david adams's Publications List

Ocular involvement in familial amyloid polyneuropathy

2013-12-08T09:05:34Z

Familial amyloid polyneuropathy (FAP) or transthyretin (TTR) amyloid polyneuropathy is a progressive sensorimotor and autonomic neuropathy of adult onset, which is transmitted as an autosomal dominant trait. In addition to neurologic symptoms, FAP may be associated with weight loss, cardiac and renal failure and ocular complications. FAP is a devastating disease, causing death within 10years after...

Rousseau A, Kaswin G, Adams D, Cauquil C, Théaudin M, Mincheva Z, M'garrech M, Labetoulle M, Barreau E. (2013) J Fr Ophtalmol. 36: 9 779-88

Varicella-zoster virus acute myelitis in a patient with MS treated with natalizumab.

2013-05-09T21:27:44Z

We report a case of varicella-zoster virus (VZV) myelitis in a woman with relapsing-remitting multiple sclerosis (RRMS) receiving natalizumab, a humanized monoclonal antibody that induces an immunosuppression localized to the CNS.

Jennifer Yeung, Cécile Cauquil, Guillaume Saliou, Ghaida Nasser, Sophio Rostomashvili, David Adams, Marie Théaudin (2013) Neurology 80: 19 1812-1813

Immunological Hallmarks of JC Virus Replication in Multiple Sclerosis Patients on Long-Term Natalizumab Therapy.

2013-05-09T21:26:29Z

Progressive multifocal leukoencephalopathy (PML) is the main adverse effect of natalizumab. Detectable JC virus-specific effector memory T-cell (TEM) responses may indicate ongoing JCV replication. We detected JCV-specific TEM responses in blood of patients with multiple sclerosis (MS) treated with natalizumab, including 2 patients with PML. The frequency of detection of these responses increased ...

Houria Hendel-Chavez, Marie-Ghislaine de Goër de Herve, Claire Giannesini, Anne-Aurélie Mazet, Caroline Papeix, Céline Louapre, Audrey Chardain, Nassima Boutarfa, Marie Théaudin, David Adams, Jacques Gasnault, Bruno Stankoff, Yassine Taoufik (2013) J Virol 87: 10 6055-6059

Recent advances in the treatment of familial amyloid polyneuropathy.

2013-12-08T09:23:43Z

The treatment of familial amyloid polyneuropathy (FAP) requires a multidisciplinary approach, mainly neurological and cardiological. It includes specific treatments to stop the progression of systemic amyloidogenesis, the symptomatic treatment of the peripheral and autonomic neuropathy and the treatment of organs severely involved by amyloidosis (heart, eyes, kidneys). First-line specific treatmen...

David Adams (2013) Ther Adv Neurol Disord 6: 2 129-139

Effect on disability and safety of Tafamidis in late onset of Met30 Transthyretin Familial Amyloid Polyneuropathy.

2013-08-18T12:03:09Z

BACKGROUND AND PURPOSE: The aim of this study was to assess the effect of Tafamidis, which slows the progression of early stages of Met30 transthyretin (TTR) familial amyloidosis polyneuropathy (FAP) in more advanced cases. METHODS: The study was a prospective, non-randomized controlled trial carried out at the French national reference centre for FAP with follow-up at 1 year. Thirty-se...

Lozeron P, Théaudin M, Mincheva Z, Ducot B, Lacroix C, Adams D and French Network for FAP (CORNAMYL) (2013) Eur J Neurol. :

A correlation between fractional anisotropy variations and clinical recovery in spinal cord infarctions.

2013-05-09T21:28:51Z

To describe diffusion-weighted imaging and diffusion tensor imaging variations in spinal cord infarctions.

Marie Théaudin, Guillaume Saliou, Christian Denier, David Adams, Denis Ducreux (2013) J Neuroimaging 23: 2 256-258

Reversibility of acquired amyloid polyneuropathy after liver Retransplantation

2013-08-18T12:05:33Z

Domino liver transplantation (DLT) has become an accepted procedure designed to address problems with organ limited supply. However, cases of acquired amyloid neuropathy are increasingly being recognized following this procedure. Until now, only one patient had undergone liver retransplantation and follow-up findings were not reported. We describe the case of a 72-year-old patient with partial rec...

Antonini, TM, Lozeron, P; Lacroix, C, Mincheva, Z , Durrbach, A , Slama, M, Vibert, E, Samuel, D, Adams, D (2013) Am J Transplant :

Safety and efficacy of an RNAi Therapeutic for TTR Amyloidosis.

2013-10-22T21:03:33Z

Coelho T, Adams D, Silva A, et al. ( 2013) N Engl J Med 369: 819-829.

[Review of the recent literature on peripheral neuropathies: Therapeutic advances.]

2013-12-08T09:00:16Z

Improvement of therapeutic strategies for peripheral neuropathies requires multicentric clinical trials. For chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a randomized controlled multicentric study compared IgIV to pulses of methylprednisolone (MP) given for 6months. The primary endpoint was treatment discontinuation due to inefficacy or intolerance; 45 patients were enrolled: ...

Adams D. (2013) Rev Neurol 169: 1004-1009

An amyotrophic lateral sclerosis-like syndrome revealing an amyloid polyneuropathy associated with a novel transthyretin mutation.

2013-08-18T12:08:00Z

Objective: Familial amyloid polyneuropathy (FAP) is typically a predominantly sensory and autonomic neuropathy with progressive and late motor involvement leading to death within 10 years. Recently, prognosis was transformed with liver transplantation. Methods: We report an atypical sporadic pure motor and bulbar neuropathy initially mistaken for amyotrophic lateral sclerosis (ALS) in a 50-year-ol...

Lozeron P, Lacroix C, Theaudin M, Richer A, Gugenheim M, Adams D, Misrahi M. (2013) Amyloid.. :

Prophylactic pacemaker implantation in familial amyloid polyneuropathy.

2012-05-05T11:56:11Z

BACKGROUND: Familial amyloid polyneuropathy (FAP) is an autosomic dominant disease with a high rate of conduction disorders and increased risk of sudden death. Prophylactic cardiac pacing may be considered in asymptomatic patients with FAP. However, the potential benefits are unknown. OBJECTIVE: To document conduction disorders in a large series of FAP and the incidence of high-degree atrioventric...

