Rev Clin Esp. 2003 Aug;203(8):373-7.
[Neurological involvement in systemic
sclerosis]
[Article in Spanish]
Campello Morer I, Velilla Marco J, Hortells Aznar JL, Almárcegui Lafita C, Barrena
Caballo R, Oliveros Juste A.
Unidad de Neurología. Fundación Hospital Manacor. Baleares. Spain.
isacam@saludalia.com
Comment in:
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Rev Clin Esp. 2003 Aug;203(8):361-2.
Abstract
INTRODUCTION: Systemic sclerosis (SS) is recognized as the connective tissue
disease which less frequently presents neurological complications; in recent studies it is
demonstrated, however, that neurological involvement in SS is more frequent of what it
had been assumed. PATIENTS AND METHODS: Clinical neurological exploration
was done in 26 patients with definitive SS; an electroneurogram was carried out in 23
cases in order to determine the prevalence of central neurological pathology and of
peripheral neuropathy, to define its characteristics, and to investigate possible
associations with clinical parameters and with autoimmunity. RESULTS: 23 cases
(88%) were females and 3 cases (12%) males; the median age was 57.5 12.0 (SD) years,
while the median age to the diagnosis was 51.3 12.3 (SD) years and the median period
of natural history of disease was 6.2 3.1 years. Seven patients (26.9%) showed
involvement of the CNS, being the headache and the neuropsychiatric manifestations
the most common conditions (11.5%). Peripheral neuropathy prevalence was 39.1% (9
cases); according to the distribution of the injury, the polyneuropathy prevailed in
30.4% of cases. With regard to the functional selectivity, the sensitive-motor forms
were most frequent (55.6%); according to the most involved structure, the axonal
neuropathy was most common (44.4%).Discussion. The possible pathogenic
mechanisms of the neurological pathology in this disease are discussed.
Clin Exp Dermatol. 1990 Mar;15(2):91-4.
Neurological complications of systemic
sclerosis--a report of three cases and
review of the literature.
Berth-Jones J, Coates PA, Graham-Brown RA, Burns DA.
Department of Dermatology, Leicester Royal Infirmary, UK.
Comment in:
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Clin Exp Dermatol. 1991 Sep;16(5):403.
Abstract
We report three cases of systemic sclerosis demonstrating four different neurological
complications: trigeminal neuropathy, peripheral neuropathy, carpal-tunnel syndrome
and prolonged response to local anaesthesia. A review of the literature reveals a wide
range of neurological abnormalities associated with systemic sclerosis. When they
occur, these are often presenting features.
PMID: 2161303 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms
Publication Types:
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Case Reports
Review
MeSH Terms:
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Adult
Anesthesia, Local/adverse effects
Carpal Tunnel Syndrome/etiology
Cranial Nerve Diseases/etiology*
Female
Humans
Middle Aged
Peripheral Nervous System Diseases/etiology*
Scleroderma, Systemic/complications*
Trigeminal Nerve
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Muscle Nerve. 2003 Sep;28(3):330-5.
Peripheral neuropathy in scleroderma.
Poncelet AN, Connolly MK.
Department of Neurology, Box 0114, University of California, San Francisco, San
Francisco, California 94143, USA. ponce@itsa.ucsf.edu
Abstract
Because patients with scleroderma report neuropathic symptoms including numbness,
paresthesias, and dysesthesias, we assessed peripheral nerve function in such patients.
Fourteen scleroderma patients underwent complete neurologic examination, nerve
conduction studies (NCS) and quantitative sensory testing (QST). Neurologic
examination revealed reduced vibration (7) or pinprick (4) sensation in the upper or
lower extremities, focal atrophy or proximal weakness (2), and decreased deep tendon
reflexes (2). NCS showed reduced sensory nerve action potentials (1) and carpal tunnel
syndrome (1). QST of the upper and lower extremity revealed increased cold or
vibration detection thresholds in 8 of 14 patients. Our findings suggest that peripheral
neuropathy occurs in patients with scleroderma at a higher frequency than previously
appreciated. These findings cannot be ascribed to compression neuropathies, but rather
involve large and small fibers in a non-length-dependent fashion. Larger, prospective
studies using the more sensitive QST as well as pathologic studies of nerve, including
cutaneous innervation, are needed to further assess the characteristics and etiology of
the neuropathy.
