ABSTRACT
OBJECTIVE: To describe temporal profile of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients with definite, relapsing multiple sclerosis (MS). BACKGROUND: Peripheral demyelinating neuropathy has been rarely reported in association with central nervous system demyelinating disorder indistinguishable from MS. METHODS: In addition to usual diagnostic studies for CIDP and MS in all 5 patients, we studied proximal segments of nerves using deep tendon reflex latency measurements of biceps reflex, patellar reflex, and ankle reflex. RESULTS: All patients with MS subsequently (4-22 years) developed definite CIDP. Two of these patients developed multiple cranial nerve and spinal root enhancement on subsequent imaging without new intraparenchymal enhancement after a diagnosis of CIDP. The deep tendon reflex latencies were prolonged at more than 2 sites in all patients. Cerebral spinal fluid protein increased (70 +/- 19 to 144.8 +/- 17.4 mg/dL, P = 0.0001) at time of diagnosis of CIDP. Clinical improvement was observed in all patients after intravenous immunoglobulin therapy. CONCLUSIONS: When patients with MS develop CIDP, manifestations of central and peripheral disease involvement seem to respond to intravenous immunoglobulin. These cases suggest that there may be common antigenic targets in central and peripheral nervous system in this subset of patients.
Subject(s)
Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Multiple Sclerosis, Relapsing-Remitting/complications , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Adult , Aged , Disability Evaluation , Electromyography , Female , Humans , Magnetic Resonance Imaging , Male , Multiple Sclerosis, Relapsing-Remitting/therapy , Neural Conduction/immunology , Peripheral Nervous System/physiopathology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/pathology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/therapy , Reflex, Stretch , Severity of Illness Index , Spinal Nerve Roots/physiopathology , Statistics, Nonparametric , Time Factors , Treatment OutcomeABSTRACT
Small-fiber neuropathy (SFN) is diagnosed on the basis of clinical features and specialized tests of small-fiber function because standard nerve conduction studies are normal. Thus, the objective of this study was to determine the value of deep tendon reflex (DTR) latency measurement in the diagnosis of SFN in patients with preserved DTR on clinical examination. We prospectively examined electromyographic reflexes from the biceps brachii [biceps brachii reflex (BR)], patellar [patellar reflex (PR)], and ankle [ankle reflex (AR)] using a manually operated electronic reflex hammer attached to electromyography machine and recorded by means of surface electrodes in 18 patients with SFN and 38 controls. Intra- and inter-evaluator reliability was good (intraclass correlation coefficient: 0.80-0.91, p < 0.01). In controls, the latencies at all sites were correlated to the height (R= 0.6, p < 0.01). Compared with controls, in patients with SFN, the mean latency in milliseconds was prolonged at all sites (BR: 12.8 +/- 1.6 vs. 8.9 +/- 1.9, p < 0.01; PR: 23.0 +/- 5.8 vs. 17.4 +/- 2.4, p < 0.01; and AR: 34.5 +/- 4.8 vs. 30.0 +/- 2.4, p < 0.01). The sensitivity [61.1% (95% CI: 51-94.9)] and specificity [92% (95% CI: 73-97.3)] of BR latency were roughly equal to those of PR and AR. We conclude that DTR latencies were significantly abnormal in the majority of the patients with SFN, suggestive of subclinical involvement of large myelinated fibers. DTR latency measurement is a reproducible, valuable, sensitive tool in the evaluation of mild subclinical involvement of large fibers.
Subject(s)
Nerve Fibers/physiology , Neurologic Examination/methods , Peripheral Nervous System Diseases/diagnosis , Reflex, Stretch/physiology , Aged , Ankle/innervation , Ankle/physiology , Autonomic Nervous System/physiopathology , Brachial Plexus/physiology , Electromyography , Female , H-Reflex/physiology , Humans , Knee/innervation , Knee/physiology , Male , Middle Aged , Neural Conduction/physiology , Observer Variation , Pain/diagnosis , Pain/etiology , Peripheral Nervous System Diseases/classification , Prospective Studies , Reproducibility of Results , Sensation Disorders/diagnosis , Sensation Disorders/etiologySubject(s)
Parkinson Disease/pathology , Parkinson Disease/physiopathology , Parkinsonian Disorders/pathology , Parkinsonian Disorders/physiopathology , Brain/pathology , Brain/physiopathology , Diagnosis, Differential , Humans , Lewy Body Disease/pathology , Lewy Body Disease/physiopathology , Multiple System Atrophy/pathology , Multiple System Atrophy/physiopathology , Neural Pathways/pathology , Neural Pathways/physiopathology , Neurons/pathology , Supranuclear Palsy, Progressive/pathology , Supranuclear Palsy, Progressive/physiopathologyABSTRACT
O presente estudo relata os resultados da primeira aplicaçäo no Brasil, e em português, de uma escala analógica desenvolvida no Centro de Memória da Universidade da Califórnia para avaliar a metamemória, ou seja, a persepçäo e julgamento dos indivìduos quanto ao funcionamento de sua própria memória. Com o objetivo de analizar a influência da idade e do nìvel de instruçäo no desempenho na escala, o instrumento foi aplicado em uma amostra de 90 pacientes internados nas unidades de clìnica médica do Hospital de Clìnicas de Porto Alegre, que näo apresentavam patologias ou fatores de risco para comprometimento das funçöes da memória. A análise das 18 questöes da escala demonstrou a influência da idade em cinco delas, enquanto que o nìvel de instruçäo apresentou efeito em apenas uma questäo. O escore global para escala foi também estudado
Subject(s)
Humans , Male , Female , Adolescent , Middle Aged , Age Factors , Memory , Self-Assessment , Cross-Cultural Comparison , Hospitals, GeneralABSTRACT
O presente estudo relata os resultados da primeira aplicacao no Brasil, e em portugues, de uma escala analogica de senvolvida no Centro de Memoria da Universidade da Clifornia para avaliar a metamemoria, ou seja, a percepcao e julgamento dos individuos quanto ao funcionamento de sua propria memoria. Com o objetivo de analisar a influencia da idade e do nivel de instrucao no desempenho na escala, o instrumento foi aplicado em uma amostra de 90 pacientes internados nas unidades de clinica medica do Hospital de Clinicas de Porto Alegre, que nao apresentavam patologias ou fatores de risco para comprometimento das funcoes da memoria. A analise das 18 questoes da escala demonstrou a influencia da idade em cinco delas, enquanto que o nivel de instrucao apresentou efeito em apenas uma questao. O escore global para escala foi tambem estudado.