Vincent Algalarrondo, Sylvie Dinanian, Christophe Juin, Denis Chemla, Soumiya L Bennani, Claude Sebag, Violaine Planté, Dominique Le Guludec, Didier Samuel, David Adams, Michel S Slama (2012) Heart Rhythm :

Treatment of familial amyloid polyneuropathy.

2012-05-05T11:56:11Z

The treatment of familial amyloid polyneuropathies (FAP) is complex and requires a neurological and cardiological multidisciplinary coverage. It includes specific treatments to control the progression of the systemic amyloidogenesis, the symptomatic treatment of the peripheral and autonomic neuropathy (digestive, urinary, sexual, postural hypotension) and the treatment of organs severely involved ...

David Adams, Didier Samuel, Michel Slama (2012) Presse Med :

Axonal regeneration and neuroinflammation: roles for the translocator protein 18 kDa.

2012-05-05T11:59:30Z

After a traumatic injury of the nervous system or in the course of a neurodegenerative disease, the speed of axonal regeneration and the control of the inflammatory response are fundamental parameters of functional recovery. Spontaneous regeneration takes place in the peripheral nervous system, although the process is slow and often incomplete. There is currently no efficient treatment for enhanci...

C Girard, S Liu, D Adams, C Lacroix, M Sinéus, C Boucher, V Papadopoulos, R Rupprecht, M Schumacher, G Groyer (2012) J Neuroendocrinol 24: 1 71-81

Diffusion MRI and tensor tractography in ischemic optic neuropathy.

2012-05-05T11:57:38Z

Ischemic optic neuropathies (IONs) are among the most prevalent diseases causing visual impairment in middle-aged and elderly people. While arteritic ION is an ocular emergency and requires early diagnosis and immediate treatment with systemic high-dose corticosteroids to prevent further visual loss, treatment options for non-arteritic ION remain limited. We describe the case of a woman with unila...

C Cauquil, R Souillard-Scemama, M Labetoulle, D Adams, D Ducreux, C Denier (2012) Acta Neurol Belg :

Hereditary systemic amyloidosis due to Asp76Asn variant β2-microglobulin.

2012-12-23T22:00:04Z

We describe a kindred with slowly progressive gastrointestinal symptoms and autonomic neuropathy caused by autosomal dominant, hereditary systemic amyloidosis. The amyloid consists of Asp76Asn variant β(2)-microglobulin. Unlike patients with dialysis-related amyloidosis caused by sustained high plasma concentrations of wild-type β(2)-microglobulin, the affected members of this kindred had normal...

Sophie Valleix, Julian D Gillmore, Frank Bridoux, Palma P Mangione, Ahmet Dogan, Brigitte Nedelec, Mathieu Boimard, Guy Touchard, Jean-Michel Goujon, Corinne Lacombe, Pierre Lozeron, David Adams, Catherine Lacroix, Thierry Maisonobe, Violaine Planté-Bordeneuve, Julie A Vrana, Jason D Theis, Sofia Giorgetti, Riccardo Porcari, Stefano Ricagno, Martino Bolognesi, Monica Stoppini, Marc Delpech, Mark B Pepys, Philip N Hawkins, Vittorio Bellotti (2012) N Engl J Med 366: 24 2276-2283

[Autonomic peripheral neuropathy].

2012-12-23T22:00:03Z

The mechanisms of dysautonomic disturbances are varied and mostly acquired. They can result from lesions of sympathetic or parasympathetic vegetative fibers located in the peripheral contingent, or in the somatic contingent by demyelination or axonal loss; or more rarely by cellular bodies in the sympathetic or parasympathetic ganglia. Several chronic peripheral neuropathies can be associated with...

David Adams, Cecile Cauquil, Pierre Lozeron (2012) Presse Med 41: 11 1128-1136

Vasculitis neuropathy mimicking lower limb mono-radiculopathy: a study and follow-up of 8 cases.

2013-05-09T21:30:08Z

The aim of this study was to describe misleading lower limb mono radiculopathy revealing peripheral nerve vasculitis. Retrospective review of eight patients with biopsy confirmed vasculitis presenting as mono-radicululopathy in a tertiary referral centre dedicated to patients with rare peripheral neuropathies. Patients presented with chronic (6/8) or acute (n = 2) radiculopathy in L4, L5 or S1 t...

Pierre Lozeron, Catherine Lacroix, Mathilde Michon, Marie Theaudin, Marie-Christine Petit Lacour, Christian Denier, David Adams (2012) Intern Emerg Med :

Congenital vertebral duplication: a predisposing risk factor for dissection.

2012-05-05T11:57:38Z

Spontaneous cervical-artery dissection (CAD) is a major cause of cerebral ischaemia in young adults. While their pathophysiology remains still poorly understood, CAD is considered today as a multifactorial disease determined by general and local predisposing factors; these predisposing factors being predominantly constitutional abnormalities of the arterial wall. Here, we report an MRI-confirmed d...

Elsa Melki, Ghaidaa Nasser, Christophe Vandendries, David Adams, Denis Ducreux, Christian Denier (2012) J Neurol Sci 314: 1-2 161-162

Circadian rhythm of blood pressure reflects the severity of cardiac impairment in familial amyloid polyneuropathy.

2012-12-23T22:00:04Z

Cardiac amyloidosis due to familial amyloid polyneuropathy (FAP) includes restrictive cardiomyopathy, thickened cardiac walls, conduction disorders and cardiac denervation. Impaired blood pressure variability has been documented in FAP related to the Val30Met mutation.

Vincent Algalarrondo, Ludivine Eliahou, Isabelle Thierry, Abdeslam Bouzeman, Madalina Dasoveanu, Claude Sebag, Ghassan Moubarak, Dominique Le Guludec, Didier Samuel, David Adams, Sylvie Dinanian, Michel S Slama (2012) Arch Cardiovasc Dis 105: 5 281-290

Amyloid neuropathies.

2012-12-23T22:00:04Z

As amyloid neuropathies have benefited from recent major progress, this review is timely and relevant.

