Update: The Care of the Patient with Amyotrophic Lateral Sclerosis

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1 Update: The Care of the Patient with Amyotrophic Lateral Sclerosis Case Presentation: Part I A 54-year-old woman presents to the neurology clinic referred by her primary care physician for evaluation of weakness. She reports that over the past 4 months she has noted some difficulties with walking up stairs. In addition she is having some trouble opening jars and doing activities that require her to raise her hands over her head. The weakness is continuous throughout the day. She is not having any breathing difficulties. She denies any cramping or muscle pain. She has not had any swallowing difficulties. She is not having blurred vision. She has hypertension and diabetes. She has a history of migraines and neck pain. She has had a hysterectomy. She has no known drug allergies. She is taking atenolol and metformin. She drinks a glass of wine each night and smokes one pack of cigarettes a day. She has a family history of heart disease in her mother. On physical exam she is alert and responsive. She is sitting comfortably in the chair. Her vitals are BP 128/80, HR 72, and RR 18, and her temperature is 99. She is alert and oriented to person, place, and situation. She is able to name three objects, and her language is normal. Fund of knowledge is normal. Memory is intact for three words in 5 minutes. Funduscopic examination is normal, with normal optic discs visualized. Cranial nerves were normal except for tongue fasciculations and mild spastic dysarthria. Motor examination is 4/5 in the arms and 4+/5 in the legs bilaterally. There is spasticity in the legs and no drift. Fasciculations are noted in the arms and legs bilaterally. Reflexes are 3/4 symmetrically bilaterally, with toes up-going. Sensory is intact to light touch, pinprick, and vibration bilaterally. Romberg is negative. The coordination is intact to finger to finger and heel to shin. She is able to stand with some effort and walk without assistance. She has difficulties with toe and heel walking. You discuss with the patient that further investigation is needed and that an EMG will help to clarify the diagnosis. You review the MRI images of the brain and cervical spine that the primary care doctor had ordered 2 months prior for her headaches and neck pain. The MRI of the brain was normal, and the cervical spine showed mild degenerative disease only, without neuroforaminal narrowing or cord impingement. Her normal complete blood count, basic metabolic panel, thyroid, and cholesterol testing performed by the primary care doctor one month prior were also reviewed. You provide information to her about the differential diagnosis for these symptoms and answer all of her questions. Greater than 50% of an 80-minute face-to-face visit is spent discussing the differential diagnosis, evaluation, potential treatment, and prognosis of muscle weakness. You arrange follow-up for 4 weeks and advise the patient to return with a family member.

2 ICD-9-CM 1 Discussion At this visit only muscle weakness and fasciculations are documented. It is acceptable to use symptom codes for outpatient visits and also to order testing. Muscle weakness has its own code in ICD-9-CM: Muscle weakness Fasciculations do not have their own code, and is referenced in the index to: 781.0, Abnormal involuntary movements. This is a very nonspecific code and it is probably not necessary to report it for ordering neurophysiologic testing unless it was the only symptom or finding. Selection of the correct Evaluation and Management code is relatively easy in this case since 80 minutes was spent face to face with the patient and more than half of the time was expended discussing issues of counseling and coordination of care such as differential diagnosis, evaluation, potential treatments, clinical trials, and prognosis. Using the time base of the E&M coding process, code would be used for a level 5 outpatient consultation. Case Presentation: Part II The patient returns to the office 4 weeks later. Since the last visit she has had progression of her weakness. She is now walking with a cane for support. She has started to develop mild symptoms of shortness of breath. She is experiencing some mild difficulties with swallowing her pills. She is experiencing more fatigue during the day. She has some pain in her legs related to her stiffness. On physical exam the she is alert and responsive. She is sitting comfortably in the chair. Her vitals are BP 118/70, HR 72, and RR 22, and her temperature is 99. She is alert and oriented to person, place, and situation. She is able to name three objects, and her language is normal. Fund of knowledge is normal. Memory is intact for three words in 5 minutes. Funduscopic examination is normal (with optic discs visualized). Tongue fasciculations are noted, and there is mild tongue weakness. She has a delay in her swallow and mild spastic dysarthria. Sternocleidomastoid is 5/5 bilaterally. Motor examination is 4/5 in the arms and 4/5 in the legs bilaterally. There is spasticity in the legs and no drift. Fasciculations are noted in the arms and legs bilaterally. Reflexes are 3/4 symmetrically bilaterally, with toes up going. Sensory is intact to light touch, pinprick, and vibration bilaterally. Romberg is negative. The coordination is intact to finger to finger. Heel-to-shin testing is difficult secondary to stiffness, but there is no obvious dysmetria. She is able to stand with moderate effort and walks with a cane. She is unable to do toe-and-heel walking. The EMG shows clear evidence of a motor neuron disorder.

