1. In this case report, a patient with multiple-system atrophy was alleviated from orthostatic hypotension symptoms upon installation of an implantable system.

2. The implantable system features epidural electrical stimulation to the thoracic spinal cord in response to detected body position via accelerometers.

Evidence Rating Level: 3 (Average)

Study Rundown: Orthostatic hypotension is a hallmark symptom in multiple-system atrophy, predisposing these patients to syncopal episodes when the body is upright. Given that these symptoms are caused by degeneration of neurons within the thoracic spinal cord, the research group developed an implantable neuroprosthesis that pulse stimulated the thoracic spinal cord in response to upright body position detected by three-axis accelerometers. The case report follows a 48-year-old woman with multiple-system atrophy of the parkinsonian type who was unable to walk for more than a few minutes due to orthostatic hypotension. Prescribed levodopa-benserazide dosages were increasingly adjusted to accommodate for worsening symptoms until she was eventually bedridden being unable to stand for longer than 60 seconds without a syncopal episode. The implantable system was installed by surgery, then orthostatic hypotension was evaluated over 7 days by tilt-table test. With the system, the patient was able to maintain a slower decline in systolic blood pressure over 10 minutes in an upright position. After 1 month of neurorehabilitation, the patient was able to decrease the number of syncope episodes per day and increase her walking distance. These measures were further improved after 3 months. This case provides support for the use of a novel implantable device to overcome orthostatic hypotension in patients with multiple system atrophy. However, these results are limited by the unknown long-term effects of the device and the unknown contribution from previous rehabilitation attempts prior to the device installation.

Click to read the study in NEJM 

Relevant Reading: Neuroprosthetic baroreflex controls haemodynamics after spinal cord injury

In-Depth [case series]: In this case report, a 48-year-old woman with a 4-year history of multiple-system atrophy of the parkinsonian type had a history of gradually worsening orthostatic hypotension with symptoms of inability to stand or walk short distances without presyncope symptoms. Pharmacological therapy by levodopa-benserazide was unable to slow the progression of the disease, which eventually rendered her bedridden given her predisposition to syncope. The preoperative assessment found a drop of systolic blood pressure from 120 mmHg to less than 50 mmHg within a minute upon sitting upright by the tilt-table test. The patient underwent a surgical procedure to install an implantable system for spinal cord stimulation to minimize depression of sympathetic nervous system activity which was activated by a three-axis accelerometer system to detect upright body positions. Tilt-table tests performed after surgical implantation found that without power, the patient’s systolic blood pressure dropped from 160 mmHg to 75 mmHg over 3 minutes, but with the system turned on, this decrease was only observed over 10 minutes. Combined with autonomic neurorehabilitation for the next 3 weeks, the patient was able to maintain a systolic blood pressure of at least 80 mmHg with no presyncope symptoms 10 minutes after sitting upright. Accordingly, 3 months after implantation, the patient experienced no syncope when she was in an upright position, going from 14 syncope episodes per week before implantation to almost 0. The patient was also able to increase her walking distance from 0 to over 250 meters. Taken altogether, this case report demonstrates the first evidence of using an implantable system to alleviate orthostatic hypotension in a patient with multiple-system atrophy.

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