Neuromuscular Signs and Symptoms: Muscle stiffness, ptosis, axial muscle weakness 1 W. David Arnold, MD AAPMR 2015 Disclosure: I receive funding through the RMST program (NICHD 5K12HD )

Objectives Use “unknown” cases to: –Highlight clinical signs/symptoms: muscle stiffness Ptosis Axial muscle weakness –Present key history and examination features –Discuss variable presentations of each sign/symptom and the differential

Case 1: “Lifelong muscle stiffness” Case 1 Video

This finding can be described clinically and electrically Clinical video EMG video

“Paradoxical” findings Video

Case 2: Ptosis 56 year old man Progressive ptosis and proximal limb weakness Some symptoms of dysphagia 2 nd opinion: myasthenia gravis? ACHR and MuSK antibodies are negative Poor response to immunomodulatory therapies & pyridostigmine Family history of seronegative myasthenia gravis in mother

Case 2: Pre and post eyelid surgery Image 1 Image 2

Fatigable ptosis and diplopia Image/Video

Before and after treatment

Ptosis, EOM weakness, diabetes, exercise intolerance Image

Case 3: Head Drop/Chin on chest 64 year old man who presents with 2 year history of progressive inability to hold his head upright PMH: hypertension, hyperlipidemia, lymphoma s/p mantle field radiation about 20 years ago, hypothyroidism PSH: aortic valve replacement FH: no history of neuromuscular symptoms Diagnosis? image

Camptocormia/Bent Spine Syndrome Video

Thanks