David Adams, Pierre Lozeron, Catherine Lacroix (2012) Curr Opin Neurol 25: 5 564-572

Regional difference and similarity of familial amyloidosis with polyneuropathy in France.

2012-12-23T22:00:04Z

Familial amyloidosis with polyneuropathy (FAP) in France have a large genetic heterogeneity with 29 transthyretin (TTR) gene mutations; Met30-TTR is the most frequent one (62%); followed by Tyr77-TTR (11.8%) and Phe77-TTR (6.2%). Analysis of 60 FAP patients diagnosed during the period 2008-2010 showed amyloid polyneuropathy was initially suspected in only 38% patients. TTR Met30 of Portuguese ance...

David Adams, Pierre Lozeron, Marie Theaudin, Zoia Mincheva, Cecile Cauquil, Clovis Adam, Aissatou Signate, Christophe Vial, Thierry Maisonobe, Emilien Delmont, Jerome Franques, Jean-Michel Vallat, Guilhem Sole, Yann Pereon, Arnaud Lacour, Andoni Echaniz-Laguna, Micheline Misrahi, Catherine Lacroix (2012) Amyloid 19 Suppl 1: 61-64

Symptomatic and proven de novo amyloid polyneuropathy in familial amyloid polyneuropathy domino liver recipients.

2012-05-05T11:56:11Z

D Adams, C Lacroix, T Antonini, P Lozeron, C Denier, A M Kreib, S Epelbaum, F Blandin, V Karam, D Azoulay, R Adam, D Castaing, D Samuel (2011) Amyloid 18 Suppl 1: 169-172

Varied patterns of inaugural light-chain (AL) amyloid polyneuropathy: a monocentric study of 24 patients.

2012-05-05T11:56:11Z

D Adams, P Lozeron, M Theaudin, C Denier, O Fagniez, K Rerat, A Signate, P Corcia, C Lacroix (2011) Amyloid 18 Suppl 1: 93-95

[New elements in the diagnosis and the treatment of primary AL amyloid polyneuropathy and neuropathy due to POEMS syndrome].

2012-05-05T11:56:11Z

Primary AL amyloid polyneuropathy (AL-PN) and neuropathy due to POEMS syndrome (POEMS-N) are rare, associated with a monoclonal gammopathy (MG) IgGλ or IgAλ at a low rate and systemic manifestations. They are invalidating and life-threatening.

D Adams, P Lozeron, M Theaudin, V Ribrag, J-H Bourhis, C Lacroix (2011) Rev Neurol (Paris) 167: 1 57-63

Hematological disorders related cerebral infarctions are mostly multifocal.

2012-05-05T11:57:38Z

Hematological disorders (HD) have been estimated to implicate approximately 1% of patients with arterial ischemic stroke. However, previously published studies are mostly retrospective or based on case reports or small series in selected young patients. We herein prospectively included consecutive patients with MRI-confirmed cerebral arterial infarctions among individuals admitted in our stroke un...

Ombeline Fagniez, Gérard Tertian, Marie Dreyfus, Denis Ducreux, David Adams, Christian Denier (2011) J Neurol Sci 304: 1-2 87-92

Short and long-term effect of IVIg in demyelinating neuropathy associated with MGUS, experience of a monocentric study.

2012-05-05T11:57:38Z

The optimal treatment for demyelinating neuropathy associated with MGUS and anti-MAG neuropathy is not known.

M Théaudin, P Lozeron, C Lacroix, P Chrétien, B Ducot, C Denier, D Adams (2011) Rev Neurol (Paris) 167: 12 897-904

Validation of a new language screening tool for patients with acute stroke: the Language Screening Test (LAST).

2012-05-05T11:57:38Z

Standard aphasia scales such as the Boston Diagnosis Aphasia Evaluation are inappropriate for use in acute stroke. Likewise, global stroke scales do not reliably detect aphasia, and existing brief aphasia screening scales suitable for patients with stroke have several limitations. The objective of this study was to generate and validate a bedside language screening tool, the Language Screening Tes...

Constance Flamand-Roze, Bruno Falissard, Emmanuel Roze, Lisa Maintigneux, Jonathan Beziz, Audrey Chacon, Claire Join-Lambert, David Adams, Christian Denier (2011) Stroke 42: 5 1224-1229

Advances in the treatment of chronic inflammatory demyelinating neuropathies in 2010.

2012-05-05T12:02:58Z

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon disease of the peripheral nerves. The diagnosis is based mainly on clinical presentation, cerebrospinal fluid (CSF) examination, and electrodiagnostic studies. Treatment of CIDP has at least two components: supportive care and specific therapy including primarily steroids, intravenous immunoglobulins (IVIg), and plasmapheresis...

P Lozeron, D Adams (2011) J Neurol 258: 9 1737-1741

Aphasia in border-zone infarcts has a specific initial pattern and good long-term prognosis.

2012-05-05T11:57:38Z

While border-zone infarcts (BZI) account for about 10% of strokes, studies on related aphasia are infrequent. The aim of this work was to redefine specifically their early clinical pattern and evolution.

C Flamand-Roze, C Cauquil-Michon, E Roze, R Souillard-Scemama, L Maintigneux, D Ducreux, D Adams, C Denier (2011) Eur J Neurol 18: 12 1397-1401

Brugada syndrome revealed by vertigo caused by cerebellar infarction.

2010-06-20T06:44:16Z

C Denier, T Guedj, S Dinanian, D Adams (2010) Eur J Neurol 17: 5 e35-e36

Liver transplantation for familial amyloid polyneuropathy

2010-06-20T06:33:58Z

Familial amyloid polyneuropathy (FAP) is the most serious of the hereditary neuropathies in adults and is due to endoneurial amyloid deposits. These sensorimotor and autonomic diseases are very progressive and disabling. A "typical" patient with FAP is 30-years-old, of Portuguese origin, and has insidiously developed pains or sensory loss in the feet and digestive disorders, such as diarrhea, and ...

David Adams, Michel Slama, Didier Samuel (2010) Presse Med 39: 1 17-25

Intracerebral hemorrhage caused by thrombosis of developmental venous anomaly: total recovery following anticoagulation.

