In early 2013 my husband noted a twitch in his right/dominant hand ring finger while working on his computer when the right mouse button would click unintentionally. This became a little more frequent and seemed to quickly develop into a unilateral and then bilateral tremor. We went to see the GP and said it was likely essential tremor. We weren't satisfied so we went on to see a neurologist. He said concurred and gave a prescription of propanolol when needed. Two days after this appointment, my husband and I played table tennis one night and the following morning he woke up complaining of excruciating pain in a diamond-shaped pattern on his upper arm/shoulder area and similar shaped pain on the top muscle of his forearm (below the elbow). He took pain medication for a few days and then finally after seeing no relief, he went back to the GP. He prescribed PT 3x/week for 3 weeks. During the second week of PT, his pain completely went away and he started experiencing muscle weakness and atrophy in his thumb and his wrist started to drop. The GP ordered a MRI. MRI showed bulging disc at C5-C6 and recommended follow-up with neurologist. Went back to the neurologist and showed him the MRI. He did EMG and NCS. EMG noted moderate ulnar neuropathy and R C5 radiculopathy. He then referred to a neurosurgeon/specialist. The neurosurgeon gave no credence to the EMG report, reviewed the MRI and did a physical exam and said there's something else going on and that "the findings didn't add up." He recommended going to a neuromuscular specialist to get a comprehensive upper extremity EMG. Went to see the neuromuscular specialist who started off doing NCS and then did EMG of all extremities. He reported widespread neurogenic disorder preferentially affecting motor fibers. We were then referred to see a sub-specialist. After consulting with a specialist at both George Washington University Hospital and Johns Hopkins, we've happily landed our decision with the terrific doctor at Hopkins in January of this year.
After an exhaustive consultation, EMG/NCS, spinal tap, countless blood tests, MRIs and further consult with hematologist (@ Hopkins), all signs are leading to MMN. In March, my husband had his loading dose of IVIg 60g/day for 5 days. We believe he developed hemolytic anemia after this treatment but due to timing of blood tests could not confirm it. He was then started on a 2-day infusion every 3 weeks of 75g/day in early May. He's now completed 3 mos of that treatment and the doctor is now changing his frequency to every 2 weeks.
With regards to his symptoms - some historical I'm adding in as I'm not sure they are related or not. In 1997 on a long international trip, my 6'7"/325 lb husband said he experienced burning/tingling in the upper/side (lower hip) part of his right thigh as a result of feeling "tight" in the coach seats on an 18 hour journey. That burning/tingling eventually turned into a numbness. In 2006 my husband was diagnosed with achalasia (i.e., constant constriction of his lower esophageal sphincter) and had a Heller myotomy with partial fundoplication. Due to the achalasia, he has an amotile eosphagus. Around the same time he was diagnosed with plantar fasciitis. To summarize his current symptoms, he experiences some tremors (more notably in fine motor activities), has had wrist drop in the right wrist, all fingers in the right hand have dropped and is now experiencing a finger drop in his first index finger on his left hand and marked weakness in his left wrist. He sweats profusely from activity, but most notably from just standing. He feels he is unable to find his center of gravity when he's standing and he feels that his quadriceps feel like they don't want to work when he stands in place. He's OK with movement although stamina and endurance then play a factor. After the IVIg treatments, he noticed a marked improvement in what was previously diagnosed as plantar fasciitis, because he can now put his heels on the ground where before he would avoid heel to ground contact and put his weight on the balls of his feet. He reports stiffness in his neck in the mornings but attributes that to the pillow situation he has as he has to sleep at an angle due to his achalasia.
His 3 mos assessments from PT/OT show marked improvement in his abilities and strength although the strength testing that his doctor just did show no change in the right hand/arm and a marked weakness in left wrist (this was also noted by PT/OT). My husband says he feels an overall sense of well-being after IVIg treatments and feels that improvements are at a "galatial pace".
Of note, his co-GM1 autoantibody values were off the charts elevated, he was also found to have IgM monoclonal gammopathy of undetermined significance (MGUS) and he doesn't show conduction block except in one location.
From all my learnings online about this disease, I understand that the dose/response of IVIg is tailored specifically to each patient. Perhaps we're very early in the journey, but would appreciate anyone's insights. I tremendously admire the amount of education I have seen patients have about the disease and would be indebted to you in getting myself better educated. Thanks in advance.