Usefulness of somatosensory evoked potentials for monitoring the clinical course of patients with chronic inflammatory demyelinating polyradiculoneuropathy.

INTRODUCTION/AIMS: Somatosensory evoked potentials (SSEPs) are described as a supportive tool to diagnose chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, there is a lack of studies determining the effectiveness of SSEPs in monitoring the clinical course of individuals with this condition. The aims of this study are to evaluate the utility of SSEPs in monitoring patients with CIDP and to assess their association with clinical outcomes following immunomodulatory therapy. METHODS: This was a single-center retrospective observational study that included patients who met European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP between 2018 and 2023. SSEPs were performed at diagnosis and during follow-up after the start of immunomodulatory treatment. Fisher's exact test was employed to assess the association between clinical improvement and SSEP improvement. RESULTS: Eighteen patients were included in the study. Ten patients had a typical CIDP pattern and 11 were male. In 17, SSEPs were abnormal prior to the start of immunomodulatory treatment. In patients who showed clinical improvement with immunomodulatory therapy, we observed that 15/17 had partial or complete improvement in SSEPs. Patients who showed no clinical improvement with first-line treatment exhibited worsening SSEPs. There was a significant association between clinical and SSEPs improvement (p = 0.009). DISCUSSION: We observed a positive association between improvement in SSEPs and clinical improvement in patients with CIDP. Our data suggest that SSEPs may be useful for monitoring the clinical course of patients with CIDP, but additional, larger studies are needed.

Transcranial magnetic stimulation as a prognostic tool in stroke.

Our aims were to evaluate the prognostic usefulness of magnetic motor evoked potentials (MMEPs) in ischemic stroke, to study the evolution of MMEP abnormalities and the relationships between MMEP abnormalities and infarction topography. We prospectively analyzed 50 consecutive ischemic stroke patients who were followed up to 1 year. MMEPs were recorded 1, 3, 30 and 90 days after stroke and we measured amplitudes and latencies/central motor conduction times (CMCTs) of MMEPs from hypothenar, biceps brachiallis, gastrocnemius and quadriceps. Univariate and multivariate analyses of the clinical and MMEPs data were performed. Patients with Rankin 0-3 at 1 year had had acutely MMEPs with shorter latencies and higher amplitudes than patients with Rankin 4-5 or deceased patients. Increased blood pressure correlated with increased survival, whereas increased heart rate and hyperglycemia correlated with increased mortality. The variables infarction size on second CT, age, and first day CMCT-S1 correctly classified 1 year outcome on discriminant analysis. The inclusion of MMEPs values increased the probability of correct classification from 76% to 84%. We conclude that in patients with nondisabling strokes MMEPs may have an independent value in the prediction of prognosis, increasing the accuracy of prognosis calculations made employing clinical and laboratory data. Topography of lesions should be considered when analyzing MMEP abnormalities after stroke.

Reflex epilepsy.

We report 3 cases of reflex epilepsy, 2 of them triggered by reading (reading epilepsy) and one by mathematical calculation (epilepsy arithmetics). Electroencephalographic abnormalities predominated in the dominant hemisphere. The pathophysiologic mechanisms involved in this rare form of epilepsy are commented on, particularly the role played by the association of mental concentration and emotional strain. A possible deficiency in GABA-ergic inhibitory synapses would cause a deficit in cortical inhibition. Different drugs have been used successfully in patients with reflex epilepsy. Psychotherapy relieves anxiety and has been shown to be an effective complement to medical treatment.

[Hand arthropathy of professional origin: milkingman hand]. / Artropatía de las manos de origen profesional: mano del ordeñador.

OBJECTIVE: The aim of this case-control study was to know possible correlates between distinctive pattern of hand arthrosis and length manual milking. PATIENTS AND METHODS: Between January 1990 and January 1996, we retrospectively reviewed the patients with symptomatic arthrosis in whose hands was identified this pattern: degenerative disease of distal interphalangeal joints with radial deviation and flexion of distal phalanges in association to arthrosis involving metacarpophalangeal and interphalangeal joints of the thumb. The first 88 cases of hand arthrosis without this clinical and radiographic pattern make the control group. Patients suffering inflammatory rheumatic diseases were excluded. RESULTS: 35 patients with this special pattern of hand arthrosis were recruited. The majority of patients were women (94.3%) and coming from rural environment (94.3%). 32 patients had performed daily manual milking over a period of 20 years, but in three patients the pattern was not related to this activity (odds ratio = 928: p < 0.001). Symptoms were more frequent in the control-group (57% versus 26%; p < 0.04), in which no characteristic pattern of alignment abnormality was found. CONCLUSION: A typical pattern of hand arthrosis was associated with prolonged manual milking: degenerative disease of metacarpophalangeal and interphalangeal joints of thumb, radial deviation associated with flexion of distal phalanges of second at fourth fingers and arthrosis involving their distal interphalangeal joints, often linked to sight ulnar deviation of middle phalanges.

[Chronic demyelinating auto-immune acquired neuropathy: Lewis-Sumner syndrome]. / Neuropatías adquiridas autoinmunes desmielinizantes crónicas: síndrome de Lewis-Sumner.

INTRODUCTION: Knowledge of the chronic auto-immune acquired neuropathies has progressed rapidly since the recent description of the Lewis-Sumner syndrome and multifocal motor neuropathy. Some of the most interesting aspects of these conditions are the presence of persistent conduction blocks and the challenge of understanding the mechanisms involved. These blocks are difficult to identify. CLINICAL CASE: We describe the case of a female patient with Lewis-Sumner syndrome, with persistent sensory and motor blocks. The electrophysiological characteristics and differential diagnosis of the two conditions are reviewed.

