soon after the patient had sustained open fractures of the same limb [28]. For MND, a potential link with preceding trauma has long been an area of controversy.…”

Section: Discussionsupporting

confidence: 69%

“…Whilst needle EMG, SEP and VEP are important in excluding other diagnoses, MEP can confirm the presence of corticospinal tract disease. MEP have been reported in three previous cases [28, 30, 33] and showed unilaterally prolonged central motor conduction times and—in one case—borderline central motor conduction times in the contralateral lower limb. In our cohort, abnormalities in central motor conduction times were also unilateral, except in patient 1, where MEP were also delayed in the asymptomatic contralateral upper limb when tested 14 years after onset (Fig.…”

Section: Discussionmentioning

confidence: 83%

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Mills’ syndrome revisited

et al. 2019

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Mills’ syndrome is an idiopathic, slowly progressive, spastic hemiparesis. We describe three cases that have been under review for a minimum of 11 years (range 11–19). In all patients, symptoms started in a leg, with a mean age of onset of 59 years (range 53–63). The only abnormality on laboratory investigations was a mildly elevated CSF protein in one case. MRI demonstrated focal T2 hyper-intensity located eccentrically in the cervical cord ipsilateral to the symptomatic side. No cerebral abnormality was demonstrated. Whilst visual and somatosensory evoked potentials were unremarkable, motor evoked potentials were abnormal in all patients: central motor conduction times were significantly prolonged unilaterally in two patients and bilaterally but asymmetrically in the third. Beta-band (15–30 Hz) intermuscular coherence, a potentially more sensitive method of assessing upper motor neuron integrity, was absent unilaterally in one patient and bilaterally in the other two. One patient developed amyotrophy and thus a picture of amyotrophic lateral sclerosis after 16 years, suggesting that Mills’ syndrome is part of the motor neuron disease spectrum. Both amyotrophy and subclinical contralateral upper motor neuron disease can therefore be features of Mills’ syndrome. However, even with the most sensitive electrodiagnostic techniques, unilateral upper motor neuron disease can remain the only abnormality for as long as 10 years. We conclude that whilst Mills’ syndrome should be classified as a motor neuron disorder, it is a distinct nosological entity which can be distinguished from amyotrophic lateral sclerosis, upper motor neuron-dominant amyotrophic lateral sclerosis and primary lateral sclerosis. We propose diagnostic criteria for Mills’ syndrome, and estimate a point prevalence of at least 1.2:1,000,000 based on our well-defined referral population in the North of England.

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Section: Discussionsupporting

confidence: 69%

Section: Discussionmentioning

confidence: 83%

Mills’ syndrome revisited

et al. 2019

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“…This latter condition, also known as the "hemiplegic variant", is characterized by slow progressive ascending weakness, usually starting in a distal lower limb, and then progressing to a proximal ipsilateral lower limb and upper limb, associated with pyramidal signs on the affected side, and sometimes also on the contralateral one [58]. Facial and bulbar weakness may be present, as well as slight sensory disturbances [59]. In most patients, the syndrome remained strictly unilateral after 15 years, although the involvement of the contralateral side has been reported in about 30% [59].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Facial and bulbar weakness may be present, as well as slight sensory disturbances [59]. In most patients, the syndrome remained strictly unilateral after 15 years, although the involvement of the contralateral side has been reported in about 30% [59]. The scarcity of reports on this condition, as well as the paucity of complementary resources necessary to better define its pathophysiological mechanisms, led to doubt about the authenticity of this entity [58].…”

Section: Clinical Featuresmentioning

confidence: 99%

Primary Lateral Sclerosis: An Overview

Vacchiano,

Bonan,

Liguori

et al. 2024

JCM

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Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder which causes the selective deterioration of the upper motor neurons (UMNs), sparing the lower motor neuron (LMN) system. The clinical course is defined by a progressive motor disability due to muscle spasticity which typically involves lower extremities and bulbar muscles. Although classically considered a sporadic disease, some familiar cases and possible causative genes have been reported. Despite it having been recognized as a rare but distinct entity, whether it actually represents an extreme end of the motor neuron diseases continuum is still an open issue. The main knowledge gap is the lack of specific biomarkers to improve the clinical diagnostic accuracy. Indeed, the diagnostic imprecision, together with some uncertainty about overlap with UMN-predominant ALS and Hereditary Spastic Paraplegia (HSP), has become an obstacle to the development of specific therapeutic trials. In this study, we provided a comprehensive analysis of the existing literature, including neuropathological, clinical, neuroimaging, and neurophysiological features of the disease, and highlighting the controversies still unsolved in the differential diagnoses and the current diagnostic criteria. We also discussed the current knowledge gaps still present in both diagnostic and therapeutic fields when approaching this rare condition.

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Mills’ syndrome: progressive hemiplegia with atrophy of unilateral cerebral peduncle and pontine base

Wang

Chen

2015

Neurol Sci

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Mills’ syndrome: an italian case and revision of the literature

Cited by 6 publications

References 5 publications

Mills’ syndrome revisited

Mills’ syndrome revisited

Primary Lateral Sclerosis: An Overview

Mills’ syndrome: progressive hemiplegia with atrophy of unilateral cerebral peduncle and pontine base

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