A summary of “Clinical Evaluation of MS Patients” by Dr. Salvatore Sclafani, Interventional Radiologist ISET 2011 January 20, 2011 written by Kristen Cuenca, RNC, MSN, WorldMed Assist

On January 20, 2011, Dr. Sclafani spoke at the ISET conference in Miami Beach. He explained how patients are diagnosed and clinically evaluated with MS. It was an interesting topic as he addressed the crowd of vascular surgeons and endovascular physicians, interventionl radiologists and more. He described MS as a chronic inflammatory disease of unknown etiology. He Currently, 1:700 Americans have MS with 25% of cases in North America. There are 10,000 new cases yearly which are more than patients currently on hemodialysis. Many experience disability, with 50% unable to work within 10 years of diagnosis and 50% are unable to walk.

Dr. Sclafani explained the types of MS starting with Clinically Isolated Syndrome (CIS) defined as one de-myelinating episode lasting 24 hours. The second type he defines is Radiologically Isolated Syndrome (RIS) for which a person has lesions on MRI, but no symptoms as of yet. RIS patients have a high risk of developing MS in the near future, as  he explains later on in his lecture.  A third type is Relapsing-Remitting MS which includes 80% of MS patients world-wide presently. Secondary Progressive MS occurs in 50-60% of RRMS patients and is the fourth type, in which RRMS patients discontinue periods of remittance and change to a steady progression of symptoms. And, finally, 10% of MS patients are diagnosed with Progressive MS from which symptoms have worsened since diagnosis, with no periods of remittance and is the fifth type.

Dr. Sclafani went on to list and describe the most common symptoms of MS for the audience of endovascular surgeons and interventional radiologists. Fatigue was listed as the most disabling, widely experienced symptom affecting 78% of MS patients. Fatigue in MS has no known etiology or cause. Cognitive impairment is the most common complaint of MS patients including attention deficits, short-term memory loss, and conceptual reasoning/problem-solving abilities. 50% of MS patients experience severe depression. Other mood disturbances, including suicide and extreme emotional lability, are noted among MS patients.

Cortico-spinal complaints affect 30-40% of MS patients as the initial attack and are demonstrated as motor deficits. More than 60% have motor disability with legs being greater than arms. Bilateral deficits are more common than unilateral deficits. These complaints include stiff, painful muscles, cramps, spasms, and contractures as well as clonus on medical exam. Ultimately, patients may suffer spastic paraparesis and eventually, quadriparesis, if disease progression is not halted. Somatosensory complaints are described in 65% of MS patients, including excruciating pain, numbness, icy cold, burning, lancing, tearing, and biting pain symptoms. Lhermite’s sign, which includes a sudden electric shock radiating down spine or extremities during neck flexion, is often felt in MS patients. Additionally, MS patients complain of headaches, especially migraines as well as the torso tightness which has come to be known as the “MS hug”.

Visual pathways are also affected with 14-28% of MS patients diagnosed with optic neuritis. Other visual symptoms include: decreased vision, impaired color vision, blinding by lights, visual field cuts, dimming, photophobia, pain on eye movement and blurry, tunnel vision. Many patients are first diagnosed with MS due to visual symptoms.  Brain stem and cerebellar symptoms are present in MS patients including nystagmus, oscillopsia, diplopia and gaze paresis. Occasionally, patients experience facial palsy and craniocervical dyskinesia which is described as torticollis, blepharospasm, and involuntary movements of the tongue, vocal cords, face, and muscles. Additional brain stem symptoms attributed to MS involve the ear such as vertigo, tinnitus, hearing loss, and hyperacusis. Affected balance is demonstrated in gait disturbances in 10% of patients. MS patients may also have an intention tremor or violent ataxia.  

