This Month in The Archives of Neurology

Hughes and colleagues provide succinct recommendations for the management of patients with Guillain-Barré syndrome. Acute patient management strategies are provided in detail.

Tang et al describe the first occurrence of a heat shock protein 27 (Hsp27) gene mutation causing Charcot-Marie-Tooth (CMT) disease in Chinese patients. The C379T mutation in Hsp27 causes a late CMT disease type 2F phenotype with a mild sensory disorder. David E. Pleasure, MD,⁴⁰⁰²⁰ provides editorial perspective.

Orlandi et al report that a low flow velocity in the middle cerebral artery as measured by transcranial Doppler monitoring and microembolic signals may impair the clearance of microemboli and should be considered a forecast of cerebrovascular symptoms during carotid artery stenting.

Perren and colleagues describe combined polar-paramedian thalamic infarction in 12 patients who were selected from 208 consecutively registered patients with thalamic strokes in the Lausanne Stroke Registry. Key features of this syndrome included amnesia preceded by a period of altered consciousness and vertical eye movement disturbances. Severe and persistent amnesia may be due to coexisting damage to the anterior and dorsomedial nuclei.

Maulaz et al reviewed 309 patients who were receiving regular aspirin therapy. Aspirin therapy had been discontinued for 13 patients with strokes and 4 control subjects. Aspirin interruption yielded an odds ratio for ischemic stroke/transient ischemic attack of 3.4. Thus, they emphasize the importance of aspirin therapy compliance and the potential risk associated with discontinuing aspirin therapy in patients at risk for cerebrovascular disease and coronary artery disease.

Maeder-Ingvar and colleagues reviewed the Lausanne Stroke Registry and found that 195 (4.1%) of 4802 patients met the clinical criteria for pure monoparesis involving the face (22%), arm (63%), or leg (15%).There was a wide range of stroke localization and etiology in monoparesis.

Koch et al report that large arterial disease is frequently associated with acute multiple brain infarction in the posterior circulation. This diffusion-weighted imaging study supports the importance of embolism as the main mechanism of infarction in patients with vertebrobasilar occlusive disease.

Shin and colleagues discuss the mechanisms of recurrent vascular events (strokes or coronary heart disease) in subtypes of ischemic stroke. The pattern of recurrent stroke differed for intracranial and extracranial groups. Recurrent strokes in the extracranial distribution were often unpredictable with respect to the site of recurrence and degree of pre-existing stenosis. None of the patients in the extracranial group had recurrence as intracranial disease or vice versa.

Avorn et al report that dopamine agonists significantly increase the degree of sudden uncontrollable somnolence in a dose-related manner. Patients in this series taking a dopamine agonist were nearly 3-fold as likely to have episodes of sudden uncontrollable somnolence compared with all other patients receiving Parkinson disease medication.

Okun et al analyzed causes of suboptimal results of deep brain stimulation (DBS) for a movement disorder. They reviewed 41 consecutive patients with suboptimal results from DBS surgery over a 24-month period. Issues related to inadequate preoperative screening, surgical and device-related complications, and programming and medication adjustments were factors in the evaluation. They report that with appropriate specific intervention, 51% of patients who complained of “failed” DBS procedures ultimately had good outcomes, but 34% of patients had persistently poor outcomes despite maximal intervention. This case series provides important insights into reasons for DBS failures and proposes strategies to manage patients with DBS more effectively.

Vilchez et al describe a new R1905X founder mutation in the dysferlin gene (DYSF) that produced 3 dysferlinopathy phenotypes without intrafamilial heterogeneity. This mutation of note was expressed as 3 different clinical syndromes (limb-girdle muscular dystrophy, Miyoshi distal myopathy, and distal anterior dysferlinopathy), but only 1 phenotype was found in the same family.

As reported by de Lau et al, patients with Parkinson disease had an increased risk of dementia (hazard ratio, 2.8; 95% confidence interval, 1.8-4.4), and it was especially pronounced in participants carrying at least 1 apolipoprotein E gene (APOE) ε2 allele (13.5; 4.5-40.6). Parkinson disease was associated with an increased mortality risk (1.8; 1.5-2.3).

Afridi et al report on positron emission tomographic scans labeled with radioactive water (H₂¹⁵O) in patients with migraines both during an episode and interictally. Comparing the migraine scans with interictal scans, there was significant activation in the dorsal pons. Activation was also seen in the right anterior cingulate, posterior cingulate, cerebellum, thalamus, insula, prefrontal cortex, and temporal lobes. In some patients, migraines involve a subcortical (pons) activation.

Wüllner and colleagues describe specific positron emission tomography scan differences in spinocerebellar ataxia (SCA) types 1, 2, 3, and 6. Decreased regional cerebral glucose metabolism was found in the cerebellum of all patients with SCA; the brainstem of patients with SCA1, SCA2, and SCA3; the thalamus and putamen of patients with SCA3; and the parietal cortex of patients with SCA2. The binding potential of ¹¹C-d-threo-methylphenidate (a dopamine transporter) was reduced in the striatum in patients with SCA2 and SCA3.