7/28/2013

Susan Perlman, MD Symptomatic Medication For PPS

By: Susan Perlman, M.D.
University of California, Los Angeles (UCLA)

Susan Perlman, M.D. is Associate Clinical Professor of Post-Polio Clinic at UCLA. Since 1988, the clinic has evaluated and treated 600 polio survivors, with an approach combining neurological assessment, neurorehabilitation techniques, medication, intervention and consultation with associates in orthopedics, medicine, sleep disorders, psychology and alternative (complementary) medicine. The clinic coordinates the dedicated support groups in Southern California and offers educational outreach to the health care community
POST-POLIO SYNDROME is a constellation of new symptoms (fatigue, weakness, pain, cold intolerance, muscle atrophy, or new problems with activities of daily living), occurring in survivors of definitively (by history, exam or electrical studies) proven acute poliomyelitis, after a period of at least 15 years of stable recovery and performance, and in the absence of any other medical or neurological condition. It is felt to result from the weakening and loss of previously recovered lower motor neuron connections to muscle, possibly due to aging, greater fragility of the recovered nerves, or immune system dysregulation. Onset can be insidious, progression is usually slow, and treatment is most successful with rehabilitation strategies.
            - Susan Perlman, MD
Until we better understand the causes of post-polio syndrome, we will have no curative medication. At best, we can use medication to treat the symptoms and to improve the quality of life, and we can avoid using medication that could make the symptoms worse. Certain other diseases (elevated blood cholesterol levels, high blood pressure, heart disease, and cancers) require use of medications with side effects that can exacerbate symptoms of post-polio syndrome. These should be used, but with careful monitoring of the polio survivor's functioning.
Symptomatic Medication
The three primary symptoms [of PPS] that we can treat with medication are weakness of muscle, fatigue (individual muscle and generalized) and pain, i.e., post-polio pain, overuse pain, bio-mechanical pain and bone pain ( Gawne, AC, 1995). 

Drugs to reverse muscular atrophy or to improve strength by stimulating motor nerve endings to reconnect with muscle fibers (nerve growth factors) are all still experimental. They are currently being tested for use with other motor nerve diseases. Only insulin-like growth factor type 1 (IGF-1), also known as myotrophin or somatomedin-C, has been tested in people with post-polio.
syndrome (Miller, RG, 1997) (see chart) It brought no change in strength or fatiguability but did improve recovery from fatigue after exercise.
Human Growth hormone has been given to increase a person's natural level of IGF-1 but showed little or no improvement in strength (Gupta, KI, 1994).
Another approach has been to develop and test drugs that would protect the nerve-muscle connection from the new damage in the first place (neuro-protective agents). Again, several have been studied in other diseases, but only selegiline has been tested in post-polio syndrome, bringing some improvement in symptoms but no clear stabilization of the disease (Bamford, CR, 1993). Although many people use over-the-counter antioxidant preparations of various types, these have never been formally tested to prove any ability to slow down the changes of post-polio syndrome.
Anabolic steroids, often used by body builders to improve muscle bulk and power, have been tried by polio survivors and other persons with neuromuscular diseases, but The Medical Letter on Drugs and Therapeutics reports the side effects (risk of prostate cancer in men, masculinization in women) greatly outweigh the potential benefits.

Metabolic stimulants (L-carnitine*, L-acylcarnitine, co-enzyme Q), used to improve the ability of muscle to make energy and possibly reduce fatigue and improve strength, have also been tried by polio survivors, but have been associated with rare allergic reactions and insomnia (Lehmann, C, 1994; Nibbett, J, 1996). 
* A placebo-controlled study, as yet unpublished, recently done in Germany showed no significant difference between placebo and L-carnitine.
Specific anti-fatigue drugs can act either in the brain itself (on pathways controlled by dopamine and nonadrenaline) or by improving communication at the nerve-muscle connection. These are, respectively, central and peripheral agents. Centrally acting anti-fatigue medications include amantadine [Symmetrel], bromocriptine [Parlodel], selegiline [l-deprenyl], pemoline[Cylert], ephedrine and certain antidepressants (selective serotonin re-uptake inhibitors [Prozac (fluotoxine), Seroxat (paroxetine), Zoloft, Paxil, Effexor, etc.], which may also have nonadrenaline activity). All have been tested in other fatiguing neurologic illnesses but only the first three have been studied in post-polio syndrome. Amantadine provided no reduction in fatigue (Stein, DP, 1995), but bromocriptine (Bruno, RL, 1996) and selegiline (Bamford, DR, 1993) did. Several studies have been done using pyridostigmine, [Mestinon] a peripherally-acting drug, (Trojan, DA, 19931995Seizert, BP, 1994; Trojan DA, 1997 *) that reflected variable effects on fatigue, possible mild improvement in strength in very weak muscles and notable side effects (primarily gastrointestinal).  Read about this article 
CODIGO G "14" El pasado mes de febrero de 2009 y como resultado de la reunión anual del Comité de Revisión y Actualización de la Organización Mundial de la Salud, (OMS) que tuvo lugar en Delhi, durante el mes de octubre de 2008, la Clasificación Internacional de Enfermedades, en su versión 10 (ICD-10) ha adjudicado un lugar específico al Síndrome Post-Polio (SPP) clasificándolo bajo el código "G14" y excluyéndolo del código B91 (Secuelas de poliomielitis), en el que antes ese organismo lo consideraba abarcado Más informes

