ALS and muscle consultation
Head: Prof. Dr. med. S. Petri
The institute founded by the Society for Muscular Dystrophy (DGM) certified Neuromuscular Center of the MHH (Hannover Medical School) is an interdisciplinary association of the following Clinical Departments:
Neurology, Neuropathology, Pediatric Muscle Consultation, Cardiology, Orthopedics, Pneumology, Immunology and Rheumatology, Human Genetics, Physical Medicine and Rehabilitation, Siloah Clinic (Pneumology), Children's Hospital on the Bult (Neuropediatrics), Hanover Social Pediatric Center.
Patients with neuromuscular diseases from all over northern Germany are examined and cared for in the neuromuscular consultation hours. When new patients present, an initial outpatient diagnosis is carried out and, if necessary, an inpatient stay is organized. In addition, further treatment and regular follow-up examinations are coordinated here if long-term care appears appropriate and is desired. In addition to clinical assessment, extensive laboratory tests, genetic tests and modern imaging and neurophysiological procedures in laboratories with many years of experience with neuromuscular diseases are available for diagnosis. Furthermore, muscle and nerve biopsies can be carried out, which are examined in the Department of Neuropathology and, if necessary, in other specialized laboratories.
Outpatient care for patients with neuromuscular diseases has been provided in accordance with Section 116b since January 2011. The outpatient clinic has a special clinical and scientific focus on motor neuron diseases, in particular amyotrophic lateral sclerosis (ALS), post-polio syndrome and spinal muscular atrophy (SMA). The spectrum of diseases also includes inflammatory and hereditary muscle diseases, disorders of neuromuscular transmission (myasthenic syndromes) and inflammatory and hereditary diseases of the peripheral nervous system (autoimmune/hereditary neuropathies).
There is a long-standing close cooperation with the German Society for Muscle Diseases (DGM) .
Ms. Anke Meier, social worker at the DGM(www.dgm.org), holds regular consultation hours with advice on the provisions of the law on severe disabilities and social assistance, securing Nursing and care, and other topics. It supports patients in organizing rehabilitation measures, initiating home Nursing and other measures.
Since 2012, the Muscle Center Hannover has been part of the Center for Rare Diseases (ZSE) of the MHH. The ZSE aims to strengthen interdisciplinary cooperation in the field of rare diseases, for example through monthly case conferences and two physicians acting as guides who process and forward patient inquiries so that patients with neuromuscular diseases also come to our consultation hours in this way.
Neuromuscular seminar
Once a semester, the Neuromuscular Center organizes a neuromuscular seminar with current case presentations including neuropathological demonstrations and scientific lectures for physicians and other interested parties (schedule of Wednesday colloquia)
Donations
Dear patients, relatives and friends of the Neurological Clinical Department, we do our best to look after our patients. If you were satisfied with our services, you might want to show us that. Financial contributions in the form of donations help us the most to support our various tasks, especially scientific activities. Unfortunately, the funds we receive from the state of Lower Saxony for research and teaching are not sufficient for this.
A suitable donation option would be
Society of Friends of Hannover Medical School
Commerzbank Hanover
BANK ACCOUNT NUMBER : 250 400 66
Account number : 3 120 003 01
IBAN: DE12 2504 0066 0312 0003 01
BIC: COBADEFFXXX
Purpose: Sub-account 11000238 - ALS - Neurological Department Clinical Department MHH (first name and surname if applicable)
The Society of Friends of the MHH can issue correct donation receipts if you send your address(freundedermhh@mh-hannover.de).
We are extremely grateful for every donation. Of course, we are happy to show our donors the Clinical Department and the scientific Facilities or Institutions of the Department of Neurology in person.
With best regards
Comm. Director Prof. Dr. med. S. Petri
Studies
ALS:
- ROCK-ALS: a Randomized, Placebo-Controlled, Double-Blind Phase IIa Trial of Safety, Tolerability and Efficacy of the Rho Kinase (ROCK) Inhibitor Fasudil in Amyotrophic Lateral Sclerosis. The study investigates the efficacy and tolerability of the substance Fasudil as well as the effect on the course of ALS disease. Fasudil is an inhibitor of the enzyme Rho-kinase (called ROCK), which is supposed to inhibit the destruction of motor nerve cells by stabilizing the cytoskeleton (cell framework).
- REFALS: Effects of oral Levosimendan (OMD-109) on respiratory function in patients with ALS. Levosimendan increases the performance of weakened muscles by activating troponin, has anti-inflammatory properties and can reduce the deposition of proteins (protein aggregation) in nerve cells in ALS.
- ORARIALS-01: A Phase III, Randomized, Placebo-Controlled Trial of Arimoclomol in Amyotrophic Lateral Sclerosis. Arimoclomol is a chaperone activator designed to delay the pathological deposition of specific proteins (protein aggregation of TDP-43, SOD-1 and FUS) in motor neurons.
Post-polio syndrome:
- Multicenter, prospective, randomized, placebo-controlled, double-blind, parallel-group study to investigate the efficacy and safety of intravenous immunoglobulins (Flebogamma 5% DIF) in patients with post-polio syndrome (FORCE).
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis is a very serious disease of the central and peripheral nervous system. With the exception of rare hereditary forms, its exact cause is still unknown. The disease leads to damage to motor nerve cells in the brain (primary motor cortex, brain stem) and spinal cord. This leads to muscle wasting (atrophy), muscle weakness (paresis) and involuntary muscle twitching (fasciculations). If the motor nerve cells located in the brain stem are affected, the speech, chewing and swallowing muscles are weakened. If the anterior horn cells located in the spinal cord are affected, muscle atrophy and weakness develop in the arms and legs and also in the respiratory muscles. The disease of the central motor system, i.e. the nerve cells in the cerebral cortex and their connections to the spinal cord, leads to weakness and an increase in muscle tone (spastic paralysis).
As the cause of ALS is not yet known, there is currently no causal treatment that can halt or even cure the disease. So far, only the active ingredient riluzole, which reduces the cell-damaging effect of the neurotransmitter glutamate and has shown a significant, albeit moderate, increase in life expectancy in studies, has been admitted for the treatment of ALS in Europe. Other drugs are in various phases of clinical trials and may be admitted in the next few years. (Link to studies). In addition, a range of treatment measures are available that can alleviate the symptoms and improve the patient's quality of life.
Spinal muscular atrophy
Spinal muscular atrophy (SMA) is a hereditary motor neuron disease caused by mutations in the SMN (survival of motoneuron) gene. The loss of motor nerve cells in the spinal cord leads to a progressive decline in motor functions, accompanied by paralysis, sometimes also respiratory weakness and curvature of the spine. The disease can be visible at birth or shortly afterwards, but can also occur later in childhood or young adulthood and progress at different rates. A distinction is made between four disease types (SMA1 - 4) depending on the severity and age at onset.
Since June 2017, the antisense oligonucleotide nusinersen, which is administered directly into the cerebrospinal fluid via lumbar puncture, has been the first causal therapy for SMA to be admitted to the market. The MHH is one of the few German centers to offer this therapy.