PMID: 12929193 [PubMed - indexed for MEDLINE]
Clin Exp Rheumatol. 1996 Nov-Dec;14(6):601-5.
Peripheral nervous system involvement
in systemic sclerosis: the median nerve as
target structure.
Lori S, Matucci-Cerinic M, Casale R, Generini S, Lombardi A, Pignone A, Scaletti C,
Gangemi PF, Cagnoni M.
Servizio di Neurofisiopatologia, USL 10, Florence, Italy.
Abstract
OBJECTIVES: To investigate the frequency and the main electrophysiological
characteristics of the canalicolar passage nerve involvement in patients with systemic
sclerosis (SSc). METHODS: Thirty-two SSc patients were enrolled in the study,
classified according to the type (diffuse or limited) and the duration (> / < 5 years) of
the disease. Sensory-motor nerve conduction studies (NCS) of the upper and lower
limbs, in particular at the critical canalicolar points, were conducted by recording the
Compound Muscular Action Potential (CMAP) and the Sensory Action Potential
(sNAP). The following parameters were evaluated: Motor Nerve Conduction Velocity
(MNCV) and Sensory Nerve Conduction Velocity; distal and proximal latency of the
CMAP and the onset and peak latency of the sNAP; peak-peak amplitude and negativepeak area of the CMAP and sNAP; and the Terminal Latency Index (TLI) (Terminal
Distance/MCNV x Distal latency). RESULTS: Four (12.5%) patients had a distal
neuropathy of the upper limbs (one with monolateral and two with bilateral involvement
of the median nerve and one bilateral involvement of the ulnar nerve). Fourteen (43.7%)
patients showed a decrement of the median nerve TLI and seven (21.8%) of either the
median or the ulnar nerve (Table I). Motor and sensitive conduction velocity and
latency studies did not show a statistical difference between SSc patients and controls.
The amplitude and area of the CMAP (distal and proximal), sNAP and of the median
nerve TLI were significantly decreased in patients with respect to controls.
CONCLUSION: Distal mononeuropathy of the median nerve was the most frequent
result in our patients. The involvement of the peripheral nervous system seems to be
strictly topographical, following the modifications of the tissues and vascular tone
(Raynaud's phenomenon) at the upper acral level. The neurophysiological alterations
detected in our study at the wrist level may not be linked merely to a compressive event
but also to microvascular involvement. Nerve involvement closely connected with the
pathogenesis and distribution of SSc should be considered when peripheral nervous
system involvement is the initial symptom of the disease.
PMID: 8978953 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms
Publication Types:
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Clinical Trial
Randomized Controlled Trial
MeSH Terms:
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Adult
Aged
Aged, 80 and over
Electrophysiology/methods
Evoked Potentials, Motor
Female
Humans
Male
Median Nerve/physiopathology*
Middle Aged
Neural Conduction/physiology*
Peripheral Nervous System Diseases/etiology*
Peripheral Nervous System Diseases/physiopathology
Psychomotor Performance/physiology*
Scleroderma, Systemic/complications*
Scleroderma, Systemic/physiopathology
Ulnar Nerve/physiopathology
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Q J Med. 1991 Aug;80(292):661-75.
Peripheral nerve dysfunction in
scleroderma.
Schady W, Sheard A, Hassell A, Holt L, Jayson MI, Klimiuk P.
Department of Neurology, University of Manchester, Manchester Royal Infirmary,
Salford.
Abstract
Peripheral neuropathy in patients with scleroderma is thought to be rare. We have
undertaken a quantitative assessment of peripheral nerve function in 29 patients with
either limited cutaneous scleroderma or progressive systemic sclerosis. Tactile
thresholds were raised in the fingers in 28 per cent of patients and in the foot in 50 per
cent. Two-point discrimination was abnormal in 10 patients, thermal thresholds were
abnormal in five and vibration thresholds were abnormal in one. Nerve conduction
studies showed abnormalities in six patients, five of whom had clinical signs of a mild
peripheral neuropathy: the mean duration of disease in these six patients was 10 years
longer than that in the remainder of the patients. There was electrophysiological
evidence of a subclinical carpal tunnel syndrome in two patients. The sympathetic skin
response was recorded in 16 patients who had not been subjected to sympathectomy for
Raynaud's phenomenon, and was abnormal in four. These results indicate that
peripheral nerve dysfunction in scleroderma, though mild, is not as uncommon as
previously thought. The abnormal cutaneous sensory thresholds may be partly due to
altered viscoelastic properties of the skin, but abnormal responses in the lower limbs to
tests of tactile sensitivity, the clinical findings and the disturbances of nerve conduction
argue in favour of an additional neuropathic process in some patients. Low grade distal
nerve trunk ischaemia may be responsible.
