Stiff Person Syndrome (SPS) Management Plan - for Emergency Departments / Paramedics
 

Loren’s routine SPS treatment regimen:

1. Daily 1.5g clonazepam (0.5 morning, 0.5 lunch/afternoon, 0.5 before bed)

2. Breakthrough 0.25g clonazepam as needed

3. As needed 5mg in 1 mL, used with Intranasal Mucosal Atomization Device. This is
   usually when:

   1. Thoracic, neck, or voice box spasm are impacting her ability to breathe; or

   2. An aggressive, unrelenting spasm in another body part where a breakthrough  clonazepam will be too delayed, causing Loren injury.

 

Note that Loren can only take the Rivotril brand of clonazepam as she is allergic to sulphites
which are in the Paxam brand.

Loren’s respiratory and cystic fibrosis clinic, managed by A/Prof Lucy Burr, has noted that:

1. When the spasm impairs her ability to breathe (such as thoracic, neck, throat, voicebox), intranasal should be the first-line treatment if her nasal passages are clear of congestion.

2. If her nasal passages are not clear (e.g. sinus infection), then absorption is likely to be impaired, and IV midazolam would be the first-line treatment.

3. Second-line treatment would be the alternative if not resolved.

4. Third-line treatment would be per the neuroimmunology team’s recommendations (AWAITING THEIR INPUT - likely valium).

5. It is also important to note that a prior spasm caused:

   1. C5/6 cervical radiculopathy and cervical spinal cord compression and subsequent neurosurgery.

   2. Tracheal stenosis, tracheomalacia, leftward tracheal deviation and oesophageal compression. This exacerbates the risk of spasms within her neck.

6. When to discharge:

   1. If Loren has recovered from any spasm relating to her thoracic region and her ability to breathe and her respiratory status is deemed satisfactory; and

   2. She has returned to oral treatments only;

   3. Then she would be safer at home than in hospital due to the risk of her highly immunocompromised status (which also includes Primary Immunodeficiency).

​

THIS REMAINS IN DRAFT - AWAITING FEEDBACK

Our neuroimmunology team recommends that, if Loren presents to hospital, she be administered in accordance with the above. In addition to that, we make the following comments:

1. Regarding additional treatment options:

   1. If taking 5mg in 1 mL intranasal midazolam and IV Midazolam does not resolve the spasm and/or rigidity, the next course of action would be XXX [AWAITING - likely valium].

   2. Collaboration per below.

 

Regarding collaboration

1. If the treatment options outlined above are not alleviating the symptoms, we request you contact:

   1. Mater Public Neuroimmunology:  Dr Andrew Swayne XX [AWAITING]

   2. Mater Public Respiratory: A/Prof Lucy Burr XX [AWAITING]

   3. Prof Stuart (Albert) Reece, Loren’s GP, on 0424 439 639, noting that Stuart best understands the complexity of Loren’s combination of rare diseases and their interplay.

 

Regarding discharge

1. If Loren has returned to oral treatment only and the key cause of admission has been alleviated, then this is an indicator for discharge; and

2. If Loren has returned to being mobile and the risk of falls has been removed, this is also an indicator of discharge.

3. Note that her SPS is triggered by:

   1. Startle - such as unexpected noise, touch, lights, peripheral movement;

   2. Stress, overwhelm and emotion.

      1. This would be triggered by being in a hospital environment due to the risks of further SPS triggers present in a hospital environment and her immunocompromised status.

      2. It is exacerbated by significant prior hospital trauma from such risks becoming a reality.

   3. Keeping her within a hospital environment longer than necessary is likely to exacerbate and/or trigger new SPS episodes, and she should be considered for discharge.

4. If Loren is at risk of falls, remains on non-oral treatment, or has respiratory implications, then it would be necessary to keep her in hospital and ensure we are engaged for collaboration.

   1. The key to keeping Loren safe is consideration of her immunocompromised state and risk of exposure to infectious conditions, and placing her in an environment that does not trigger her SPS as per above.

5. On discharge, Loren should ensure her Allied Health Team is engaged for intensive physiotherapy and rehabilitation to actively manage her rigidity and mobility.