On behalf of the german society of neurology (dgn) we developed guidelines on neurogenic dysphagia (nd), which were accepted i
Guidelines of the German Society of Neurology (DGN) on Neurogenic Dysphagia (ND) Mario Prosiegel
Neurologisches Krankenhaus München (NKM), Tristanstr. 20, D-80804 Munich, Germany
The abovementioned guidelines were accepted by the DGN guidelines commission in September 2004 and will be published by the DGN in 2005. The main recommendations of these guidelines are as follows: ! Diagnosis of ND is possible in most cases by accurate history taking and neurological
examination. If the etiology of ND is unknown, a checking list may be of value (see below), in order not to forget any cause.
! For bedside screening examination we recommend the 50-ml water test combined with
assessment of pharyngeal sensibility or with pulse oximetry.
! Concerning the monitoring of swallowing therapy, the diagnostic expressiveness of
videofluoroscopic swallowing study (VFFS) and fiberoptic endoscopic evaluation of swallowing (FEES) is probably equivalent. At least in the beginning of swallowing therapy, VFSS should be performed in order to reveal (frequent) dysfunctions of the upper esophageal sphincter (UES) which cannot be seen by FEES.
! With regard to VFSS we recommend a training period of at least ½ year in about 300
patients under supervision of an experienced radiologist. In Germany, only phoniatrists are obliged to study FEES so that other professions need to see to their own further education themselves.
! Important sequelae of ND, which should be avoided or minimized by special
penetration/aspiration, aspiration pneumonia, dependence on tube feeding and/or tracheal cannula, high health care costs, reduced quality of life and death.
! L-dopa, amantadine or ACE inhibitors may be applied in certain cases, since they might
elicit the swallowing reflex or are protective against aspiration pneumonias. Study results are, however, contradictory.
! Many disturbances which occur in association with ND can be treated pharmacologically
very successfully, e.g hiccup with a combination of baclofen, domperidone and a proton pump inhibitor (Gabapentin may be added in severe cases); gastroesophageal reflux disease (GERD) should be treated with proton pump inhibitors, since GERD may aggravate a preexisting ND.
! Oral hygiene of the patients and hand desinfection of the people handling them seem to be
effective interventions against aspiration pneumonia in ND patients.
! The prerequisites for cricopharyngeal myotomy in cases of UES dysfunction are: normal
elevation of hyoid and larynx, non-successful swallowing therapy in achieving the opening of the UES and pharyngeal pressure sufficient to propel a bolus through the UES. Therefore, manofluoroscopy is necessary prior to these procedures. The same prerequisites hold true for Botulinum toxin injection of the UES as an alternative method. Balloon catheter dilatation of the UES cannot be generally recommended for lack of study results and small examined patient numbers.
! If tube feeding is indicated in acute neurological diseases such as stroke, nasogastral tube
feeding seems to be superior to feeding via percutaneous endoscopic gastrostomy (PEG) within the first month. In longlasting ND (> 1 month) PEG feeding is the treatment of choice; in progressive diseases such as amyotrophic lateral sclerosis, PEG should be
performed before forced vital capacity (fVC) is less than 50-60%; in the latter case PEG should be performed using noninvasive positive pressure ventilation, oxygen support and conscious sedation; an alternative is percutaneous radiological gastrostomy (PRG).
! In cases with trachetomy and a blocked cuff, intermittent subglottic secretion drainage
seems to be an effective intervention for aspiration pneumonia.
! Patients with a dilatative tracheostomy should neither be admitted into rehab units or
nursing homes nor be discharged home without trained personell.
! There are many effective methods of functional swallowing therapy. The efficacy of
restitutional methods has not yet sufficiently been investigated. Compensatory methods (postural changes and swallowing techniques/maneuvers) are effective depending on the dysphagic disturbance pattern. Dietary modification such as thickening of liquids are effective, provided that their effect is controlled by use of VFSS and/or FEES.
! Faciooral tract therapy (F.O.T.T.), which may also be performed in uncooperative or
unconscious patients with ND, is an empirical therapy, but cannot be recommended for lack of evidence-based results regarding efficacy.
Checking list in cases of unknown etiology of neurogenic dysphagia Electromyography (EMG), repetitive nerve stimulation
Critical illness polyneuropathy/myopathy
(CIP/CIM), acute inflammatory demyelinating polyneuritis (AIDP)/acute Guillain-Barré syndrome (aGBS), myotonic dystrophies, myasthenia gravis, Lambert-Eaton myasthenic syndrome (LEMS)
Multiple sclerosis (MS), brainstem diseases, multisystem atrophies (MSA), Chiari malformation
Acetylcholine receptor (AChR) Ab; muscle-specific
tyrosine kinase (MuSK) antibodies Myositis-specific auto-Ab; anti-Jo-1-auto-Ab
Polymyositis (PM), dermatomyosistis (DM), inclusion body myositis (IBM); anti-Jo-1 syndrome
anti-dsDNA/ribosomal P Ab; anti-SS-A/Ro/SS-B/LA Ab;
Systemic lupus erythematodes (SLE); Sjögren´s
myositis-specific Ab; anti-Scl-70 Ab, anti-Centromer Ab;
syndrome (SS); PM/DM/(IBM); systemic sclerosis
(SSc); mixed connective tissue disease (MCTD)
Antineutrophil cytoplasmic antibodies (ANCAs), HBSAG:
c-ANCA (proteinase 3 [PR3]), p-ANCA (myeloperoxidase Wegener's granulomatosis (c-ANCA); microscopic [MPO]), anti-endothelial cell antibodies (AECA), HBSAG polyangiitis (p-ANCA); classic polyarteriitis
Variants of Guillain-Barre syndrome (GBS):
Polyneuritis cranialis; Miller Fisher syndrome
CADASIL (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) (granular osmophilic material [GOM] in arterial walls)
MSA, progressive supranuclear palsy (PSP)
CADASIL, spinobulbar muscle atrophy (SBMA; Kennedy´s disease), oculopharyngeal muscle dystrophy (OPMD)
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