Rheum Dis Clin North Am. 1996 Nov;22(4):879-92.
The nervous system in systemic sclerosis
(scleroderma). Clinical features and
pathogenetic mechanisms.
Cerinic MM, Generini S, Pignone A, Casale R.
Institute of Internal Medicine IV, University of Florence, Italy.
Abstract
The involvement of the nervous system in SSc is well recognized today. Different
pathogenetic mechanisms are suggested that may alternatively explain the multiform
appearance of the clinical spectrum (mononeuritis, mononeuritis multiplex, carpal
tunnel syndrome, and so forth). It is now clear that the ANS is the earliest structure
targeted by the disease in the gastrointestinal tract. The importance of this observation
has not yet been adequately interpreted but may, together with the increasing evidence
of the nervous system involvement in SSc, become a leading factor in understanding of
the importance of the nervous system in the onset, development, and maintenance of the
disease.
PMID: 8923601 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms
Adv Exp Med Biol. 1999;455:73-83.
Systemic sclerosis. A clinical overview.
Generini S, Fiori G, Moggi Pignone A, Matucci Cerinic M, Cagnoni M.
Department of Medicine, University of Florence, Italy.
Abstract
Systemic Sclerosis (SSc) is a multisystem disease that affects the skin and internal
organs (i.e., gastrointestinal tract, lung, heart, kidney and peripheral nervous system). In
the early phase, lung involvement is characterized by interstitial inflammatory
alterations that are detected by bronchoalveolar lavage analysis and high resolution
computed tomography (ground glass). As the disease progresses, fibrotic changes
become evident and the diffusing capacity for carbon monoxide (DLCO) is impaired.
Cardiac involvement in SSc can be manifested as myocardial disease, pericardial
disease, conduction system disease, or arrhythmias. Cardiac involvement is a poor
prognostic factor, but the diagnosis may be late or missing because of the frequent
discrepancy between clinical manifestations and the real cardiac involvement. For this
reason, resort to all the available diagnostic procedures is recommended to achieve an
early diagnosis. The motility disorders are a major feature of gastrointestinal
involvement in SSc, striking any part of this system (especially esophagus and anorectal
region). Kidney involvement and scleroderma renal crisis are now considered rare
because of the introduction of ACE inhibitors. Some patients may develop myositis or
erosive arthropathy that complicate enormously the joint retraction induced by skin
fibrosis. The peripheral nervous system (PNS) is also targeted by SSc: a distal
mononeuropathy of the median nerve is a frequent and early feature; autonomic nerve
dysfunction (parasympathetic impairment and marked sympathetic overactivity) seems
to be a fundamental etiologic factor linked to the development of microvascular, cardiac
and gastrointestinal alterations. The whole approach to the SSc patient is very complex
and must consider, at the same time, many organs and systems. Thus, a global vision of
SSc patient is needed in order to assure an early diagnosis of specific organ involvement
as well as early treatment. Systemic Sclerosis (SSc) is a multisystem disease, that
affects the skin, the gastrointestinal tract, the lung, the heart and the kidney. The extent
and severity of internal organ involvement are the more important factors influencing
the disease outcome and prognosis in SSc. In recent years, it has become evident that
early diagnosis and accurate staging of visceral involvement are fundamental for
appropriate management and therapeutic approach to the disease. Diagnostic procedures
for specific organ and system involvement are now more sensitive because of the
continuous technological improvement and, mostly, because they take advantage of the
studies carried out in other diseases by other medical branches. This review will
consider briefly the most frequent and important organ involvement in SSc.
PMID: 10599326 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms
Publication Types:
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Review
MeSH Terms:
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Gastrointestinal Diseases/physiopathology
Heart Diseases/physiopathology
Humans
Kidney Diseases/physiopathology
Lung Diseases/physiopathology
Musculoskeletal Diseases/physiopathology
Nervous System Diseases/physiopathology
Scleroderma, Systemic/physiopathology*
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Scand J Rheumatol. 1999;28(4):260-1.
