Patient case (clinical description): A 47-year-old man comes to the clinic for the follow up of his neuromuscular disease. He experienced gradual, progressive weakness of the left upper extremity over the last year.  Over the last few months, he has also noticed weakness in the right upper extremity.  BP is 120/75, PR is 80 and temperature is 37 C. Reflexes are brisk in the upper extremities, and the plantar responses are extensor.  Mild gait ataxia is present. The patient is under treatment of Riluzole 50 mg BID with the diagnosis of ALS.

Patient's own account: I've been suffering from a neuromuscular disease for a while now, and I went to my doctor's office. I'm now a 47-year-old man and over the past year I experienced a progressive and gradual weakness of my left upper extremity, and over the past month, I also noticed a weakness over my right upper extremity. My heart rate was 120/75, and my  PR was 80 with 37°C for temperature. My reflexes are not good in my upper extremities, and I have trouble with my balance. I'm also under Exservan 50 mg for my sclerosis.

Clinical trial: A Registry-Based Clinical Trial of Pimozide in Patients With Neuromuscular Junction Transmission Dysfunction Due to ALS

Eligibility criteria:
Inclusion Criteria:

          1. Patients classified as having clinically definite, clinically probable, or clinically
             probable (laboratory-supported) ALS according to the El-Escorial diagnostic criteria
             for ALS

          2. Evidence of decremental response greater or equal to 5.0% in at least one nerve-muscle
             pair at the initial screening visit

          3. Age 18 years or greater

          4. Consent to participate in the Canadian Neuromuscular Disease Registry (CNDR)
             (follow-up study component only).

        Exclusion Criteria:

          1. Diagnosis of clinically possible or clinically suspected ALS as defined by the
             El-Escorial diagnostic criteria for ALS

          2. If the subject is taking riluzole the dose must be stable for 30 days prior to
             randomization visit. Riluzole cannot be initiated during the study.

          3. History of Parkinson's disease

          4. History of traumatic brain injury

          5. History of neuroleptic malignant syndrome

          6. History of hypersensitivity or serious adverse reaction(s) to a neuroleptic medication

          7. History of prolonged QTc interval > 500 ms

          8. History of hyponatremia < 130 mmol/L

          9. History of current heparin or warfarin use

         10. History of hepatic and/or renal impairment that may affect pimozide metabolism

         11. History of current pregnancy or breastfeeding

         12. Current antipsychotic use

         13. Presence of central nervous system depression, comatose states, liver disorders, renal
             insufficiency, and blood dyscrasias

         14. Presence of depressive disorders or Parkinson's syndrome

         15. History of congenital long QT syndrome or with a family history of this syndrome and
             in patients with a history of cardiac arrhythmias or Torsade de Pointes

         16. Presence of acquired long QT interval, such as associated with concomitant use of
             drugs known to prolong the QT interval

         17. Presence of hypokalemia or hypomagnesemia

         18. Presence of clinically significant bradycardia (heart rate < 50 beats per minute)

         19. The concomitant use of CYP 3A4-inhibiting drugs such as azole antimycotics, antiviral
             protease inhibitors, macrolide antibiotics and nefazodone

         20. The concomitant use of CYP 2D6-inhibiting drugs such as quinidine is also
             contraindicated

         21. Concomitant use of serotonin reuptake inhibitors, such as, sertraline, paroxetine,
             citalopram and escitalopram

         22. Severe dysphagia with risk of aspiration

         23. Has taken any compound under current or known future study as a potential therapy for
             ALS less than 30 days prior to dosing OR history of exposure to stem cell therapy for
             treatment of ALS at any time
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 18 Years old.


Eligibility (NLI label): Entailment