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Biomedical subjects

L Guilloton

Publications and source records attributed to L Guilloton.

At least 19 recordsLinked to original sources
PubMed2006

[Polyneuropathy involving cranial nerves associated with monoclonal IgM antibodies with anti-MAG/SGPG/SGPLG/sulfatides activity].

INTRODUCTION: A typically distal and symmetrical, slowly progressive sensorimotor demyelinating neuropathy is caused by monoclonal IgM against myelin-associated glycoprotein (MAG) and SGPG, SGLPG glycolipids in the context of a benign IgM paraproteinemia. We studied a patient with a neuropathy that fulfilled the diagnostic criteria for CIDP in whom IgM kappa anti-MAG/SGPG/SGLPG were detected. OBSERVATION: The patient was a 57-year-old man who had developed a slowly progressive distal sensorimotor neuropathy, involving the lower then upper limbs, with cranial nerves palsies (oro-pharyngo-laryngo territory). ENMG showed a demyelinating neuropathy with a disproportionate slowing of conduction in distal segments of motor and axonal features in the lower limbs. The first routine laboratory analysis revealed negative or normal findings. Several serum protein electrophoreses were normal. The third cerebrospinal fluid examination demonstrated a moderate and late rise in CSF protein level with no cells. Monoclonal IgM-kappa against MAG/SGPG/SGLPG, was detected; anti-MAG antibody titre in the serum was 20 059 BTU (N<1000). A small IgM-kappa paraprotein was identified by immunofixation. Electron microscopy failed to show nerve fibers with widening of outer lamellae of the myelin. There is no clinical improvement after different treatments, immunoglobulins IV, cortisteroids, plasma exchange, rituximab. CONCLUSION: It is not known whether this neuropathy is an atypical form of PNMAG or an CIDP associated with anti-MAG. When ENMG show a disproportionate slowing of conduction in distal segments of motor nerves, one should screen the serum with immunofixation to identify small monoclonal components. If IgM-MGUS is present, search should be undertaken for anti-MAG/SGPG/SGLPG antibodies. Diagnosis enables optimal treatment using, in severe cases, expensive current strategies with immunoglobulins IV, plasma exchange, and corticosteroids, or, in the event of no response, rituximab before resorting to more toxic drugs like cyclophosphamide.

Antibodies, Anti-Idiotypic↗
PubMed2004

[Paraneoplasic pandysautonomia with anti-Hu antibodies: a presentation of pulmonary adenocarcinoma identified by PET scanning].

A 74-year-old man developed pandysautonomia with severe orthostatic hypotension. Search for a paraneoplastic etiology was confirmed with the positivity of anti-Hu antibodies. [18] Fluorodeoxyglucose positron emission tomography (PET) enabled recognition of a lung adenocarcinoma. We emphasize the usefulness of PET scans for early diagnosis of cancer.

Adenocarcinoma↗
PubMed2004

[Split-cord malformation (diastematolmyelia) presenting in two adults: case report and a review of the literature].

INTRODUCTION: Split cord malformation (SCM) is an uncommon developmental anomaly characterized a cleft spinal cord. In type I, each of the hemicords is contained within an individual dural tube whereas in type II there is a common dural tube housing both hemicords. Commonly diagnosed in childhood, adult presentation is exceptional. METHODS: We report the case of two women whose type II SCM was discovered at the age of 40 and 54 years. RESULTS: The first patient complained of chronic lombar and radicular chronic pain with dysuria. Physical examination revealed a radicular syndrome with abolition of the left Achille reflex and a lombar hair tuft. MRI showed a disc herniation at the L5-S1 level, with a partial SCM at the level of the L2 vertebra, spina bifida and tethered cord. The second patient complained of lombar pain with perineal irradiation for 6 years. Physical examination showed a lombar cutaneous angioma. MRI revealed a thoraco-lombar SCM at the T12 to L1 level, with spina bifida. No spur could not be identified in either patient. No further surgical treatment was given. CONCLUSION: Based on these two observations, we propose a review of literature reporting 90 cases of adult SCM.

Adult↗
PubMed2003

[Acute severe leukoencephalitis with posterior lesions due to Borrelia burgdorferi infection].