3 You discuss the diagnosis of amyotrophic lateral sclerosis (ALS) with the patient and her husband. You answer all of their questions about etiology, prognosis, and treatments and provide handouts and website URLs for further reading. You discuss the initiation of riluzole. She is interested in starting this medication. You also order pulmonary function testing to evaluate her shortness of breath and suggest a visit to a multidisciplinary ALS clinic for another opinion and a full evaluation. You discuss and order a swallow evaluation with a modified barium swallow test to evaluate her dysphagia and risk of aspiration. You address end-of-life issues and ask the patient to discuss this further with her family. You schedule a follow-up for her in 6 to 8 weeks. Greater than 50% of this hour visit was spent discussing evaluation and treatment of ALS. Second Visit First a note regarding coding for the EMG: If the study had been normal, the code would have been that for muscle weakness as above. Since the EMG documented motor neuron disorder, the diagnosis code reported with the EMG would be: Anterior horn cell disease, unspecified There is no unspecified code for motor neuron disease, so this defaults to the unspecified for the parent category of anterior horn cell disease. The visit note documents this patient has ALS. The diagnosis code for this visit would be: Amyotrophic lateral sclerosis The symptom codes for which testing is ordered should also be listed. These would be: Other dysphagia, neurogenic dysphagia Shortness of breath Once again, using the time-based billing process for face-to-face patient care in the outpatient setting, one would choose the code which is a level 5 established patient. To code for using the time-based process, one needs to spend more than 20 minutes of a 40-minute visit. There is no mechanism to code for the additional 20 minutes. Nerve Conduction Studies, Reflex and Late Response Testing (For listing of nerves considered for separate study, see Appendix J in CPT 2009) Nerve conduction, amplitude and latency/velocity study, each nerve; motor, without F-wave study motor, with F-wave study

4 95904 sensory (Report 95900, 95903, and/or only once when multiple sites on the same nerve are stimulated or recorded) H-reflex, amplitude and latency study; record gastrocnemius/soleus muscle record muscle other than gastrocnemius/soleus muscle (To report a bilateral study, use modifier 50) Numbers of Motor and Sensory Nerves Studied With the approval by the AMA CPT Editorial Panel, the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) developed a list of all motor and sensory nerve conduction studies that can be coded as separate procedures. If a procedure is on this list, it could be coded as a separate unit of 95900, or The latest version is included in Appendix J of CPT 2009, and is specifically referenced in the section on nerve conduction studies: (For listing of nerves considered for separate study, see Appendix J). The statement (Report 95900, 95903, and/or only once when multiple sites on the same nerve are stimulated or recorded) has remained in CPT 2009, however. It now serves solely as a reminder that a nerve conduction study assessing different segments of a single nerve cannot be coded as separate units. For example, study of four segments of the right ulnar motor nerve (without F-waves) (1) axilla-above elbow, (2) above-below elbow, (3) below elbow-wrist, and (4) wrist-abductor digiti minimi muscle can only be coded as one unit of Numbers of Studies That Should be Performed A table outlining the recommended numbers of motor and sensory nerve conduction studies that can be used to diagnose 90% of patients with certain common conditions and symptoms is included in Appendix J of CPT CPT Changes 2006: An Insider's View explained the rationale behind this table as follows: The maximum number of studies table summarizes the recommended maximum number of studies per diagnostic category necessary for a physician to arrive at a diagnosis in 90% of patients with that final diagnosis, when performing needle electromyography (EMG) tests ( and ); nerve conduction studies (95900, 95903, and 95904); and other EMG studies (95934, 95936, and 95937). The numbers in the table are to be used as a tool to detect outliers to assist in appropriate reporting. Each number in the table represents one study or unit. The maximum numbers are designed to apply to

5 a diverse range of practice styles as well as practice types, including those at referral centers where more complex testing is frequently necessary. In simple, straightforward cases, fewer tests will be necessary. This is particularly true when results of the most critical tests are normal. In complex tests, the maximum numbers in the table will be insufficient for the physician to arrive at a complete diagnosis. In cases where there are borderline findings, additional tests may be required to determine if the findings are significant. The appropriate number of studies to be performed should be left to the judgment of the physician performing the electrodiagnostic (EDX) evaluation; however, in the small number of cases that require testing in excess of the numbers listed in the table, the physician should be able to provide supplementary documentation to justify the additional testing. Such documentation should explain what other differential diagnostic problems needed to be ruled out in that particular situation. In some patients, multiple diagnoses will be established by EDX testing, and the recommendations listed in the table for a single diagnostic category will not apply. It should be noted that in some situations it is necessary to test an asymptomatic contralateral limb to establish normative values for an individual patient. Normal values based on the general population alone are less sensitive than this approach; therefore, restrictions on contralateral asymptomatic limb testing will reduce the sensitivity of electrodiagnostic tests. Electromyography Needle electromyography procedures include the interpretation of electrical waveforms measured by equipment that produces both visible and audible components of electrical signals recorded from the muscle(s) studied by the needle electrode Needle electromyography; one extremity with or without related paraspinal areas two extremities with or without related paraspinal areas (For dynamic electromyography performed during motion analysis studies, see ) three extremities with or without related paraspinal areas four extremities with or without related paraspinal areas larynx (Do not report modifier 50 in conjunction with 95865) (For unilateral procedure, report modifier 52 in conjunction with 95865) hemidiaphragm