The autonomic nervous system has dysfunction with MS patients. Dr. Sclafani described multiple symptoms which could be attributed to issues with the nervous system, including the inability to sweat as well as tachycardia or bradycardia with sympathetic/parasympathetic imbalance. These disturbances result in decreased vascular tone to the extremities demonstrated as purple, swollen feet. The autonomic nervous system controls temperature regulation which is altered in MS patients with frequent hypothermia as well as intolerance to significant temperature changes. The ANS also affects the urinary system and disturbances cause urinary frequency, urgency, hesistancy and incontinence as well as bowel constipation. Sexual dysfunction including erectile dysfunction and inorgasmia are caused by ANS issues.

After listing the various symptoms common to MS patients, Dr. Sclafani went on to discuss the Expanded Disability Status Scale (EDSS) based on neurologic testing on a scale of 0.0-10.0, 0 being normal and 10 being death from MS. Pyramidal, cerebellar, brain stem, sensory, bowel and bladder, visual and mental areas are assessed in order to give a MS patient an EDSS. The EDSS can be used to determine progression of symptoms or for comparison purposes. Dr. Sclafani explained that decubitus ulcers, pneumonia and urinary tract infections are commonly found among patients with the higher EDSS.

Dr. Sclafani explained that the diagnosis of MS is based on the revised McDonald criteria as applied by the neurologist. These criteria state there must be at least 2 distinctive, separate clinical attacks lasting at least 24 hours separated by at least 30 days. The diagnosis is based on objective clinical signs, not based on symptoms complained of by the patient or patient history. Additional criteria include multiple episodes disseminated over time and space where lesions in brain lead to different neurological explanations. Exclusion of other better explanations for the neurologic deficits, radiologic and diagnostic data add to the clinical diagnosis when data is unclear.

The earliest presentations of possible MS include neurological syndromes of several types. Monofocal presentations include single brain lesions explaining a single symptom or the patient can have multifocal presentations which are described as multiple brain lesions provoking multiple neurologic deficits. These early presentations can also be monophasic/occurring once or multiphasic/relapsing. Progressive symptoms may be present, as well. Differentiating MS from other diseases is especially challenging, as MS is considered a diagnosis of exclusion meaning the diagnosis is made when no other plausible diagnosis can explain the patient’s symptoms.

Clinically Isolated Syndrome (CIS) is described by Dr. Sclafani in this monophasic presentation with underlying inflammatory demyelinating disease. CIS has 5 types of which the first two types are considered highly suggestive of an MS diagnosis.  Monophasic presentations most often appear in the optic nerve, brain stem, spinal cord, or brain hemispheres. CIS includes lesion on MRI without symptoms as well as symptoms with normal MRI. Radiologically Isolated Syndrome (RIS) is included in this category too which is defined by the presence of multifocal lesions on MRI but no symptoms as of yet.

Dr. Sclafani added that there are many presentations of MS-like symptoms which were too numerous to list. There are many non-demyelinating inflammatory conditions such as: vasculitis, infectious, neoplastic, congenital and metabolic. Any of these various diagnoses can present with manifestations comparable to MS symptoms. In addition, Dr. Sclafani lists other idiopathic inflammatory diseases which  mimic MS such as: CIS, Neuromyelitis Optica, Opticospinal MS in Asians, Acute Disseminated Encephalomyelitis as well as a few very unusual syndromes.

According to Miller et al (2008, Journal of MS position paper), 79 “red flag” symptoms are highly suggestive of MS including demographic, clinical, laboratory, and MRI findings which form the basis for the neurologist to differentiate. Miller et al describes 36 “major” symptoms which strongly point to a non-MS diagnosis as well as 32 intermediate symptoms and 11 minor symptoms. An algorithm is used to lead the clinician to a possible diagnosis of MS, although for many patients, a clear-cut diagnosis is not possible for many years.

Thanks to Dr. Sclafani, many physicians present at the ISET conference obtained a clearer perspective of the intricacies which encompass MS and its diagnosis. This was an important step in the process of intertwining two of the disciplines in medicine which are very important to CCSVI diagnosis and treatment: vascular and neurologic medicine. Dr. Sclafani’s vast expertise in the area of MS was useful in clarifying the presence of symptoms often NOT attributed to MS, however, present nonetheless in MS patients.

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