[Severe form of hereditary neuralgic amyotrophy without SEPT9 gene mutation]. / Névralgie amyotrophiante héréditaire à forme sévère sans mutation du gène SEPT9.

INTRODUCTION: Hereditary neuralgic amyotrophy (HNA) is a rare condition characterized by recurrent episodes of painful paralysis preferentially affecting the brachial plexus. It is often linked to a mutation in the SEPT9 gene. CASE REPORT: A 69-year-old female patient experienced a dozen episodes of severe neurological deficit mainly affecting the brachial plexus and the phrenic and recurrent nerves. The diagnosis of HNA without SEPT9 gene mutation was retained. DISCUSSION: HNA can have significant sequelae. A genetic heterogeneity exists and mutations in the SEPT9 gene may not be found. Immunomodulatory and corticosteroid treatments have sometimes proved to be effective.

[Suprascapular nerve entrapment at the spinoglenoid notch]. / Syndrome canalaire du nerf sus-scapulaire dans le défilé spino-glénoïdien.

The suprascapular nerve passes through the spinoglenoid notch with a risk of entrapment. This results in distal nerve lesion characterized by isolated paralysis of the infraspinatus muscle and, most often, by shoulder pain. We report 7 clinical and electromyographical cases of pure infraspinatus muscle paralysis. The value of the electrodiagnosis, which demonstrated prolonged suprascapular distal nerve latencies (over 5 milliseconds) in the infraspinatus muscle affected while latencies were normal in the supraspinatus muscle, is emphasized. Mechanical factors were associated with paralysis in 5 cases. Compressive synovial cysts were found in 2 patients operated upon. Surgical enlargement of the spinoglenoid notch regularly and rapidly relieves pain and sometimes helps in recovery of the infraspinatus muscle.