[Van Buchem disease. Study of 2 cases and review of the literature]. / Enfermedad de Van Buchem. Estudio de dos casos y revisión de la literatura.

We report two members of one family, a 51-year-old man a 16-year-old son, with enlargement of the jaw, palatine taurus, endosteal sclerosis of the neurocranium and symmetrical diaphyseal cortical thickening. On the basis of those typical findings, the diagnosis of recessive endosteal hyperostosis (Van Buchem's disease) was mode. This unusual hereditary sclerosing bone dysplasia is discussed with respect to the clinical and radiological features as well as its distinction from other sclerosing disorders.

[Pseudotumor in the forearm caused by focal myositis. A rare form of idiopathic inflammatory myopathy]. / Pseudotumor en antebrazo causado por miositis focal. Una forma inusual de miopatía inflamatoria idiopática.

Focal myositis is a rare form of idiopathic inflammatory myopathy. We describe an additional case of focal myositis in a 54-years-old woman presenting as a painful pseudotumor of the left forearm. Muscle enzymes were normal but generalized electromyographic abnormalities were found. We consider that focal myositis is a heterogeneous clinical syndrome, and the cases showing only focal involvement can be different from those that evolve into polymyositis.

[Septic arthritis caused by Salmonella enteritidis in systemic lupus erythematosus]. / Artritis séptica por Salmonella enteritidis en el lupus eritematoso sistémico.

Systemic Lupus Erythematosus (SLE) is among the chronic diseases thought to predispose patients to severe Salmonella infections. However, arthritis and osteomyelitis due to this microorganisms are more frequently seen in patients with sickle-cell disease than SLE. We report two cases of SLE and osteoarticular infections by Salmonella enteritidis: A 36-years old woman with bilateral knee arthritis associated with femoral osteomyelitis and a 22-years-old woman who presented with left knee arthritis.

[Delay in the diagnosis of McArdle's disease]. / Retraso en el diagnóstico de la enfermedad de McArdle.

We describe the case of a 14-year-old patient with fatigability and muscular cramps beginning in the first infancy and started by physical exercise. The clinical record, the increase of creatinine-kinase without clinical or biological signs of inflammation and the electromyographic normality, suggested the presence of this disease, which was confirmed through microscopic studies (optic and electronic) and histochemistry. We review the clinical and biological characteristics, the diagnostic methods for this type of glycogenosis and its differential diagnosis, because we think that the low suspicion levels among the clinicians contribute to the few cases published in the literature.

[Polyarthritis and hepatitis as presentation forms of secondary syphilis]. / Poliartritis y hepatitis como presentación de la sífilis secundaria.

We present a luetic secondarism in a 40-year-old woman starting as symmetric polyarthritis and hepatitis. The latter presence of cutaneous rash, oral aphtae and anemia, as well as the positivity of anticore antibodies (1/160 core pattern) and anti-cardiolipin antibodies initially suggested a connectivopathy. The diagnosis of secondary syphilis was confirmed by the luetic seropositive results (RPR and FTAabs) and the microscopic observation of mobile microorganisms compatible with Treponemas in the exudate obtained by scraping of the cutaneous lesions. The proteiform expressivity of luetic secondarism is stressed, as well as its rare presentation with articular inflammatory manifestations.

[The coexistence of rheumatoid arthritis, ankylosing spondylitis and intestinal necrotizing vasculitis]. / Coexistencia de artritis reumatoide, espondilitis anquilosante y vasculitis necrotizante intestinal.

We present the case of a 44-year-old male with ankylosing spondylitis and seropositive rheumatoid arthritis whose evolution was complicated by an intestinal necrotizing vasculitis type nodose panarteritis with fatal consequences. We discuss this exceptional association and its clinicopathological characteristics.

[Refractory idiopathic liquefying panniculitis]. / Paniculitis lobular licuefaciente idiopática refractaria.

We present a 28-year-old patient with liquefying idiopathic lobular panniculitis, a variant of the Weber-Christian's disease and intermittent episodes of arthritis in the left foot refractory to sequential treatment with anti-inflammatory drugs, high doses of prednisone, chloroquine, dapsone, colchicine, cyclosporine and methotrexate.

[Disseminated extrapulmonary tuberculosis with skin, lymph node and bone involvement]. / Tuberculosis extrapulmonar diseminada con afección cutánea, ganglionar y ósea.

Disseminated extrapulmonary tuberculosis is uncommon in no immunocompromised hosts. We described the case of a 68-year-old HIV seronegative man, who presented with a 5 months history of constitutional symptoms, generalized lymphadenopathy, evening fever, osteomyelitis of the left fibula and cutaneous lesions (papules and pustules). There was neither clinical nor radiological evidence of pulmonary involvement. On the basis of bacteriological and pathological findings the diagnosis of disseminated extrapulmonary tuberculous was made.

[Asymmetric oligoarthritis in Castleman's disease]. / Oligoartritis asimétrica en la enfermedad de Castleman.

The angiofollicular lymph node hyperplasia (Castleman's disease) is an entity of unknown etiology. Its diagnosis is based on clinical and histological criteria. We present the case of a 20-year-old male patient with asymmetric oligoarthritis and fever which response to nonsteroidal antiinflammatory drugs as onset of abdominal Castleman's disease mixed type.

[Polymyalgia syndrome and atrial myxoma]. / Síndrome polimiálgico y mixoma auricular.

We present a case of polymyalgia-like syndrome in a 62-years-old woman with four month history of severe headache, muscular claudication, asthenia, normochromic, normocytic anaemia and elevation of erytrocyte sedimentation rate. A diagnosis of giant left atrial myxoma was made brought about by thoracoabdominal magnetic resonance. Their surgical exeresis was followed by gradual disappearance of symptoms and normalization of laboratory parameters.