Patients presenting with multiple cranial neuropathies are not uncommon in neurologic clinical practice. The evaluation of these patients can often be overwhelming due to the vast and complicated etiologies as well as the potential for devastating neurologic outcomes. Dysfunction of the cranial nerves can occur anywhere in their course from intrinsic brainstem dysfunction to their peripheral courses. The focus of this review will be on the extramedullary causes of multiple cranial neuropathies as discussion of the brainstem syndromes is more relevant when considering intrinsic disorders of the brainstem. The goals are to provide the reader with an overview of those extramedullary conditions that have a predilection for causing multiple cranial nerve palsies. In turn, this will serve to provide a practical and systematic approach to allow for a more targeted diagnostic evaluation of this, often cumbersome, presentation.
A 23‐year‐old, previously healthy, deployed U.S. soldier presented with bilateral temporal lobe seizures recalcitrant to multiple antiepileptic drugs and anti‐seizure anaesthetic agents. He received methylprednisolone, intravenous immunoglobulins, plasma exchange, and rituximab for presumed autoimmune encephalitis before achieving seizure freedom. Six weeks after presentation, the aetiology of his refractory seizures was found to be due to autoantibodies targeting the anti‐GABA(B)‐receptor. This case is noteworthy for being the first reported case of anti‐GABA(B)‐receptor limbic encephalitis presenting with new‐onset refractory status epilepticus (NORSE), a clinical syndrome that often carries a grave prognosis and in which a treatable aetiology is often never discovered. Our case also supports testing for GABA‐receptor autoantibodies and the upfront use of multi‐modal immunotherapy in patients presenting with limbic encephalitis and new refractory seizures.
Ulnar neuropathy at the elbow (UNE) is the second most frequent compression neuropathy. While other diagnostic imaging tools are emerging to assist in the diagnosis of UNE, electrodiagnosis remains the gold standard. However, the electrodiagnostic approach to UNE presents unique challenges limiting its diagnostic accuracy. We review advances in 5 areas relevant to the diagnosis of UNE: technologic advancements with modern EMG machines have allowed for reconsideration of the question of experimental error and lesion detection; how temperature effects can lead to misdiagnosis; the effect of body mass index on the electrodiagnosis of UNE; the validation of short segment studies; and the emerging role of high-resolution sonography as a diagnostic tool.
A 30-year-old man developed unexplained rhabdomyolysis, persistently increased creatine kinase and severe debilitating muscle cramps. After a nondiagnostic neurologic evaluation, he was referred for a muscle biopsy, to include histology/histochemistry, a myoglobinuria panel, and a caffeine halothane contracture test. Only the caffeine halothane contracture test was positive, and a subsequent ryanodine receptor type 1 gene evaluation revealed a mutation functionally causative for malignant hyperthermia. His identical twin brother, who was suffering from similar complaints, was found to share the same mutation. They each require oral dantrolene therapy to control symptoms, despite difficulty in identifying health care providers familiar with treating this disorder.
Acquired focal dystonia is often precipitated by minor local traumatic injury, resulting in severe pain and disability. An active duty soldier with shoulder dystonia, after a fall, that responded partially to botulinum toxin A is described. Post-traumatic dystonia as a neurological illness is discussed, with emphasis on mechanisms, precipitating causes, differential diagnosis, and treatment implications for battlefield clinicians.
Reliability of CTE and across-elbow NCV are similar. Shorter distances, if measured linearly, can be used to determine across-elbow ulnar nerve conduction. Muscle Nerve 55: 664-668, 2017.
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