Hemiparetic Primary Lateral Sclerosis: Revisiting Mills Syndrome

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Abstract

A slowly progressive hemiparesis beginning in a single limb with evolution to the ipsilateral limb was originally described in 8 patients in 1906 by Mills. We present 5 cases of progressive hemiparetic corticospinal tract degeneration, identified by the clinical presentation and the exclusion of other etiologies using serological, imaging, and electrodiagnostic studies.

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Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria.

C. E. Pringle, A. Hudson, D Munoz … (1992)

Eight patients with a homogeneous syndrome of progressive symmetric spinobulbar spasticity were studied. Clinical features were limited to those associated with dysfunction of the descending motor tracts and included spastic quadriparesis, pseudobulbar affect, spastic dysarthria, hyper-reflexia and bilateral Babinski signs. Lower motor neuron findings were absent and higher cognitive function preserved. Median age of onset was 50.5 yrs and median disease duration was 19 yrs. Neuropathologic features (including morphometric analysis) in the single autopsied case confirmed the selective involvement of the motor cortex. There was complete absence of Betz cells from layer 5 of the precentral cortex and the remaining pyramidal cells were significantly smaller than those seen in normal controls. Magnetic resonance imaging (MRI) revealed atrophy of the precentral gyrus and positron emission tomography (PET) scans showed diminished glucose [18F]fluorodeoxyglucose uptake in the pericentral cortex. Magnetic motor cortex stimulation revealed markedly prolonged central motor conduction times. The literature is reviewed and diagnostic criteria for primary lateral sclerosis based on clinical, laboratory and imaging features are proposed.

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Onset, natural history and outcome in idiopathic adult motor neuron disease.

Forbes Norris, Rodger Shepherd, Eric Denys … (1993)

Cases of adult-onset idiopathic motor neuron disease (MND) identified from January 1970 through December 1986 were studied in a defined area of California. The patients were followed prospectively throughout the illness in 99% of cases. Among 708 cases aged 25-74 years at onset, the most common type (86%) was typical, sporadic amyotrophic lateral sclerosis (SporALS). The risk of bulbar onset and shorter survival times increased with age in both men and women. About 4%, mainly younger men, experienced unusually long courses with milder paralysis, but could not be identified early in the illness. They probably represent one extreme of the ALS spectrum rather than a distinct subtype. Familial ALS (FamALS) was diagnosed in 7%. It developed earlier in life but ran a slightly longer course, which suggests a different disease process. Overall there was a statistically significant predominance of males, especially in 17 cases (2%) of progressive muscular atrophy (PMA). There were 26 cases (4%) classified as primary lateral sclerosis (PLS). Progressive bulbar palsy was not found; that diagnosis usually denotes merely the bulbar onset of ALS.

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Mills' and other isolated upper motor neurone syndromes: in vivo study with 11C-(R)-PK11195 PET.

Chris Shaw, N Leigh, M. Turner … (2005)

(11)C-(R)-PK11195 positron emission tomography (PET) was used to explore and delineate in vivo the cortical lesion in three clinically isolated upper motor neurone syndromes of similar presentation, with reference to the syndrome of progressive spastic hemiparesis first described by Mills. Three patients with isolated UMN syndromes underwent (11)C-(R)-PK11195 PET of the brain. One patient fulfilled criteria for PLS. Two others had clinical features similar to the cases described by Mills; one of these had a high cervical cord inflammatory lesion previously noted on magnetic resonance imaging. The patient with PLS showed focal increase in the binding of (11)C-(R)-PK11195, indicating microglial activation, in the motor cortex contralateral to the predominantly affected limbs. Of the other two patients, one demonstrated marked increases in binding in the superior frontal region (supplementary motor region) contralateral to the affected limbs. In contrast, no focal areas of increased binding were seen in the cerebral cortex of the third patient, who had a high cervical cord lesion and was presumed to have extra-cerebral inflammatory disease. (11)C-(R)-PK11195 PET demonstrates in vivo that active pathology may be detectable many years after the onset of symptoms, and that it can occur in disparate sites with clinically similar presentations. We conclude that Mills' syndrome is a purely clinical description that should be reserved for patients with a progressive spastic hemiparesis for which no other explanation can be found.

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Journal

Journal ID (publisher-id): CRN

Journal ID (pmc): CRN

Journal ID (doi): 10.1159/issn.1662-680X

Title: Case Reports in Neurology

Publisher: S. Karger AG

ISSN (Electronic): 1662-680X

Publication date Subscription-year: 2015

Publication date Collection: September – December 2015

Publication date (Electronic): 24 September 2013

Volume: 7

Issue: 3

Pages: 191-195

Affiliations

^aDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, Md., ^bNeurology Specialists of Monmouth County, West Long Branch, N.J., and ^cDepartment of Neurology, Drexel University College of Medicine, Philadelphia, Pa., USA

Author notes

*Nicholas J. Maragakis, MD, Department of Neurology, Johns Hopkins University School of Medicine, 855 North Wolfe St., Rm 248, Baltimore, MD 21205 (USA), E-Mail nmaragak@jhmi.edu

Article

Publisher ID: 440713 Pmcid ID: PMC4611067 Other ID: Case Rep Neurol 2015;7:191-195

DOI: 10.1159/000440713

PMC ID: PMC4611067

PubMed ID: 26500542

SO-VID: 3b06f93c-4e0a-4109-b55d-782485153565

License:

Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) ( http://www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

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Hemiparetic Primary Lateral Sclerosis: Revisiting Mills Syndrome

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Abstract

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Karger: Neurology and Neuroscience

Most cited references 12

Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria.

Onset, natural history and outcome in idiopathic adult motor neuron disease.

Mills' and other isolated upper motor neurone syndromes: in vivo study with 11C-(R)-PK11195 PET.

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