Jason S. Hawley

Psychogenic dystonia and peripheral trauma

Dystonia in association with peripheral trauma is a well-described clinical syndrome. The syndrome goes by many names—“traumatic” dystonia, “fixed” dystonia, peripherally induced dystonia, or complex region pain syndrome (CRPS) dystonia. We reviewed the role of peripheral trauma in the development of dystonia, focusing on 4 subtypes—cervical dystonia, focal limb dystonia, CRPS dystonia, and psychogenic dystonia. We show that peripheral trauma inducing, provoking, or precipitating structural changes within the CNS leading to dystonia is not an accepted concept, and current evidence supporting a pathophysiologic mechanism is virtually nonexistent. A better approach to this clinical syndrome is to define it as fixed abnormal posturing that is most commonly psychogenic. While symptomatic treatment of pain and spasms with medication can be beneficial, early psychological evaluation and patient-specific treatment is important. Modalities such as physical and occupational therapy should be utilized early. Finally, it should be emphasized that like many psychogenic movement disorders, it remains a highly disabling and distressing disorder.

Hemiballismus: Current concepts and review

Hemiballism is a rare movement disorder characterized by a high amplitude movement of an entire limb or limbs on one side of the body. The acute development of hemiballismus is often caused by focal lesions in the contralateral basal ganglia and STN. Many etiologies exist for this rare disorder with vascular causes and nonketotic hyperglycemia being the most common. Clearer understanding of the pathophysiology of hemiballism has led to important insights into the function and interaction of structures within the basal ganglia. Newer models of basal ganglia function have been proposed based on the study of the pathophysiology of hemiballism. Prognosis is favorable for most patients with complete resolution with or without treatment. Medical and surgical treatments are often successful in reducing or completely ameliorating the movements in those patients with more severe movements.

Subarachnoid Pneumocephalus From Epidural Steroid Injection

We present a case of severe posterior headache and diplopia following an epidural steroid injection for lumbar pain.

Post-Traumatic Shoulder Dystonia in an Active Duty Soldier

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.

Intramedullary spinal sarcoidosis: clinical improvement reflected in T-lymphocyte subpopulation ratios

Study design:Case report.Objectives:A case report of spinal sarcoidosis improving clinically and radiographically with treatment which correlated with improvement in cerebrospinal fluid T-lymphocyte subpopulation ratios.Setting:Walter Reed Army Medical Center.Case report:A 46-year-old man presented with an enhancing spinal cord lesion. Lymph node biopsy confirmed sarcoidosis, and cerebrospinal fluid (CSF) analysis showed elevation in the ratio of two T-lymphocyte subpopulations. Treatment with steroids resulted in clinical resolution and immunocytologic improvement in the CSF.

A Tale of Two Tauopathies

A 77-year-old man developed progressive language and balance difficulties. He had difficulty rising from a chair, with falls occurring within the first year of symptoms. After 1 year, rapid, spontaneous jerks appeared in the left hand, which he described as “useless.” Neurologic examination 3 years after his initial symptoms demonstrated 23/30 on the Mini-Mental State Examination and an applause sign. He had apathy, without a pseudobulbar affect. Aside from mild word-finding difficulties, his language examination was normal. Cranial nerve examination showed vertical gaze limitation with loss of vertical fast phases on testing of optokinetic nystagmus (OKN). Vertical gaze limitations could be overcome with oculocephalic maneuvers. Motor examination revealed markedly increased tone in his left arm, with moderate left greater than right bradykinesia, without tremor. Left ideomotor apraxia was present. Action and postural myoclonus was present in the left arm. There was neck extension and axial rigidity. Gait was slow and slightly wide-based with impaired postural reflexes. Stereognosis and graphesthesia were intact. A trial of levodopa/carbidopa 900 mg per day showed no symptomatic benefit. Brain MRI without gadolinium revealed asymmetric, focal atrophy of the right frontoparietal area and the midbrain tegmentum (figure).nnnnFigure Brain MRI n(A) Axial T1-weighted brain MRI shows atrophy of the …

Intra-Arterial tPA Treatment for Basilar Artery Thrombosis in the Combat Zone: An Example of Modern Nontrauma Medical Care in War

Military physicians deploy primarily to care for traumatic injuries. However, critically ill medical patients are also evaluated and treated in theater with similar capability as hospitals in the United States because of the close proximity of medical and surgical specialists and advanced equipment in combat support and theater hospitals. We report a case of a 33-year-old soldier diagnosed with a basilar infarct, treated with fibrinolytics, and reversal of severe neurological deficits while treated in the U.S. operational Afghanistan Theater.

Psychogenic dystonia and peripheral traumaAuthor Response

PSYCHOGENIC DYSTONIA AND PERIPHERAL TRAUMA Jay A. Van Gerpen, Rochester, MN: I read the article by Drs. Hawley and Weiner, who frequently equate fixed, painful postures that abruptly occur after peripheral trauma as a sine qua non of peripheral-induced dystonia.1 In table 2,1 they indicate that these are not universal features of this condition. Therefore, I ask the authors to consider the following case: A man with a left L5/S1 radiculopathy gradually developed persistent “tightness, like a bow string” in his left gastrocnemius ambulating only with eventual hypertrophy of this muscle. In addition, when this patient’s father was in his late adolescence, he gradually developed stable, nonfixed “torticollis” after a “whiplash” injury. The patient’s paternal aunt had the insidious onset of persistent “neck shaking” after a thyroidectomy. Each of these individuals has a c.446 T greater than C sequence variant in their THAP1 gene.2 Do the authors believe that there is “ample evidence” to support a link between THAP1 mutations and dystonia3,4? If so, what would be the most likely explanation for these patients developing symptoms and signs consistent with organic dystonia in areas of their body associated with trauma? Tracy Butler, New York: Drs. Hawley and Weiner1 note that there is little evidence of a pathophysiologic mechanism for peripheral trauma inducing or provoking focal dystonia. One mechanism that could be considered involves aberrant thalamic plasticity. In a study of patients with peripheral limb or nerve injury, PET using [C]PK11195—a ligand of the translocator protein expressed by activated microglia—revealed long-lasting microglial activation in the thalamus contralateral to injury.5 The degree of microglial activation in patients with peripheral injury is comparable to that seen in CNS disorders such as epilepsy.6 Microglia cells, in addition to their role as the brain’s resident immune cells, are increasingly understood to be key mediators of synaptic plasticity and remodeling.7 Given that thalamic injury is well known to cause focal dystonia,8 it is conceivable that an abnormally intense, widespread or otherwise dysregulated thalamic microglial response to peripheral injury may lead to focal dystonia in some patients, and other problems such as pain. Future studies using [C]PK11195 PET can evaluate this hypothesis. Additionally, it is important to point out that “nonorganic” is not a synonym for “psychogenic”; psychogenic disorders are also brain disorders, and elucidating their neural basis is critical to optimizing treatment.