## Abstract We report a patient with a progressive motor disorder dominated by pyramidal signs in all four extremities and cervical dystonia in the form of torticollis, who had imaging features of cervical cord tumor on magnetic resonance imaging (MRI) scanning. Ependymoma was the final diagnosis b
Propriospinal myoclonus due to cord compression in the absence of myelopathy
β Scribed by David Shprecher; Howard Silberstein; Roger Kurlan
- Publisher
- John Wiley and Sons
- Year
- 2010
- Tongue
- English
- Weight
- 267 KB
- Volume
- 25
- Category
- Article
- ISSN
- 0885-3185
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β¦ Synopsis
Catatonia is a syndrome of motor dysregulation characterized by fluctuating stupor, mutism, negativism, catalepsy, automatic obedience, and stereotypy. 1 Other motor signs include gegenhalten, mitgehen, waxy flexibility, echophenomena, and ambitendency. The diagnosis requires two to four features. 2 The pathophysiology of catatonia remains unclear but may involve dysfunction of frontal circuitry and motor regulatory brain areas. Catatonia is most closely associated with mood disorders but also results from medications (e.g., dopamine blockers and serotonergic agents), metabolic derangement, seizures, or focal structural brain lesions. 2 We present a patient with Parkinson's disease (PD) who developed catatonia during deep brain stimulation (DBS) surgery complicated by intracranial hemorrhage.
The patient was a 60-year-old right-handed woman with levodopa-responsive PD for 12 years. Prior to surgery, she was taking levodopa 800 mg, entacapone 800 mg, and ropinirole 8 mg daily but experienced debilitating motor fluctuations, abrupt ''off'' periods, and dyskinesias, despite adjustments to her medication regimen. She had a history of nonmelancholic depression treated with citalopram 20 mg daily. During presurgical evaluation, she had no dementia on neuropsychological testing or evidence of an active mood disorder. Off-on Unified Parkinson's Disease Rating Scale (UPDRS) testing demonstrated at least a 32% improvement to her standard morning levodopa dose. She was deemed an appropriate DBS candidate and underwent surgical placement of DBS electrodes into each subthalamic nucleus using microelectrode recordings. The surgery was conducted in the off medication state, over 12 hours after her last dose of PD medication. Mild hypertension during the procedure was controlled with low doses of metoprolol.
Before DBS electrode placement, the patient spoke only when asked questions or to complain of intermittent left arm pain. During right electrode placement, she gradually developed dystonic flexion posturing of the left arm and intermittent flexion of the right arm and left leg. When asked questions or given commands, she was mute. She had gegenhalten in both arms and eyelids upon passive manipulation. She had no motor weakness. Other catatonic features included a protruding grimace (schnauzkrampf), bilateral grasp reflexes, and mitgehen in the right arm.
Differential considerations included a worsening of her parkinsonian ''off'' state versus catatonia. Lorazepam 2 mg
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