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The Centre for Neuromuscular Disease

Brigham Womens Logo

Institute of Neurology (IoN)

National Hospital for Neurology and Neurosurgery (NHNN)


Contact Information:

Centre for Neuromuscular Disease
Department Molecular Neuroscience,
Institute of Neurology and National Hospital for Neurology
University College London
Queen Square
London
WC1N 3BG

Phone: 00 44 0207 837 3611 ext 4251
FAX: 00 44 0207 692 1208

Contact Person:

Dr. Michael G Hanna BSc(Hons) M.D., FRCP
Consultant Neurologist and Reader in Clinical Neurology
e-mail: m.hanna@ion.ucl.ac.uk

Consultants

Dr. MG Hanna, M.D., FRCP
Dr. MM Reilly, M.D., FRCP, FRCPI
Professor NW Wood, Ph.D., FRCP
Professor DM Kullmann, M.D., Ph.D., FRCP (Experimental Epilepsy Group - Department of Clinical and Experimental Epilepsy)

Other members of the team:

Anne Grayson
NSCAG secretary

Cath Evans
Specialist Neuromuscular Nurse

Dr. N. Murray
Consultant Clinical Neurophysiologist

A Physiotherapist (including myometry) is on-site.

Research Fellows

Dr. Fialho
Dr. Graves
Dr. Niranjanan

Channel DNA diagnostics

Dr. MB Davies
Dr. WF Wakeling
Dr. E Stanley

Current Studies:

5301: Andersen-Tawil Syndrome: Genotype-Phenotype Correlation and Longitudinal Study

5302: Episodic Ataxia Syndrome: Genotype-Phenotype Correlation and Longitudinal Study

5303: Non-dystrophic Myotonias: Genotype-Phenotype Correlation and Longitudinal Study

 

About Us:

History of the NHNN and IoN

In 1859 the National Hospital for the Paralyzed and the Epileptic was founded at Queen Square, London. Over the years the name of the hospital changed, most recently to the National Hospital for Neurology and Neurosurgery (NHNN), but throughout this time the hospital has cared for patients with a wide range of neurological illnesses. Closely linked to the hospital, the Institute of Neurology (IoN) was established in 1950 as one of the federated Institutes of the British Postgraduate Medical Federation of the University of London. It is the leading neurological research institute in the UK with a world-wide reputation for its academic work. In 1997 the IoN became a postgraduate institute of the University College London (UCL), In parallel the NHNN merged with the University College London Hospital Trust (UCLH), one of London’s major teaching hospitals.

The Centre for Neuromuscular Disease

At the Centre for Neuromuscular Disease, which is an integral part of the NHNN, over 1000 patients are assessed each year. The principle clinics of the centre are the muscle clinic (run by Dr MG Hanna) and the peripheral nerve clinic (run by Dr MM Reilly). Both clinics not only receive referrals from all over the Southeast of England but have a large national referral base for complex neuromuscular disorders. A high proportion of patients assessed are under regular follow-up. The centre also has significant inpatient activity both for diagnosis (e.g. muscle/nerve biopsy and neurophysiology) and treatment (e.g. immunosuppressive regimes and inpatient therapy services). Our team includes a specialist neuromuscular nurse who can be directly contacted by patients for advice and information. There are close links with the therapy services including physiotherapy, occupational therapy and speech and language therapy. Quantitative muscle strength measurement equipment operated by physiotherapists allows us monitor muscle strength more precisely thus helping to guide treatment and facilitating research.

The Neurological Channelopathies - Clinical Assessment, Treatment and Research

The investigation of neurological channelopathies has been a major research initiative within the Institute of Neurology since 1997 when Dr. Hanna established the Ion Channel Research group. In 1999 Dr Hanna and colleagues from Newcastle, Oxford and London established a consortium to improve the diagnostic accuracy and advice available to patients with certain rare neuromuscular diseases. As part of this National consortium we have received funding from the Department of Health via the National Specialist Commissioning Advisory Group (NSCAG) since 2001 to offer a comprehensive diagnostic and advisory service to patients with a group of neuromuscular disorders known as the skeletal muscle channelopathies. These disorders include conditions such as periodic paralyses and inherited myotonias. All these conditions are treatable but achieving an accurate diagnosis is complex. A precise diagnosis depends on a coordinated approach, which begins with a detailed clinical assessment and often also involves specialized neurophysiological assessment and DNA analysis.

The clinical service at the National Hospital offers a one-stop same-day assessment in which patients with muscle channelopathies are evaluated clinically by Dr Hanna and then undergo detailed electrophysiological testing in collaboration with Dr Nick Murray, Consultant Neurophysiologist. With informed consent patients are then offered detailed genetic testing to achieve a DNA-based diagnosis in collaboration between Dr MG Hanna, Dr MB Davis and Professor NW Wood in the DNA laboratory. Patients are followed up to receive genetic counseling and treatment. Effective medications are available in accurately diagnosed patients and often significantly improve quality of life. In addition advice and genetic testing is offered to patients who cannot attend for an assessment at the NHNN. Dr Hanna also has an interest in other neurological channelopathies, in particular episodic ataxias. We regularly assess, treat and follow-up patients with episodic ataxia and other brain channelopathies.

DNA testing is carried out by a team of dedicated scientists within the Department of Molecular Neuroscience under the lead of Professor NW Wood and Dr MB Davis. We currently offer screening for common mutations causing hypo- and hyperkalaemic periodic paralysis, myotonia congenita and paramyotonia congenita. On a research basis we can also test for mutations in KCNJ2 causing Andersen’s syndrome, CACNA1A causing episodic ataxia type 2 and KCNA1 causing episodic ataxia type 1. The range of tests available is under constant review and will expand significantly in the future.

Research activities are focused on examining the relationship between clinical phenotypes and genetic mutations identified in both muscle and brain channelopathies. We work in close collaboration with Professor DM Kullmann, who runs a basic neuroscience laboratory with facilities to examine newly identified mutations electrophysiologically in in-vitro expression systems.

Support

The National Muscle Channelopathy Service is supported by the Department of Health (NSCAG – National Commissioning Advisory Group). Research is supported by MRC, Wellcome, CINCH and UCLH Special Trustees.

Useful Links:

Experimental Epilepsy Group - Department of Clinical and Experimental Epilepsy

The Institute of Neurology Home Page

The National Hospital for Neurology and Neurosurgery

The Muscular Dystrophy Campaign Web Site - A UK based charity that gives advice and support to patients and relatives with muscular dystrophy and allied disorders; web site includes information on non-dystrophic myotonias and periodic paralysis

European Neuromuscular Centre Home Page - European network to stimulate collaboration and research into neuromuscular disorders

Scottish Muscle Network Web Site

Wales Neuromuscular Web Site