2010-06-20T06:44:16Z

F Toulgoat, D Adams, G Nasser, D Ducreux, C Denier (2010) Eur Neurol 63: 4 254-255

Translocator protein (18 kDa) (TSPO) as a therapeutic target for neurological and psychiatric disorders.

2012-05-05T12:01:06Z

The translocator protein (18 kDa) (TSPO) is localized primarily in the outer mitochondrial membrane of steroid-synthesizing cells, including those in the central and peripheral nervous system. One of its main functions is the transport of the substrate cholesterol into mitochondria, a prerequisite for steroid synthesis. TSPO expression may constitute a biomarker of brain inflammation and reactive ...

Rainer Rupprecht, Vassilios Papadopoulos, Gerhard Rammes, Thomas C Baghai, Jinjiang Fan, Nagaraju Akula, Ghislaine Groyer, David Adams, Michael Schumacher (2010) Nat Rev Drug Discov 9: 12 971-988

Watershed infarctions are more prone than other cortical infarcts to cause early-onset seizures.

2012-05-05T12:02:06Z

Early-onset seizures(ESs) have been reported in 2% to 6% of strokes. Most previous studies have been retrospective and did not systematically perform cerebral magnetic resonance imaging (MRI).

Christian Denier, Pascal Masnou, Yacouba Mapoure, Raphaelle Souillard-Scemama, Thierry Guedj, Marie Théaudin, Ombeline Fagniez, Claire Join-Lambert, Pierre Lozeron, Béatrice Ducot, Denis Ducreux, David Adams (2010) Arch Neurol 67: 10 1219-1223

Multifocal deficits due to leukemic meningoradiculitis in chronic lymphocytic leukemia.

2010-06-20T06:44:16Z

Symptomatic nervous system leukemic infiltration is rarely observed in CLL. Various clinical manifestations including headache, confusion, cranial nerve palsies, focal central deficits and peripheral neuropathies have been seldom reported, occurring in less than 1% of patients. We report herein 2 CLL patients with unusual clinical presentations of nervous system invasion. They presented multiple p...

C Denier, G Tertian, V Ribrag, P Lozeron, C Bilhou-Nabera, T Lazure, K Abbed, C Lacroix, D Adams (2009) J Neurol Sci 277: 1-2 130-132

Long-term course of demyelinating neuropathies occurring during tumor necrosis factor-alpha-blocker therapy.

2010-06-20T06:44:16Z

OBJECTIVE: To report the long-term follow-up (mean, 41 months; range, 25-55 months) of patients with demyelinating neuropathy occurring after tumor necrosis factor-alpha (TNF-alpha) blocker treatment (infliximab [Remicade], etanercept [Enbrel], and adalimumab [Humira]). BACKGROUND: Demyelinating neuropathy is a rare adverse event of anti-TNF-alpha therapy. Improvement usually occurs after drug int...

Pierre Lozeron, Christian Denier, Catherine Lacroix, David Adams (2009) Arch Neurol 66: 4 490-497

[Metabolic neuropathies].

2012-05-05T12:02:58Z

Due to the high prevalence of diabetic neuropathies, metabolic neuropathies are common. "Diabetic neuropathy" is a slowly progressive painful sensory neuropathy evolving with a length dependent pattern. Slight distal weakness of toes extensor can be observed. Trophic changes and autonomic neuropathy can lead to severe complications (diabetic foot ulceration or symptomatic postural hypotension). Mu...

Pierre Lozeron, David Adams (2008) Rev Prat 58: 17 1903-1909

Late and fatal deterioration of an intracerebral hemorrhage attributable to the onset of a high titer of acquired factor V inhibitor.

2010-06-20T06:44:16Z

C Denier, T Bouteredjiret, M Dreyfus, C Lacroix, D Adams (2008) Eur Neurol 60: 4 206-207

A small deep infarct revealing leukoencephalopathy, calcifications and cysts in an adult patient.

2010-06-20T06:44:16Z

I Wargon, M-C Lacour, D Adams, C Denier (2008) J Neurol Neurosurg Psychiatry 79: 2 224-225

Monoclonal gammopathy and neuropathy.

2010-06-20T06:33:58Z

PURPOSE OF REVIEW: To provide clinically useful guidelines in the management of neuropathy associated with monoclonal gammopathy from a review of the most recent literature and our own experience. RECENT FINDINGS: Recent data on neuropathy associated with monoclonal gammopathy come from better descriptions of subgroups, and from new treatment compounds that have shown encouraging results in differ...

Pierre Lozeron, David Adams (2007) Curr Opin Neurol 20: 5 536-541

Domino liver transplantation from familial amyloidotic polyneuropathy donors: how close is the damocles sword to the recipient?

2010-06-20T06:33:58Z

Didier Samuel, David Adams (2007) Transpl Int 20: 11 921-923

A brief compound test for assessment of autonomic and sensory-motor dysfunction in familial amyloid polyneuropathy.

2010-06-20T06:44:16Z

BACKGROUND: Familial amyloid polyneuropathies (FAP) patients manifest progressive sensory-motor length dependent polyneuropathy and severe autonomic dysfunction. In this setting the autonomic manifestations include mainly postural hypotension, nausea and vomiting, diarrhea and constipation, sphincter distur- bances and erectile dysfunction. Reproducible quantitative evaluation of signs and symptom...

C Denier, B Ducot, H Husson, P Lozeron, D Adams, L Meyer, G Said, V Planté-Bordeneuve (2007) J Neurol 254: 12 1684-1688

Diagnostic pitfalls in sporadic transthyretin familial amyloid polyneuropathy (TTR-FAP).

2010-06-20T06:33:58Z

Transthyretin familial amyloid polyneuropathies (TTR-FAPs) are autosomal dominant neuropathies of fatal outcome within 10 years after inaugural symptoms. Late diagnosis in patients who present as nonfamilial cases delays adequate management and genetic counseling. Clinical data of the 90 patients who presented as nonfamilial cases of the 300 patients of our cohort of patients with TTR-FAP were rev...

V Planté-Bordeneuve, A Ferreira, T Lalu, C Zaros, C Lacroix, D Adams, G Said (2007) Neurology 69: 7 693-698

Spectrum and prognosis of neurologic complications after hematopoietic transplantation.

