Figure 1
Neuroimaging in ODS. (A) Axial T2-weighted brain MRI of the patient showing hyperintensity of the central pons (solid arrow) and (B) Axial T1-weighted brain MRI of the patient showing hypointensity of the central pons (dotted arrow).
Figure 2
Neuroimaging in ODS. Axial T1-weighted brain MRI of the patient showing hypointensities of the bilateral lentiform nuclei (red arrows).
Video 1
Before initiation of pramipexole (only on carbidopa-levodopa). Risus sardonicus with significant dysarthria, bradykinetic movement (low amplitude on finger tapping) and dystonia involving the hands are observed.
Video 2
On pramipexole 3 mg/day. Significant improvement of dysarthria and risus sardonicus with greater facial expressivity are observed.
Video 3
Upon discontinuation of pramipexole. Rotigotine was added as limited benefit with increase in other medications including levodopa, trihexyphenidyl and amantadine. Patient without noticeable improvement of dysarthria with rotigotine. Rotigotine was subsequently stopped and pramipexole was restarted and titrated up slowly on 12/2015 to 1.5 mg per day. Patient experienced improvement of dysarthria subsequently.
Table 1
Review of cases of ODS presenting with extrapyramidal symptoms.
| Study | Year | Case | Approximate time of symptom onset | Prominent clinical signs | Treatment | Outcome |
|---|---|---|---|---|---|---|
| Halimet al. [12] | 2018 | Case 1 | 4 days after presentation | Rigidity, bradykinesia, tremor, impairment of horizontal and vertical gaze | Levodopa/benserazide, trihexyphenidyl, IVIG*, dexamethasone | Improvement of symptoms |
| Rizviet al. [13] | 2012 | Case 1 | 4 days after presentation | Tremor, gait difficulty, bradykinesia, dysarthria, cogwheel rigidity | Levodopa-carbidopa | Improvement of symptoms |
| Imamet al. [14] | 2012 | Case 1 | 9 days after presentation | Bradykinesia, dysphagia, hypomimia, rigidity, spasticity | Levodopa-carbidopa | Moderate improvement of symptoms |
| Kwonet al. [15] | 2011 | Case 1 | 5 days after presentation | Rigidity, tremor, bradykinesia, postural instability, hypophonia, pseudobulbar palsy, autonomic dysfunction | Levodopa, anticholinergic medications, benzodiazepine | No improvement in symptoms (death from cardiac arrest 10 days after presentation) |
| Toftet al. [16] | 2011 | Case 1 | 28 days after presentation | Dysarthria, bradykinesia, ataxia, gait instability, dystonia | None | Persistence of symptoms |
| Case 1 | 6 years after presentation | Tremor, cogwheel rigidity after 6 years | Levodopa, apomorphine, ropinirole | Improvement of symptoms except for mild bradykinesia of left side | ||
| Postet al. [17] | 2009 | Case 1 | 15 days after presentation | Dysarthria, bradykinesia, masked face, hypophonia, cogwheel ridigity | None | Spontaneous recovery but with mild masked face |
| Sajithet al. [18] | 2006 | Case 1 | 1 week after presentation | Dysarthria, ataxia, cogwheel rigidity, tremor, decreased oro-bucco-lingual movements | Levodopa-carbidopa, baclofen | Improvement of symptoms |
| Seahet al. [19] | 2002 | Case 1 | 9 days after presentation | Tremor, rigidity, myoclonic jerks | Supportive care | Slowly resolved except for spasticity and rigidity |
| Case 1 | 1 month after presentation | Tremor, bradykinesia, rigidity, gait instability, dystonia, dyskinesia, choreoathetosis | Levodopa/bensarazide, trihexyphenidyl, baclofen | No improvement of movement disorder symptoms even after >1 year of follow up, with severe rigidity, bradykinesia and generalized dystonia | ||
| Kim et al. [20] | 2003 | Case 1 | 2 weeks after correction of hyponatremia | Masked face, dysarthria, bradykinesia, tremor, cogwheel rigidity, gait ataxia | Levodopa-carbidopa | Improvement of symptoms |
| Nagamitsuet al. [21] | 1999 | Case 1 | 2 weeks after correction of hyponatremia | Masked face, bradykinesia, dysarthria, gait disturbance, dysphagia, sialism, glossal palsy, tremor, cogwheel rigidity | Levodopa-carbidopa, amantadine | No initial improvement with amantadine but with marked improvement with levodopa |
| Schwarzet al. [22] | 1998 | Case 1 | 6 days after presentation | Bradykinesia, cogwheel rigidity, facial hypomimia, monotonous speech, parkinsonian gait with retropulsion | Levodopa/benserazide | Initial resolution of parkinsonian signs with levodopa/benserazide but with return of mentioned symptoms |
| Case 1 | 4 months after presentation | Dystonia, dysphagia, spasmodic dysphonia | Tiapride, perphenazine | Improvement of dysphagia, retrocollis, oromandibular dystonia but persistent focal dystonia of the right arm and spasmodic dysphonia even after 20 months | ||
| Maraganore et al. [23] | 1992 | Case 1 | 5 months after presentation | Dystonia, dysarthria, adductor spastic dysphonia | Trihexyphenidyl, baclofen | No improvement in symptoms, exhibited movement disorder symptoms for at least 1 year |
| Case 2 | 6 months after presentation | Tremor, forward stooped gait, dysarthria, decreased arm swing, supranuclear left horizontal conjugate gaze palsy, pseudobulbar affect | None | No improvement in symptoms, exhibited movement disorder symptoms for at least 1 year | ||
| Case 3 | 5 weeks after presentation | Dystonia, masked face | None | Dystonia worsened over the next month, lost to follow up | ||
| Tisonet al. [24] | 1991 | Case 1 | 41 days after admission | Tremor, cogwheel rigidity, gait difficulty, dystonia with choreoathetosis | Trihexyphenidyl, tiapride | Improvement of symptoms |
[i] * IVIG: Intravenous immunoglobulin.