INTRODUCTION: Central nervous system manifestations represent 0.54 to 8% of neurological complication in Lyme disease. OBSERVATION: A 78-year-old woman presented a severe meningo-encephalitis with visual disorders (agnosia, alexia) progressing towards coma. Cranial magnetic resonance imaging revealed large areas of hypersignal T2 in the white matter of the lower, parieto-occipital lobes and left temporal lobe. The cerebrospinal fluid (CSF) contained 16 then 293 white corpuscles/mm3 of lympho-monocytes, increased protein level from 2.67 to 5.83 g/l and an increase in IgG index with oligoclonal distribution of IgG. Serological Elisa analysis for Lyme disease was slightly positive in blood (confirmed by western blot) but clearly in the CSF (IgG and IgM). Treatment with ceftriaxone followed by methylprednisolone provided clinical improvement 3 months later. DISCUSSION: Acute meningo-encephalitis is often benign, protein-like and of good prognosis: the gnosic visual disorders with posterior leukoencephalopathy are unusual. A blood level of specific antibodies slightly positive on Elisa at the early stage of the infection warrants confirmation by Western blot in the blood and by Elisa in the CSF. Additional corticosteroid therapy may be required in the severe forms that evoke acute disseminated encephalomyelitis.

Acyclovir↗
PubMed2003

[Usher syndrome: a case report].

A 60-year-old Jewish woman with consangineous parents had a history of severe sensorineural hearing loss since the age of 2 years. Hearing loss had not progressed since childhood, but her visual impairment due to pigmentary retinopathy, known since childhood, had worsened 15 years ago. The diagnosis was Usher syndrome type I, a rare heterogeneous disorder of autosomal recessive inheritance. Abnormal vestibular function and ataxia with neuroimaging anomalies including cerebellar atrophy have been reported, suggesting the disease process also involves the brain.

Brain↗
PubMed2003

[Calcified brain metastases of a pulmonary neuroendocine carcinoma].

We describe an unusual case of pulmonary neuroendocrine carcinoma of intermediate cell type, revealed by calcified metastatic nodules of the brain in a 49-year-old man. Histological examination of the brain lesions disclosed necrosis and calcification in the center with small and medium-sized carcinoma cells. Pathophysiological mechanisms of such calcifications in metastasis lesions are reviewed.

Brain Diseases↗
PubMed2003

[Hashimoto's thyroiditis associated with recurrent transverse myelopathy].

A 55-year-old woman presented first episode of paraplegia at D6 level with a marked improvement of neurological signs, but relapse occurred five months later. Hashimoto's thyroiditis with euthyroidism was found. Long-term 9 months treatment with oral corticosteroids was applied. Also she was able to walk with help. Two years after the second palsy she developed a new paraplegia at D6 level with a severe residual deficit five months later in spite of oral corticosteroids. Thyroid antibodies remained positive. Devic's disease without optica neuritis associated with thyroiditis or spinal cord localisation of Hashimoto's encephalopathy was discussed.

Atrophy↗
PubMed2002

[Muscular exercise intolerance syndrome in Becker muscular dystrophy].

OBJECTIVE: To determine whether exertional muscle pain syndrome (EMP) is a benign phenotype or an early stage of Becker-type muscular dystrophy (BMD). METHODS: Muscle dystrophin analysis led to the diagnosis of BMD in 6 patients complaining of EMP. RESULTS: Three patients had a history of X-linked inheritance and age at clinical onset was 4 to 11 years in five, and one patient had a later onset aged 23. Pseudohypertrophy of the calf muscles was absent in one patient, but all had experienced mild (5/6) pelvic weakness and (or) atrophy one to 17 years after the onset. High serum CK level was present (X 14). Normal anti-dystrophin immunostaining in two cases did not rule out the diagnosis that was only made made by Western blot analysis or genetic studies. All exhibited in-frame deletions (exons 45-48) within the dystrophin gene. CONCLUSION: The 36 patients with BMD-EMP analysed in the literature, exhibited different deletions and no worsening in 66.7% of cases. Western blot was more precise than immunolabelling with 96.8% positivity versus 70.5%. Dystrophin analysis by Western blot and (or) DNA analysis should be included in the evaluation of patients with EMP syndrome without deficient muscle energy metabolism, particularly those with pseudohypertrophy of the calf muscles or high serum CK levels.

Adolescent↗
PubMed2002

[Diagnostic value of classical neurophysiologic profile in various phenotypes of hereditary, pressure-sensitive neuropathies].