2010-06-20T06:44:16Z

OBJECTIVE: To describe the neurologic complications after hematopoietic progenitor cell transplantation (HPCT) in order to design rules for their management. METHODS: We reviewed 361 consecutive patients over 6 years, including 245 autologous and 116 allogeneic HPCT recipients for hematologic malignancies (87%) and solid cancers (13%). RESULTS: Fifty-seven patients developed 65 symptomatic neurolo...

C Denier, J-H Bourhis, C Lacroix, S Koscielny, J Bosq, R Sigal, G Said, D Adams (2006) Neurology 67: 11 1990-1997

Multifocal neuropathy due to plasma cell infiltration of peripheral nerves in multiple myeloma.

2010-06-20T06:44:16Z

Patients with multiple myeloma (MM) can manifest a variety of neurologic complications. The authors report two patients who had development of a multifocal neuropathy related to infiltration of peripheral nerves by malignant plasma cells as the only manifestation of a relapse of MM, which was considered in full remission.

C Denier, P Lozeron, D Adams, D Decaudin, F Isnard-Grivaux, C Lacroix, G Said (2006) Neurology 66: 6 917-918

Pilomotor seizures associated with sequential changes in magnetic resonance imaging.

2012-05-05T12:02:06Z

Piloerection is rarely described in seizures. This symptom has been most frequently observed in patients with temporal lobe epilepsy and is rarely the principal clinical feature of seizures. No specific etiology of epilepsy associated with pilomotor seizures has been reported. We present the first case of a patient who experienced sudden and transitory epilepsy with pilomotor seizures occurring se...

Pascal Masnou, Jean-Paul Gagnepain, Amal Fouad, Denis Ducreux, David Adams (2006) Epileptic Disord 8: 3 232-237

Impact of liver transplantation on cardiac autonomic denervation in familial amyloid polyneuropathy.

2010-06-20T06:33:58Z

Familial amyloid polyneuropathy (FAP) is a rare and severe hereditary form of amyloidosis, due to the deposition of a genetic variant transthyretin essentially produced by the liver, and characterized by both sensorimotor and autonomic neuropathy. Liver transplantation (LT) is the most effective treatment to stop the progression of the disease. Cardiac amyloid infiltration is usually associated wi...

Nicolas Delahaye, François Rouzet, Laure Sarda, Carmen Tamas, Sylvie Dinanian, Violaine Plante-Bordeneuve, David Adams, Didier Samuel, Pascal Merlet, André Syrota, Michel S Slama, Dominique Le Guludec (2006) Medicine (Baltimore) 85: 4 229-238

MR diffusion tensor imaging, fiber tracking, and single-voxel spectroscopy findings in an unusual MELAS case.

2010-06-20T06:46:54Z

A 23-year-old man was admitted to the intensive care unit for respiratory failure, global lower and upper limb palsy, and higher cognitive function deterioration. Imaging, performed with a combination of the MR diffusion tensor imaging, fiber tracking, and MR spectroscopy, suggested the diagnosis of an acute severe unusual mitochondrial encephalopathy, lactic acidosis, and strokelike event, which ...

Denis Ducreux, Ghaidaa Nasser, Catherine Lacroix, David Adams, Pierre Lasjaunias (2005) AJNR Am J Neuroradiol 26: 7 1840-1844

Developments in the treatment of hereditary amyloid neuropathies

2010-06-20T06:33:58Z

D Adams (2004) Rev Neurol (Paris) 160: 12 1234-1238

Changes in renal function in patients with familial amyloid polyneuropathy treated with orthotopic liver transplantation.

2010-06-20T06:33:58Z

BACKGROUND: Familial amyloid polyneuropathy (FAP) is an autosomal dominant disease caused by a point mutation in the gene encoding transthyretin, which is secreted by the liver. Orthotopic liver transplantation (OLT) has been proposed to prevent disease progression. Little is known about long-term changes in renal function and lesions after OLT. METHODS: The renal function of 33 patients with FAP ...

Renaud Snanoudj, Antoine Durrbach, Eric Gauthier, David Adams, Didier Samuel, Sophie Ferlicot, Pierre Bedossa, Alain Prigent, Henri Bismuth, Bernard Charpentier (2004) Nephrol Dial Transplant 19: 7 1779-1785

MRI of the brachial plexus

2010-06-20T06:46:54Z

Magnetic resonance imaging is the method of choice for the evaluation of brachial plexopathy. Knowledge of the anatomy and normal imaging appearance is required. High-resolution imaging technique is necessary with the use of adequate coils. Evaluation of the brachial plexus requires T1 weighted sequences in three plans, T2 weighted sequences with fat suppression and if necessary the study is compl...

M C Petit-Lacour, D Ducreux, D Adams (2004) J Neuroradiol 31: 3 198-206

Inflammatory vasculopathy in multifocal diabetic neuropathy.

2010-06-20T06:50:51Z

Besides the common distal symmetrical sensory-motor polyneuropathy (DSP) that is often associated with autonomic dysfunction, diabetic patients may develop multifocal sensory-motor deficits (MDN) secondary to roots, plexus and nerve trunk involvement. Nerve ischaemia has been suggested as a common mechanism for the different patterns of diabetic neuropathies, yet the important clinical differences...

Gérard Said, Catherine Lacroix, Pierre Lozeron, Angèle Ropert, Violaine Planté, David Adams (2003) Brain 126: Pt 2 376-385

Genetic study of transthyretin amyloid neuropathies: carrier risks among French and Portuguese families.

2010-06-20T06:33:58Z

V Planté-Bordeneuve, J Carayol, A Ferreira, D Adams, F Clerget-Darpoux, M Misrahi, G Said, C Bonaïti-Pellié (2003) J Med Genet 40: 11

Myocardial muscarinic receptor upregulation and normal response to isoproterenol in denervated hearts by familial amyloid polyneuropathy.

2010-06-20T06:33:58Z

BACKGROUND: Patients with familial amyloid polyneuropathy, a rare hereditary form of amyloidosis, have progressive autonomic neuropathy. The disease usually does not induce heart failure but is associated with sudden death, conduction disturbances, and an increased risk of complications during anesthesia. Although cardiac sympathetic denervation has been clearly demonstrated, the postsynaptic stat...

