Stiff-person syndrome: an atypical presentation and a review of the literature.

Introduction: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder associated with muscle rigidity and spasms. A number of antibodies have been associated with disorder, including anti-glutamic acid decarboxylase and anti-amphiphysin.Case report; In this report, we present a rare case of a 79-year-old woman who presented with bilateral lower extremity weakness who was ultimately diagnosed with stiff-limb syndrome, a rare variant of SPS. Extensive laboratory and CSF studies were unrevealing. Electromyography showed significant peroneal motor neuropathy and complex repetitive discharges in the left tibialis anterior muscle. Antibodies to glutamic acid decarboxylase were significantly elevated at 124 units/mL. She was subsequently started on oral diazepam with significant improvement in her symptoms.Conclusion: The presentation of SPS can vary based on epidemiologic factors, clinical symptoms, and associated disorders. These forms can have overlapping features which may make the categorization of patients into one of these forms challenging.

Concurrent Paraspinous Myopathy and Myasthenia Gravis.

Paraspinous myopathy is a rare neuromuscular disorder characterized by selective involvement of the cervical, thoracic, or lumbar muscles. Leading clinical features include a bent spine or dropped head (antecollis). In myasthenia gravis (MG), patients may have camptocormia secondary to neuromuscular junction dysfunction of the paraspinal muscles, and this condition usually responds to acetylcholinesterase inhibitors or immunosuppressive treatments. However, concomitant MG and paraspinous myopathy with histologic and electrophysiologic evidence of myopathic changes of the paraspinal muscles has only been reported twice in the literature. In this case series, 5 patients with MG with paraspinous myopathy are reported. While neck and extension truncal weakness is not uncommon in MG, most often the weakness is due to neuromuscular junction dysfunction. Coexisting paraspinous myopathy and MG is under-recognized, is often resistant to mainstay treatments, and should be considered in patients presenting with neck or trunk extensor weakness.

Sexual function after strokes.

Strokes are the second leading cause of death and the third leading cause of disability worldwide. Thanks in part to better and more available diagnosis, treatment, and rehabilitation, the vast majority of stroke patients tend to survive strokes, particularly in the industrialized world. Motor disability and cognitive changes such as aphasia and visuospatial disorders are most often considered among the major contributors to stroke burden. This chapter discusses disorders of sexual functions as another frequent sequel of strokes. Strokes generally induce hyposexuality, but in some instances they may be followed by hypersexuality. There is some evidence suggesting that lesions of either hemisphere affect sexual activities, but for different reasons: aphasia and depression after left-hemisphere lesions, a deficit in arousal and perhaps visuospatial disorders after right-hemisphere lesions. Psychologic, psychosocial, and physical factors, as well as medications, play an important role. A better understanding of the psychosocial and physiologic mechanisms underlying sexual functioning can provide insight into improving sexual activity and therefore quality of life in patients affected by strokes and other brain lesions.

Factitious psychogenic nonepileptic paroxysmal episodes.

Mistaking psychogenic nonepileptic paroxysmal episodes (PNEPEs) for epileptic seizures (ES) is potentially dangerous, and certain features should alert physicians to a possible PNEPE diagnosis. Psychogenic nonepileptic paroxysmal episodes due to factitious seizures carry particularly high risks of morbidity or mortality from nonindicated emergency treatment and, often, high costs in wasted medical treatment expenditures. We report a case of a 28-year-old man with PNEPEs that were misdiagnosed as ES. The patient had been on four antiseizure medications (ASMs) with therapeutic serum levels and had had multiple intubations in the past for uncontrolled episodes. He had no episodes for two days of continuous video-EEG monitoring. He then disconnected his EEG cables and had an episode of generalized stiffening and cyanosis, followed by jerking and profuse bleeding from the mouth. The manifestations were unusually similar to those of ES, except that he was clearly startled by spraying water on his face, while he was stiff in all extremities and unresponsive. There were indications that he had sucked blood from his central venous catheter to expel through his mouth during his PNEPEs while consciously holding his breath. Normal video-EEG monitoring; the patient's volitional and deceptive acts to fabricate the appearance of illness, despite pain and personal endangerment; and the absence of reward other than remaining in a sick role were all consistent with a diagnosis of factitious disorder.