Polyneuropathy as initial manifestation
of systemic sclerosis (scleroderma).
Knupp-Oliveira S, Cerinic MM.
Department of Pediatrics, Universidade Federal do Rio de Janeiro, Brasil.
Abstract
We report the first case of a young female patient who developed a sensory-motor
polyneuropathy, without any skin or internal involvement characteristic of SSc, but with
a serological positivity of antitopoisomerase I antibodies. After 4 years she developed a
rapid skin tightening with lung involvement, in a full blown picture of the diffuse subset
of SSc. The case suggests that the peripheral nervous system deserves more attention, in
particular in the earliest phase of SSc.
PMID: 10503566 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms, Substances
Publication Types:
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Case Reports
Review
MeSH Terms:
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Adult
Autoantibodies/blood
DNA Topoisomerases, Type I/immunology
Diagnosis, Differential
Female
Humans
Peripheral Nervous System Diseases/etiology*
Peripheral Nervous System Diseases/physiopathology
Scleroderma, Systemic/diagnosis*
Scleroderma, Systemic/physiopathology
Substances:
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Autoantibodies
DNA Topoisomerases, Type I
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Rev Neurol (Paris). 1989;145(3):236-8.
[Sensory neuropathy of the trigeminal
nerve and scleroderma]
[Article in French]
Fain O, Guillevin L, Giroux C, Gayraud M, Royer I.
Service de Médecine Interne, Hôpital Avicenne, Bobigny.
Abstract
Sensory neuropathy of trigeminal nerve is one of the neurological complications of
systemic diseases, particularly scleroderma. Three cases are reported in which onset
occurred at different stages of the disease, the main symptom being painful
dysesthesias. All three branches of the trigeminal nerve may be affected but a
preference is apparent for V2 and V3. Signs are usually limited to hypoesthesia and the
chronic course is influenced little by treatment.
PMID: 2664978 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms
Publication Types:
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Case Reports
English Abstract
Review
MeSH Terms:
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Cranial Nerve Diseases/complications
Cranial Nerve Diseases/physiopathology
Electrophysiology
Female
Humans
Middle Aged
Scleroderma, Systemic/complications*
Scleroderma, Systemic/diagnosis
Scleroderma, Systemic/drug therapy
Sensation*
Trigeminal Nerve*
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Dermatology. 1996;193(1):22-6.
Progressive systemic sclerosis associated
with multiple mononeuropathy.
Nitta Y, Sobue G.
Department of Dermatology, Aichi Medical University, Japan.
Abstract
BACKGROUND: Progressive systemic sclerosis (PSS) is a chronic connective tissue
inflammatory disease which commonly attacks the skin and the visceral organs, but
rarely the peripheral nervous system. OBJECTIVE: The aim of this study was to
investigate PSS accompanied by peripheral neuropathy clinically,
electrophysiologically and pathologically from a sural nerve biopsy. METHODS: Two
women suffering from PSS but without any other collagen disease were studied. Both
patients developed peripheral neuropathy with multiple mononeuropathy of the limbs,
and in one woman, in the trunk as well. RESULTS: A biopsy of the sural nerve revealed
axonal and myelin segmental degeneration, loss of large myelinated fibers and an
increase of collagen fibers, but there was no evidence of vasculitis. An electron
microscopic examination revealed degenerated axons, disrupted myelin sheaths and
multilayered basal lamina in the capillaries. CONCLUSION: Mononeuropathy in PSS
suggests that ischemic neuropathy may be related to the immune-mediated
vasculopathy.
PMID: 8864613 [PubMed - indexed for MEDLINE]
Publication Types, MeSH Terms, Substances
Publication Types:
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Case Reports
Review
MeSH Terms:
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Aged
Anti-Inflammatory Agents/therapeutic use
Axons/pathology
Biopsy, Needle
Electrophysiology
Female
Humans
Middle Aged
Peripheral Nervous System Diseases/complications
Peripheral Nervous System Diseases/drug therapy
Peripheral Nervous System Diseases/pathology*
Prednisolone/therapeutic use
Retrograde Degeneration*/immunology
Scleroderma, Systemic/complications
Scleroderma, Systemic/drug therapy
Scleroderma, Systemic/pathology*
Substances:
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Anti-Inflammatory Agents
Prednisolone
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