N Delahaye, D Le Guludec, S Dinanian, J Delforge, M S Slama, L Sarda, F Dollé, H Mzabi, D Samuel, D Adams, A Syrota, P Merlet (2001) Circulation 104: 24 2911-2916

Hereditary and acquired amyloid neuropathies.

2010-06-20T06:33:58Z

Amyloid neuropathies occur in a context of hereditary (FAP) or acquired amyloidosis. They present usually as severe and progressive polyneuropathy and carry a poor prognosis. Most FAP are associated with endoneurial deposits of variant transthyretin (TTR) with substitution of one aminoacid and are secondary to a point mutation of the TTR gene. Portugal is the main endemic area of TTR-FAP, secondar...

D Adams (2001) J Neurol 248: 8 647-657

Sensory and motor peripheral neuropathy, high cerebrospinal fluid protein and monoclonal IgG immunoglobulins in a 38 year-old man

2010-06-20T06:52:01Z

D Adams, S Schaeffer, F Chapon (2000) Rev Neurol (Paris) 156: 12 1166-1170

Familial amyloid polyneuropathy type I complicated by chronic glaucoma: 3 cases

2010-06-20T06:33:58Z

PURPOSE: We report 3 cases of familial amyloid polyneuropathy type I (FAP) with amyloid infiltration of the vitreum and glaucoma. PATIENTS AND METHODS: We reviewed the records of three patients, 2 females and one male, aged 41, 47 and 83 years respectively. The 3 patients had familial amyloid polyneuropathy type I with vitreous infiltration and open angle glaucoma. The two women underwent a liver ...

E Frau, M Lautier-Frau, F Saindelle-Ribeaudeau, D Adams, A Bousquet, M Labétoulle, H Offret (2000) J Fr Ophtalmol 23: 3 217-220

The course and prognostic factors of familial amyloid polyneuropathy after liver transplantation.

2010-06-20T06:33:58Z

Familial amyloid polyneuropathy (FAP) associated with mutations of the transthyretin (TTR) gene is the most common type of FAP, a devastating disease causing death within 10 years after the first symptoms. Because most of the amyloidogenic mutated TTR is secreted by the liver, transplantation is widely used to treat these patients, but long-term quantitative evaluation of the effects of liver tran...

D Adams, D Samuel, C Goulon-Goeau, M Nakazato, P M Costa, C Feray, V Planté, B Ducot, P Ichai, C Lacroix, S Metral, H Bismuth, G Said (2000) Brain 123 ( Pt 7): 1495-1504

Domino liver transplants for metabolic disorders: experience with familial amyloidotic polyneuropathy.

2010-06-20T06:50:51Z

BACKGROUND: Shortage of liver donors means that new methods of liver procurement must be explored. In domino transplantation, organs explanted during transplantation in one patient are transplanted into a second patient. Domino procedures can be performed with livers from patients having transplantation for hepatic metabolic disorders that cause systemic disease without affecting other liver funct...

D Azoulay, D Samuel, D Castaing, R Adam, D Adams, G Said, H Bismuth (1999) J Am Coll Surg 189: 6 584-593

Cardiac sympathetic denervation in familial amyloid polyneuropathy assessed by iodine-123 metaiodobenzylguanidine scintigraphy and heart rate variability.

2010-06-20T06:33:58Z

Familial amyloid polyneuropathy (FAP) is a rare and severe hereditary form of amyloidosis, due to nervous deposits of a genetic variant transthyretin produced by the liver and characterized by both sensorimotor and autonomic neuropathy. Left ventricular systolic dysfunction is rare, but conduction disturbances and sudden deaths can occur. The neurological status of the heart has not been elucidate...

N Delahaye, S Dinanian, M S Slama, H Mzabi, D Samuel, D Adams, P Merlet, D Le Guludec (1999) Eur J Nucl Med 26: 4 416-424

Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy.

2010-06-20T06:50:51Z

OBJECTIVE: To evaluate the clinicopathologic features and prognostic factors of 100 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Comparison of clinical and biopsy findings with functional score evaluated an average of 6 years after referral. RESULTS: CIDP followed a relapsing course in 14% of the patients and a progressive course in 45%. After progressive onset,...

C Bouchard, C Lacroix, V Planté, D Adams, F Chedru, J M Guglielmi, G Said (1999) Neurology 52: 3 498-503

New transthyretin variants SER 91 and SER 116 associated with familial amyloidotic polyneuropathy. Mutations in brief no. 151. Online.

2010-06-20T06:50:51Z

Mutations of the transthyretin (TTR) gene are associated with familial amyloidotic polyneuropathy (FAP). Two new mutations were detected in French patients with TTR amyloidosis. The first patient was a 72 year old man who presented with severe and rapidly evolving sensory motor polyneuropathy of the 4 limbs, a bilateral carpal tunnel syndrome and a restrictive cardiomyopathy. His father died after...

A M Misrahi, V Plante, T Lalu, L Serre, D Adams, D C Lacroix, G Saïd (1998) Hum Mutat 12: 1

Genotypic-phenotypic variations in a series of 65 patients with familial amyloid polyneuropathy.

2010-06-20T06:33:58Z

OBJECTIVE: To investigate the genotypic-phenotypic variations in a series of patients with familial amyloid polyneuropathy (FAP). BACKGROUND: Progress in molecular genetics has led to the identification of point mutations in the transthyretin (TTR) gene in FAP--a dominantly inherited neuropathy with a fatal outcome. These findings have modified the management of patients with small-fiber neuropath...

V Planté-Bordeneuve, T Lalu, M Misrahi, M M Reilly, D Adams, C Lacroix, G Said (1998) Neurology 51: 3 708-714

Ultrastructural characterisation of the M protein in nerve biopsy of patients with POEMS syndrome.

2010-06-20T07:07:04Z

To learn more about the mechanisms of nerve lesions in POEMS syndrome, nerve specimens from four patients were studied with an immunogold method at the ultrastructural level to detect and localise the M protein in the different nerve compartments. An indirect immunolabelling technique was applied on 4% PFA fixed and LR White embedded nerve specimens. Antisera against IgG, IgA, IgM, and lambda and ...