PURPOSE: We looked for electrodiagnostic features that raise suspicion of hereditary neuropathy with liability to pressure palsies (HNPP). METHOD: A retrospective review of eight cases with confirmed histologic (one case) or chromosome 17 deletion (seven cases) analysis was performed. RESULTS: Autosomal dominant disease was present in 63% of the patients, 75% being men. Mean age at examination and at first symptom was 27 and 22 years respectively. Five patients have one or more acute nerve palsies, without residual deficit, but one presented sensory symptoms in internal saphena territory. Three patients had others phenotypes: a man presented with four episodes of facial palsy and one woman was asymptomatic; another patient with diabetes mellitus presented an associated chronic sensorimotor polyneuropathy. In agreement with other studies of HNPP we found in most patients a diffuse increase in distal motor latence, contrasting with normal or moderately decreased motor nerve conduction velocity, multiple electrophysiologic entrapment and diffuse reduction in sensory nerve action potential and/or velocity. However, this electrophysiological pattern was incomplete or masked in HNPP with cranial nerves palsies, in asymptomatic form of HNPP or in HNPP associated with polyneuropathy. CONCLUSION: These data confirm the clinical phenotypic heterogeneity of the 17p11.2 deletion and highlight the limits of the classic electrophysiological pattern in asymptomatic HNPP or associated with uncommon clinical features.

Adolescent↗
PubMed2002

[Neuritic leprosy disclosed by reversal reaction].

We report a case of neuritic borderline tuberculoid leprosy with lingering insidious growth disclosed by a brutal reversal reaction. Inflammatory polyarthralgia and sensory and motor loss in the median and ulnar territories, without skin lesions, suggested vasculitis. A few weeks later, inflammatory skin lesions developed leading to the diagnosis of biopsy proven leprosy. Leprosy should be considered as a possible diagnosis in patients with multineuritis, especially when associated with rheumatic or cutaneous manifestations. Early skin biopsy in neuritic leprosy is discussed.

Adult↗
PubMed2001

[Recurrent rhabdomyolysis revealing chronic exertional compartment syndrome of the biceps].

UNLABELLED: OBJECTIVE AND PATIENT: To report, an uncommon case of recurrent effort-related biceps compartment syndrome induced by strengthening exercises (body building) in a thirty-years-old white man. DISCUSSION-CONCLUSION: compartment syndrome is typically observed in the lower leg or in the forearm with pain, muscle tightness, cramp-like feeling during exertion. The involvement of the biceps was only described after traumatisms. Patient was initially thought to have metabolic myopathy because acute episodes of exercise induced myalgia and elevated serum CK. Tissue pressure monitoring prior and after a standard exercise test is helpful for diagnosing the recurrent form. The only alternative to fasciotomy is to stop sports activities.

Adult↗
PubMed2001

[Anterior compartment syndrome of the forearm caused by exercise: unusual cause of recurrent episodes of acute effort rhabdomyolysis].

INTRODUCTION: An effort-related compartmental syndrome is well known in the leg, but may be present infrequently, acutely or chronically in the anterior compartment of the forearm. EXEGESIS: We report a case of a 32-year-old man who presented four times after climbing exercises a bilateral compartment of the forearm, unusual because of the observation of rhabdomyolysis, but without irreversible damage. Clinical information and follow-up on two acute and 14 chronic cases were reexamined, showing a homogenous presentation. He refused fasciotomy because he stopped athletic activities. Measurement of intramuscular pressure after exertion was useful for diagnosis. CONCLUSION: A local effort-related pain must call to mind a chronic compartment syndrome of the forearm, which may risk incurring the acute form, with irreversible lesions of muscle and nerve, and possibly renal failure because of rhabdomyolysis.

Acute Disease↗
PubMed2001

[Transformation of mycosis fungoides to pleomorphic T-cell lymphoma and central nervous system involvement].

INTRODUCTION: Although mycosis fungoides is a malignant T-cell lymphoma involving mainly the skin, neurological complications are possible, with a poor prognosis. EXEGESIS: A 59-year-old man, treated for mycosis fungoides with transformation to a pleomorphic T-cell lymphoma for 1 year, was seen for mental status changes with confusion. A brain parenchyma localisation was found. CONCLUSION: This observation emphasizes the exceptional neurological tropism in the patients with mycosis fungoides. A transformation to a more aggressive cutaneous T-cell lymphoma seems necessary to induce a central nervous system involvement.

Brain Neoplasms↗