6 95867 cranial nerve supplied muscles, unilateral cranial nerve supplied muscles, bilateral thoracic paraspinal muscles (excluding T1 or T12) limited study of muscles in one extremity or non-limb (axial) muscles (unilateral or bilateral), other than thoracic paraspinal, cranial nerve supplied muscles, or sphincters FAQs - Electromyography ( ) A common question concerns how many muscles should/need to be studied per limb in order to use the limb EMG codes. The proper procedure for Medicare patients has been outlined in the Federal Register (issue of October 31, 1997, Vol. 62, No. 211, page 59090, see below). Proper Use of Needle EMG CPT Codes In order to clarify the proper use of these codes, CMS has formulated the following policies: CPT codes 95860, 95861, 95863, and (Needle electromyography of 1, 2, 3, or 4 limbs with or without related paraspinal areas). To bill these codes, extremity muscles innervated by three nerves (for example, radial, ulnar, median, tibial, peroneal, femoral, not sub-branches) or four spinal levels must be evaluated, with a minimum of five muscles studied per limb. One cannot bill paraspinals separately with these codes - unless studying paraspinals between T3-T11, in which case code is to be used. CPT code (Needle electromyography, thoracic paraspinal muscles).this CPT code should be used when exclusively studying thoracic paraspinal muscles, excluding T1 or T12. One unit can be billed, despite the number of levels studied or whether unilateral or bilateral. This code cannot be billed with CPT codes 95860, 95861, 95863, or if only T1 and/or T2 are studied when an upper extremity was also studied. CPT code (Needle electromyography; other than paraspinal (eg, abdomen, thorax). This CPT code can be billed at one unit per extremity. The code can also be used for muscles on the thorax or abdomen (unilateral or bilateral). One unit may be billed for studying cervical or lumbar paraspinal muscles (unilateral or bilateral), regardless of the number of levels tested. This code should not be billed when the paraspinal muscles corresponding to an extremity are tested and when the extremity codes 95860, 95861, 95863, or are also billed. Principles of CPT Coding, Fifth Edition states: That code may be used more than once. For example, if three muscles are tested in each upper extremity, use code with two units of service, rather than code (page 474).

7 Questions 1. In ALS riluzole has been shown to: A. Slow disease progression B. Provide remission of symptoms C. Halt disease D. Improve survival at 5 years E. Allow patients to avoid intubation The correct answer is A. Riluzole should be offered to slow disease progression (Level A) In ALS, noninvasive ventilation (NIV) should be considered to treat respiratory insufficiency to: A. Decrease fasciculations B. Lengthen survival C. Reverse respiratory problems D. Eliminate the need for percutaneous endoscopic gastrostomy (PEG) E. Provide remission of symptoms The correct answer is B. In ALS, NIV should be considered to treat respiratory insufficiency in order to lengthen survival (Level B), and may be considered to slow the decline of forced vital capacity (Level C) and improve quality of life (Level C) One intervention that may be considered when dysphagia and weight loss occur in ALS to stabilize weight is: A. Nasogastric tube B. Parenteral nutrition C. Early initiation of NIV D. PEG placement E. Early initiation of invasive ventilation The correct answer is D. PEG should be considered to stabilize weight and prolong survival in ALS (Level B). 2 CPT 2009 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association. 1. Centers for Disease Control and Prevention. International classification of diseases, ninth revision, clinical modification (ICD-9-CM) Miller RG, Jackson CE, Kasarskis EJ, et al. Practice Parameter update: The case of the patient with amyotrophic lateral sclerosis: Multi-disciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009;73: The AAN develops these clinical case examples as educational tools for neurologists and other health care practitioners. You may download and retain a single copy for your personal use. Please contact to learn about options for sharing this content beyond your personal use.

8 Disclaimer This statement is provided as an educational service of the American Academy of Neurology. It is based on an assessment of current scientific and clinical information. It is not intended to include all possible proper methods of care for a particular neurologic problem or all legitimate criteria for choosing to use a specific procedure. Neither is it intended to exclude any reasonable alternative methodologies. The AAN recognizes that specific patient care decisions are the prerogative of the patient and the physician caring for the patient, based on all of the circumstances involved American Academy of Neurology

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