20.08.2010. NORDIC ALS Alliance 2010 & Baltic Bridge, Estonia

1. LATVIAN EXPERIENCE IN DIAGNOSTICS, TREATMENT AND CARE OFAMYOTROPHIC LATERAL SCLEROSIS (ALS)PATIENTS Dr. M.S.I. Naudiņa Head of the Centre for Neuromuscular Diseases Pauls Stradins Clinical University Hospital Elīna Pūcīte 1st year resident student in neurology Pauls Stradins Clinical University Hospital 20.08.2010. NORDIC ALS Alliance 2010& Baltic Bridge, Estonia

2. Centre of Amyotrophic Lateral Sclerosis (ALS) • Head: Dr. Zaiga Kalniņa • Experience: since 1998; ca. 20 patients per year • Contact information: zaigakalnina@inbox.lv • +37167069283 • Centre of NeuromuscularDiseases(NMD) • Head: Dr. M.S.I. Naudiņa • Experience: since 1978; ca. 350 patients per year • Contact information: masolna@inbox.lv • +371 67069283

3. The Incidence of ALS •  2 people out of 100 000 each year are ALS patients in Europe; • In the last 10 years there are193 ALS patients in Latvia; • In the period of 2003-2007,there were 85 patients diagnosed with ALS.

4. Diagnostic Possibilities in Latvia 1. Clinical features (according toEl Escorial diagnostic criteria for ALS) 2. Electrophysiological studies: • Nerve conduction studies • Needle electromyography 3. Brain or spinal MRI • Other laboratory studies (need to be tailored to the clinicalcircumstances, in search of treatable look-alikes) 5. Muscle biopsy andneuropathalogical studies ?

5. ALS PatientMedical Care in Latvia Education; Physiotherapy, ergotherapy; Symptomatic treatments; Ventilatory management; Nutritional management; Disease modifying treatments.

6. Summary Positive: 1) Diagnostics of ALS comply with generally accepted practice. 2) Family doctors are eager to refer patients to specialists with unclear and rare diseases. 3) Neurologist is a popular consultant for unclear patients. 4) Neurologist's recommendations are met primarily. 5) In case of suspicion the patient is admitted to a regional hospital or multidisciplinary clinic, where broad diagnostic tests are made, including those necessary for the differential diagnosis. 6) Statement of diagnosis does not tend to be delayed. 7) Number of diagnosed cases is growing.

7. Negative: 1.Although national recommendations exist, there is no national program that guarantee care standards. Systemic problem that is similar to many rare disease's care with resulting negative aspects: • Care quality differs markedly between the capital city, regions, and is dependent on the distance to the centre of region; • Recommendation quality may differ between specialists, as appointments are limited in time, but there are no official issued educational materials for patients; • The quality of care and support are strongly correlated with initiative and own activity of patient/relatives/caregivers. • Although physiotherapy and nursing care is widely available, it is not specific for ALS patients; • Availability of psychologist and psychotheraphy depends on the own initiative of a patient and solvency; • Support groups for patients and caregivers are not widelyavailable;

8. 2. Assistive, adaptive, and rehabilitative devices are provided by national programme, but waiting is inadequately long, many patients choose paid assistive technology: • Suction devices and mechanical ventilation is available almost only in hospitals; • When respiratory problems develop, patients are admitted in intensive care unit to provide mechanical ventilation (quality of life).

9. Thank you for your attention! • Centre of Amyotrophic Lateral Sclerosis (ALS) • Dr. Zaiga Kalniņa • Contact information:zaigakalnina@inbox.lv, • +37167069283 • Centre of NeuromuscularDiseases (NMD) • Dr. M.S.I. Naudiņa • Contact information: masolna@inbox.lv, +371 67069283