D Adams, G Said (1998) J Neurol Neurosurg Psychiatry 64: 6 809-812

Painful proximal diabetic neuropathy: inflammatory nerve lesions and spontaneous favorable outcome.

2010-06-20T07:21:49Z

Proximal diabetic neuropathy is a disabling neuropathy that occurs predominantly in non-insulin-dependent diabetic patients over the age of 50. Inflammatory lesions have been found in nerve biopsy specimens of diabetic patients with severe proximal neuropathy or with other patterns of multifocal neuropathy. Some of these patients respond dramatically to treatment with corticosteroids or with other...

G Said, F Elgrably, C Lacroix, V Planté, C Talamon, D Adams, M Tager, G Slama (1997) Ann Neurol 41: 6 762-770

Multifocal motor neuropathies with conduction blocks. 39 cases

2010-06-20T06:52:57Z

Clinical, biological and electrophysiological features from a cohort of 39 multifocal motor neuropathies with conduction blocks (NMM with CB) have been studied. There were 29 males and 10 females with an average of 47.3. At the first evaluation, the mean duration of the symptoms was of 8 years with extremes between 1 and 28. Pain and paresthesias were present in respectively 10 and 18 p. 100 of th...

N Le Forestier, B Chassande, A Moulonguet, T Maisonobe, S Schaeffer, N Birouk, N Baumann, D Adams, J M Léger, V Meininger, G Said, P Bouche (1997) Rev Neurol (Paris) 153: 10 579-586

Maintained all-trans retinoic acid therapy in a patient with pseudotumour cerebri despite aggravated symptoms.

2010-06-20T06:56:44Z

D Decaudin, D Adams, P Naccache, L Castagna, J N Munck (1997) Leuk Lymphoma 27: 3-4 373-374

Higher doses of alpha-interferon do not increase the activity of the weekly cisplatin-interferon combination in advanced malignant mesothelioma.

2010-06-20T07:02:52Z

Management of advanced malignant mesothelioma (MM) still requires innovative systemic therapy as its prognosis is poorly affected by currently available chemotherapy. The combination cisplatin and alpha-interferon (alpha-INF) has synergistic antitumoral activity in preclinical models and interesting activity in phase I-II clinical trials. Weekly CDDP (60 mg/m2) and alpha-IFN (3 MUI/d: d1-d4) in co...

L Trandafir, P Ruffié, C Borel, I Monnet, P Soulié, D Adams, E Cvitkovic, J P Armand (1997) Eur J Cancer 33: 11 1900-1902

Contribution of nerve biopsy findings to the diagnosis of disabling neuropathy in the elderly. A retrospective review of 100 consecutive patients.

2010-06-20T07:06:10Z

Peripheral neuropathy is an important factor of disability in the elderly. In order to learn more on the usefulness of intensive evaluation of patients over 65 years of age with subacute or chronic disabling peripheral neuropathy, we reviewed the clinical and nerve biopsy findings of the last 100 patients of this age group who suffered from a peripheral neuropathy severe enough to justify performa...

L Chia, A Fernandez, C Lacroix, D Adams, V Planté, G Said (1996) Brain 119 ( Pt 4): 1091-1098

Ultrastructural immunolabelling of amyloid fibrils in acquired and hereditary amyloid neuropathies.

2010-06-20T06:33:58Z

Both acquired and familial amyloid neuropathies carry a poor prognosis. In addition, amyloid is sometimes difficult to visualise in nerve biopsy specimens, and the pathogenesis of nerve lesions is still a matter of controversy. In order to learn more on the subject, we studied nerve specimens from seven patients with proven amyloid neuropathy by ultrastructural immunocytochemistry in order to bett...

D Adams, G Said (1996) J Neurol 243: 1 63-67

Hereditary amyloid neuropathies

2010-06-20T06:33:58Z

D Adams, G Said (1995) Ann Med Interne (Paris) 146: 3 157-165

Haplotype analysis of French, British and other European patients with familial amyloid polyneuropathy (met 30 and tyr 77).

2010-06-20T06:33:58Z

Familial amyloid polyneuropathy (FAP) is an autosomal dominant disorder originally and most frequently described in Portugal. The usual constituent amyloid fibril protein is transthyretin (TTR) and the most frequent mutation in the TTR gene associated with FAP (including all Portuguese cases) is that at position 30 (met 30). Three different TTR haplotypes have been described in association with th...

M M Reilly, D Adams, M B Davis, G Said, A E Harding (1995) J Neurol 242: 10 664-668

Multifocal motor neuropathy with conduction block: a study of 24 patients.

2010-06-20T06:52:57Z

Twenty four patients with pure motor neuropathy are reported. The chronic motor involvement associated with fasciculations and cramps, mainly in the arms, led, in most patients, to an initial diagnosis of motor neuron disease. In some patients (nine of 24), there was no appreciable muscle atrophy. Tendon reflexes were often absent or weak. The finding of persistent multifocal conduction block conf...

P Bouche, A Moulonguet, A B Younes-Chennoufi, D Adams, N Baumann, V Meininger, J M Léger, G Said (1995) J Neurol Neurosurg Psychiatry 59: 1 38-44

Transthyretin gene analysis in European patients with suspected familial amyloid polyneuropathy.

2010-06-20T06:33:58Z

We investigated 99 patients from 64 European families (51 French, 11 British, one Italian and one Spanish) with suspected familial amyloid polyneuropathy (FAP) for transthyretin (TTR) gene mutations. Thirty-nine families were found to have point mutations causing the following amino acid substitutions: Met30 (28 families), Tyr77 (five), Arg 50 (one), Ala49 (one), Gln89 (one), Ala60 (one) and one e...

M M Reilly, D Adams, D R Booth, M B Davis, G Said, M Laubriat-Bianchin, M B Pepys, P K Thomas, A E Harding (1995) Brain 118 ( Pt 4): 849-856

Phase I/II study of paclitaxel plus cisplatin as first-line chemotherapy for advanced non-small cell lung cancer: preliminary results.