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Erradicación de La poliomielitis

Polio Tricisilla Adaptada

March Of Dimes Polio History

Dr. Bruno

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A 41-year-old man developed an acute illness at the age of 9 months during which, following a viral illness with headache, he developed severe weakness and wasting of the limbs of the left side. After several months he began to recover, such that he was able to walk at the age of 2 years and later was able to run, although he was never very good at sports. He had stable function until the age of 18 when he began to notice greater than usual difficulty lifting heavy objects. By the age of 25 he was noticing progressive difficulty walking due to weakness of both legs, and he noticed that the right calf had become larger. The symptoms became more noticeable over the course of the next 10 years and ultimately both upper as well as both lower limbs had become noticeably weaker.

On examination there was wasting of the muscles of upper and lower limbs on the left, and massively hypertrophied gastrocnemius, soleus and tensor fascia late on the right. The calf circumference on the right exceeded that on the left by 10 cm (figure1). The right shoulder girdle, triceps, thenar eminence and small muscles of the hand were wasted and there was winging of both scapulae. The right quadriceps was also wasted. The wasted muscles were also weak but the hypertrophied right ankle plantar flexors had normal power. The tendon reflexes were absent in the lower limbs and present in the upper limbs, although the right triceps was reduced. The remainder of the examination was normal.

Figure 1

The patient's legs, showing massive enlargement of the right calf and wasting on the left

Questions

1
What is that nature of the acute illness in infancy?
2
What is the nature of the subsequent deterioration?
3
What investigations should be performed?
4
What is the differential diagnosis of the cause of the progressive calf hypertrophy?

Answers

QUESTION 1

An acute paralytic illness which follows symptoms of a viral infection with or without signs of meningitis is typical of poliomyelitis. Usually caused by one of the three polio viruses, it may also occur following vaccination and following infections with other enteroviruses.1 Other disorders which would cause a similar syndrome but with upper motor neurone signs would include acute vascular lesions, meningoencephalitis and acute disseminated encephalomyelitis.

QUESTION 2

A progressive functional deterioration many years after paralytic poliomyelitis is well known, although its pathogenesis is not fully understood.2 It is a diagnosis of exclusion; a careful search for alternative causes, for example, orthopaedic deformities such as osteoarthritis or worsening scoliosis, superimposed neurological disorders such as entrapment neuropathies or coincidental muscle disease or neuropathy, and general medical causes such as respiratory complications and endocrinopathies.3

QUESTION 3

Investigations revealed normal blood count and erythrocyte sedimentation rate and normal biochemistry apart from a raised creatine kinase at 330 IU/l (normal range 60–120 IU/l), which is commonly seen in cases of ongoing denervation. Electromyography showed evidence of denervation in the right APB and FDI with polyphasic motor units and complex repetitive discharges, no spontaneous activity in the left calf and large polyphasic units in the right calf consistent with chronic partial denervation. Motor and sensory conduction velocities were normal. A lumbar myelogram was normal. Magnetic resonance imaging (MRI) scan of the calves is shown in figure2.

Figure 2

Axial T1 weighted MRI scan (TR 588 ms, TE 15 ms) of the calves, showing gross muscle atrophy and replacement by adipose tissue on the left, and hypertrophy of the muscles on the right, with only minor adipose tissue deposition

QUESTION 4

The differential diagnosis of the progressive calf hypertrophy is given in the box.

Causes of calf muscle hypertrophy

Chronic partial denervation

  • radiculopathy

  • peripheral neuropathy

  • hereditary motor and sensory neuropathy

  • spinal muscular atrophy

  • following paralytic poliomyelitis

    Neuromyotonia and myokymia

  • Isaac's syndrome

  • generalised myokymia

  • neurotonia

  • continuous muscle fibre activity due to: chronic inflammatory demyelinating polyradiculopathy, Guillain Barre syndrome, myasthenia gravis, thymoma, thyrotoxicosis, thyroiditis

    Muscular dystrophies

    Myositis

    Infiltration

  • tumours

  • amyloidosis

  • cysticercosis

    Link here