ABSTRACT
Early predictors of prognosis in Guillain-Barré syndrome (GBS) are needed to identify patients who are likely to make a poor recovery and to guide therapeutic decision-making in the acute phase. Here we investigate whether axonal protein biomarkers released into the cerebrospinal fluid (CSF) following proximal axonal damage improve the early prognostic accuracy in GBS. A prospective multicenter study including 132 patients (38 GBS, 38 neurological controls, 42 headaches, 14 chronic inflammatory demyelinating neuropathy). CSF levels of axonal [neurofilament (NfH) and tau] and glial (S100B and glial fibrillary acidic protein) protein biomarkers were measured on admission. Nerve conduction studies were performed at the time of lumbar puncture and patients were classified according to neurophysiological criteria. Outcome was assessed on the Hughes functional score (F-score). Poor outcome was defined as the inability to walk independently (F-score > or = 3). High NfH levels (>0.73 ng/ml) predicted poor outcome (P = 0.01) with an odds ratio of 7.3 and correlated with the outcome F-score (R = 0.51, P < 0.01), as did hTau levels (R = 0.47, P < 0.01). Patients with poor outcome had significantly higher CSF NfH (median 1.78 ng/ml) when compared to those with good outcome (0.03 ng/ml) or all of the control groups (neurological controls 0.18 ng/ml, headaches 0.06 ng/ml, chronic inflammatory demyelinating neuropathy 0.05 ng/ml). Except for age (P < 0.05) and need for ventilatory support (P < 0.05), none of the other features reliably predicted outcome. Improved prognostic accuracy in the acute phase of GBS seems possible using CSF NfH levels.
Subject(s)
Axons/pathology , Glial Fibrillary Acidic Protein/cerebrospinal fluid , Guillain-Barre Syndrome/cerebrospinal fluid , Nerve Growth Factors/cerebrospinal fluid , Neurofilament Proteins/cerebrospinal fluid , S100 Proteins/cerebrospinal fluid , tau Proteins/cerebrospinal fluid , Aged , Biomarkers/metabolism , Cross-Sectional Studies , Disease Progression , Female , Guillain-Barre Syndrome/physiopathology , Guillain-Barre Syndrome/therapy , Humans , Male , Middle Aged , Multivariate Analysis , Neural Conduction/physiology , Outcome Assessment, Health Care/methods , Prospective Studies , Reproducibility of Results , Retrospective Studies , S100 Calcium Binding Protein beta Subunit , Severity of Illness IndexSubject(s)
Connexins/genetics , DNA Mutational Analysis , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/genetics , Adult , Diagnosis, Differential , Female , Genetic Carrier Screening , Humans , Male , Middle Aged , Neural Conduction/genetics , Neurologic Examination , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Young Adult , Gap Junction beta-1 ProteinABSTRACT
Long-term morbidity from Guillain-Barré syndrome (GBS) is caused by axonal damage. This prospective study demonstrated that neurofilaments (NfHs), a biomarker for axonal damage, were of prognostic value in GBS. CSF NfH levels correlated with the F score and Medical Research Council summed score and were higher in patients with neurophysiologic evidence of axonal degeneration compared to those without. Pathologically high CSF NfH levels (>0.73 ng/mL) predicted worse motor and functional outcome.
Subject(s)
Guillain-Barre Syndrome/cerebrospinal fluid , Neurofilament Proteins/cerebrospinal fluid , Enzyme-Linked Immunosorbent Assay/methods , Female , Follow-Up Studies , Guillain-Barre Syndrome/drug therapy , Guillain-Barre Syndrome/physiopathology , Humans , Immunoglobulins, Intravenous/therapeutic use , Male , Middle Aged , Neural Conduction/physiology , Prognosis , Retrospective StudiesABSTRACT
A patient with primary B cell non-Hodgkin's lymphoma of the sciatic nerve is described. He presented with neuropathic symptoms in the left leg, initially diagnosed as tarsal tunnel syndrome. Magnetic resonance imaging (MRI) identified the abnormality in the sciatic nerve. A fascicular biopsy of the sciatic nerve showed a diffuse large B cell non-Hodgkin's lymphoma. The patient was treated with chemotherapy and rituximab (anti-CD20 monoclonal antibody). Four months later he was in remission, and remains so 48 months from presentation. Primary lymphoma of single peripheral nerves may be a unique subtype of extranodal lymphoma, which usually follows an aggressive course and has a variable response to current therapeutic strategies. MRI is useful, alongside electrophysiological studies, in patients with atypical peripheral nerve symptoms.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Lymphoma, B-Cell/drug therapy , Lymphoma, B-Cell/pathology , Peripheral Nervous System Neoplasms/drug therapy , Peripheral Nervous System Neoplasms/pathology , Sciatic Nerve/pathology , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal, Murine-Derived , Humans , Male , Middle Aged , Rituximab , Treatment OutcomeABSTRACT
CNS demyelinating lesions have been reported in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). There are no studies of cord atrophy in CIDP. Ten patients with CIDP underwent brain and spinal cord MRI to investigate CNS demyelination and cord atrophy. No CNS demyelination was found, but the mean cervical cord area was significantly smaller in CIDP patients vs control subjects. Spinal cord atrophy may be related to degeneration secondary to axonal loss.