2010-06-20T07:02:52Z

From March 1993 to May 1994, 32 chemotherapy-naive patients with advanced non-small cell lung cancer entered a phase I/II study to determine the maximum tolerated dose and the activity of the paclitaxel (Taxol; Bristol-Myers Squibb Company, Princeton, NJ)/cisplatin combination. The 21 men and 11 women had a median age of 59 years (range, 25 to 72 years) and a median performance status of 1 (range,...

L Belli, T LeChevalier, M Gottfried, D Adams, P Ruffie, A LeCesne, L Tete, B Pellae-Cosset (1995) Semin Oncol 22: 6 Suppl 15 29-33

Poems syndrome with high interleukin (IL)6 and IL1 beta serum levels, in a patient with thyroid carcinoma and melanoma.

2010-06-20T07:00:46Z

E Tartour, D Adams, J F Besancenot, W H Fridman, M Schlumberger (1994) Eur J Cancer 30A: 6 893-894

Motor conduction block and high titres of anti-GM1 ganglioside antibodies: pathological evidence of a motor neuropathy in a patient with lower motor neuron syndrome.

2010-06-20T06:55:01Z

A patient with a progressive lower motor neuron syndrome and neurophysiological evidence of motor axon loss, multifocal proximal motor nerve conduction block, and high titres of anti-ganglioside GM1 antibodies. Neuropathological findings included a predominantly proximal motor radiculoneuropathy with multifocal IgG and IgM deposits on nerve fibres associated with a loss of spinal motor neurons. Th...

D Adams, T Kuntzer, A J Steck, A Lobrinus, R C Janzer, F Regli (1993) J Neurol Neurosurg Psychiatry 56: 9 982-987

Usefulness of technetium-99m pyrophosphate myocardial scintigraphy in amyloid polyneuropathy and correlation with echocardiography.

2010-06-20T06:33:58Z

C Fournier, G Grimon, J P Rinaldi, A Terral, B Boujon, D Adams, A Desgrez, M Blondeau (1993) Am J Cardiol 72: 11 854-857

Transthyretin gene mutations in British and French patients with amyloid neuropathy.

2010-06-20T06:33:58Z

Five patients, two British and three French, with late onset amyloid neuropathy were found to have mutations of the transthyretin (TTR) gene associated with the Portuguese and German types of familial amyloid polyneuropathy. Familial amyloid polyneuropathy is rare in the United Kingdom and has not previously been defined at a molecular genetic level. None of the patients had a history of affected ...

K Bhatia, M Reilly, D Adams, M B Davis, C H Hawkes, P K Thomas, G Said, A E Harding (1993) J Neurol Neurosurg Psychiatry 56: 6 694-697

Gadolinium-enhanced MRI in central nervous system Behçet's disease.

2010-06-20T06:45:38Z

Two cases of central nervous system Behçet's disease, studied by gadolinium-enhanced MRI, are presented. In one patient, whose clinical picture was dominated by a brain syndrome, the gadolinium enhancement resolved with clinical improvement, although the hyperintense areas in the mesencephalon on T2-weighted images persisted. In the second, who had a pseudobulbar palsy and a mild right hemiparesi...

E Erdem, R Carlier, A B Idir, P O Masnou, A Moulonguet, D Adams, D Doyon (1993) Neuroradiology 35: 2 142-144

Late-onset familial amyloid polyneuropathy with the TTR Met 30 mutation in France.

2010-06-20T06:53:53Z

Four unrelated French cases of familial amyloid polyneuropathy are reported. Clinical onset ranged from the sixth to the ninth decade. Sensory signs were predominant initially in the lower limbs; motor changes, and in one case autonomic involvement, appeared later. Amyloid disease was clinically limited to the peripheral nervous system. In two cases, there was no evidence of familial disease. DNA ...

G Grateau, D Adams, D Malapert, M Viemont, M Delpech, G Said (1993) Clin Genet 43: 3 143-145

Chlorambucil fails to improve patients with motor neuropathies and antibodies to gangliosides.

2010-06-20T06:55:01Z

T Kuntzer, A J Steck, D Adams, F Regli (1992) J Neurol Neurosurg Psychiatry 55: 9 857-858

Demonstration of genetic mutation in most of the amyloid neuropathies with sporadic occurrence

2010-06-20T06:33:58Z

The Portuguese type of familial amyloid polyneuropathy (FAP type I) is a disabling autosomic dominant disorder, which is caused by a point mutation in the transthyretin (TTR) gene. Other TTR gene mutations have been reported recently in other FAP. In the absence of monoclonal gammopathy, sporadic amyloid neuropathies raise a problem for their pathogenicity. In this study, we have looked for TTR ge...

D Adams, M Reilly, A E Harding, G Said (1992) Rev Neurol (Paris) 148: 12 736-741

Motor neuron syndromes and monoclonal IgM antibodies to gangliosides.

2010-06-20T06:55:01Z

A J Steck, D Adams (1991) Adv Neurol 56: 421-425

Predictive value of anti-GM1 ganglioside antibodies in neuromuscular diseases: a study of 180 sera.

2010-06-20T06:55:01Z

The incidence of anti-GM1 antibodies in the serum of 104 patients with neurological diseases, 35 patients with non-neurological diseases (NND) and 41 normal controls was determined by enzyme-linked immunosorbent assay (ELISA). Anti-GM1 antibodies were found in 90% of patients presenting with a motor neuropathy (all except one had multifocal conduction blocks). A large proportion (60%) of these pat...

D Adams, T Kuntzer, D Burger, M Chofflon, M R Magistris, F Regli, A J Steck (1991) J Neuroimmunol 32: 3 223-230

Acute myeloradiculitis due to cytomegalovirus as the initial manifestation of AIDS.

2010-06-20T06:57:52Z

A 26 year old male intravenous drug abuser presented with rapidly progressive paraplegia and total incontinence. CSF examination showed elevated protein level and pleocytosis. HIV testing was positive. Anti CMV titres were mildly elevated in serum and CSF. Death occurred 26 days after the onset of neurological signs. Necrotic and inflammatory lesions with numerous inclusion bodies characteristic o...

F Mahieux, F Gray, G Fenelon, R Gherardi, D Adams, A Guillard, J Poirier (1989) J Neurol Neurosurg Psychiatry 52: 2 270-274