7/28/2013

Abnormal Movements in Sleep as a Post-Polio Sequelae


                                                         By: Richard L. Bruno, 

Published in: American Journal of Physical Medicine and Rehabilitation, 1998; 77: 1-6.
ABSTRACT
Nearly two-thirds of polio survivors report abnormal movements in sleep (AMS), with 52% reporting that their sleep is disturbed by AMS. Sleep studies were performed in seven polio survivors to objectively document AMS. Two patients demonstrated Generalized Random Myoclonus (GRM), brief contractions and even ballistic movements of the arms and legs, slow repeated grasping movements of the hands, slow flexion of the arms and contraction of the shoulder and pectoral muscles. Two other patients demonstrated Periodic Movements in Sleep (PMS) with muscle contractions and ballistic movements of the legs, two had PMS plus Restless Leg Syndrome (RLS) and one had sleep starts involving only contraction of the arm muscles. AMS occurred in Stage II sleep in all patients, in Stage I in some, and could significantly disturb sleep architecture even though patients were totally unaware of muscle contractions. Poliovirus-induced damage to the spinal cord and brain is presented as a possible cause of AMS. The diagnosis of post-polio fatigue, evaluation AMS and management of AMS using benzodiazepines or dopamimetic agents is described.
Despite numerous late-onset symptoms reported by polio survivors -- fatigue, muscle weakness, pain, cold intolerance, swallowing and breathing difficulties -- one symptom was totally unexpected: abnormal movements in sleep (AMS). As early as 1984 our post-polio patients were reporting muscle contractions as they fell asleep. The 1985 National Post Polio Survey included two questions about AMS: "Do your muscles twitch or jump as you fall asleep?" and "Is you sleep disturbed by muscle twitching?" (1) It was surprising that 63% of the 676 respondents reported that their muscles did twitch and jump during sleep and that 52% -- a third of the entire sample -- said that their sleep was disturbed by twitching.
These percentages are markedly elevated as compared to the incidence of AMS in the general population. In one survey only 29% of those without neurological disease who were at least 50 years old reported AMS, versus 63% of surveyed polio survivors who were 52 years old on average. (2) In another survey only 34% of those older than 64 reported AMS, slightly more than half the incidence of AMS in the younger post-polio sample. (3) Given the apparent increased prevalence of AMS in polio survivors, and with daytime fatigue the most commonly reported Post-Polio Sequelae (PPS), we were interested in objectively documenting AMS, relating them to possible disturbances in sleep architecture and identifying an effective treatment for AMS. (1)

METHODS
Subjects. Seven polio survivors were referred for sleep studies to a sleep disorders center. This was a sample of convenience, in that the subjects were patients presenting with PPS who themselves knew (three patients) or whose bed mates knew (four patients) that AMS were occurring. Patients were on average 54 years old and 44 years post acute polio which occurred at age 10. The patients had had AMS for a mean of eight years which was on average 35 years post acute polio. Patients reported moderate-to-severe difficulty sleeping at night and moderate-to-severe daytime fatigue that did not respond to the treatments of choice for post-polio fatigue (i.e., pacing of activities, daytime rest periods, energy conservation and use of appropriate assistive devices. (4) In addition to fatigue, patients reported an average of two limbs having late-onset muscle weakness.
Procedure. Patients underwent a standard polysomnographic evaluation with EEG and facial EMG recorded for sleep staging. (5) Blood oxygen saturation, measured using a finger pulse oxymeter, chest and abdominal wall excursion and nasal air temperature were also recorded; video monitoring of sleep was also performed. Surface EMG was recorded from patients' legs as well as from limbs in which AMS were reported.

RESULTS Read more
México a la vanguardia en el Síndrome de Post Polio

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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

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