Jan Latta

Myasthenia gravis, castleman disease, pemphigus, and anti-phospholipid syndrome

Introduction: Myasthenia gravis is an autoimmune disease marked by neuromuscular transmission failure at the neuromuscular junction. Castleman disease is a rare lymphoproliferative disease characterized by non‐cancerous angiofolicular hyperplasia of lymphatic tissue. Methods and results: We describe a young man with rapid, successive manifestations of myasthenia gravis, a solitary form of Castleman disease, pemphigus vulgaris, and anti‐phospholipid syndrome, which resulted in 2 ischemic cerebrovascular events that caused a severe central neurological deficit. Discussion: We were unable to find a similar case in the literature, but we hypothesize that the temporal concidence of these clinical entities may be related to a common immunological pathway, such as B‐cell activation. Therefore, we treated the patient with an immunosuppressant and anticoagulant treatment, as well as rituximab, a monoclonal antibody therapy against CD20+. Muscle Nerve 47:447‐451, 2013

Stiff-person syndrome following tick-borne meningoencephalitis.

Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS.

Ulnar nerve at the elbow - normative nerve conduction study.

Introduction A goal of our work was to perform nerve conduction studies (NCSs) of the ulnar nerve focused on the nerve conduction across the elbow on a sufficiently large cohort of healthy subjects in order to generate reliable reference data. Methods We examined the ulnar nerve in a position with the elbow flexion of 90o from horizontal. Motor response was recorded from the abductor digiti minimi muscle (ADM) and the first dorsal interosseous muscle (FDI). Results In our sample of 227 healthy volunteers we have examined 380 upper arms with the following results: amplitude (Amp)-CMAP(wrist) for ADM 9.6 ± 2.3 mV, MNCV at the forearm 60.4 ± 5.2 m/s, MNCV across the elbow 57.1 ± 5.9 m/s. Discussion Our study showed that motor NCSs of the ulnar nerve above elbow (AE) and below elbow (BE) in a sufficiently large cohort using methodology recommended by AANEM gave results well comparable for registration from FDI and ADM.

Miller Fisher syndrome with presynaptic neuromuscular transmission disorder

Miller Fisher syndrome is defined by a triad of symptoms, namely areflexia, ataxia, and ophthalmoparesis. The ophthalmoparesis is mostly severe, undulating weakness of eye movements with ptosis and increased fatigability resembling a neuromuscular transmission disorder. We present a 52-year-old man with severe Miller Fisher syndrome with a high level of anti-GQ1b antibodies and a presynaptic type of neuromuscular transmission disorder. The diagnosis was confirmed by stimulated single-fiber electromyography with the use of a concentric needle electrode and various stimulation rates.

Occlusion of all cardinal cerebropetal arteries without causing ischemic stroke

A 66-year-old male presented with a sudden severe headache, transient loss of consciousness and persisting vertigo. A subarachnoid hemorrhage was detected via lumbar puncture. The CT scan was negative and an MRI detected a few T2 hyperintense foci of ischemic origin and the suspected subarachnoid hemorrhage. An MRA revealed occlusions of all four magistral cerebral arteries and the presence of an excessively developed collateral circulation, later proven with DSA. The patient did not suffer from focal ischemia despite this severe cerebrovascular disease. At the time of discharge after 30 days of hospitalization, previous mild meningeal signs, cephalea and vertigo were no longer present.