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Clinical Reasoning: A 14-year-old girl with headache, seizures, and confusion

期刊: NEUROLOGY, 2019; 92 (2)

A 14-year-old girl without any relevant medical history was transferred to our institution due to worsening headache along with nausea, vomiting, and generalized tonic-clonic (GTC) seizures for 7 days. She was also noted to have multiple psychological and behavioral abnormalities for 1 day. Her headache was described as severe holocephalic pain aggravated when lying down and alleviated after vomiting. No throbbing or phonophobia/photophobia was observed. Her seizures occurred once or twice a day, lasting about 1 minute each and resolving spontaneously. Symptoms were refractory to rotundine (dopamine D1 receptor antagonist), azasetron (antiemetic), mannitol, oxcarbazepine, and phenobarbital.

IF:8.69

Teaching Video NeuroImages: A 20-year-old man with distal paresthesia

期刊: NEUROLOGY, 2019; 92 (2)

A 20-year-old man presented with a 3-week history of progressive distal paresthesia in his lower limbs. His gait became clumsy and he became unable to mobilize. The patient was vegetarian and after questioning admitted to regular recreational nitrous oxide use.

IF:8.69

Diagnosis and treatment evaluation of in-stent restenosis of carotid artery stenting using optical coherence tomography

期刊: NEUROLOGY, 2019; 92 (2)

A 65-year-old man underwent balloon angioplasty and repeat carotid artery stenting (CAS) due to in-stent restenosis (ISR). Intravascular optical coherence tomography (OCT) showed a severe ISR with fibrotic neointimal growth (figure 1). Post 6.0 x 30 mm balloon angioplasty, OCT showed intimal disruption and artery dissection (figure 2). After the 8 x 40 mm stent fully expanded, OCT showed stent struts well apposed, but tissue protrusion from the spaces between stent struts can be observed (figure 2). OCT allowed measurement of intimal hyperplasia after the CAS and observation of intimal disruption and stent strut apposition intraoperatively.(1,2)

IF:8.69

Shape-similar errors in Chinese pure alexia

期刊: NEUROLOGY, 2019; 92 (8)

A 64-year-old literate Chinese man presented with sudden onset of difficulty reading. Examination revealed right homonymous hemianopsia and pure alexia. The oral reading test showed that he often read one character as another one with a similar shape but different meaning and pronunciation (figure 1). Brain MRI showed acute infarction involving the left occipital lobe, medial temporal lobe, and splenium of corpus callosum (figure 2). Pure alexia is a clinical disconnection syndrome that was first described in a patient with a similar infarction by Dejerine in 1892.(1) Shape-similar errors may be a phenomenon characteristic of Chinese pure alexia.

IF:8.69

Teaching NeuroImages: Migrating sparganum captured on brain MRI

期刊: NEUROLOGY, 2019; 92 (10)

A 16-year-old girl presented with repeated seizure, headache, and vomiting. One year after symptom onset, MRI revealed a midbrain lesion (figure 1). Symptoms persisted despite antituberculosis treatment. Repeat MRI 1 year later showed lesion of similar size/shape, but shifted by 1.4 cm. Spirometra mansoni antibody was positive in serum and CSF. Sparganosis was verified upon surgery (figure 2). Symptoms disappeared after surgery, and she was discharged. Key features indicative of sparganosis in this case included migrating lesion and positive antibody. Definitive diagnosis requires recovery of sparganum from the lesion.(1) Surgery provides a cure; pharmacotherapy is typically ineffective.(2)

IF:8.69

Iron deposition in periaqueductal gray matter as a potential biomarker for chronic migraine

期刊: NEUROLOGY, 2019; 92 (10)

Objective To study iron deposition in red nucleus (RN), globus pallidus (GP), and periaqueductal gray matter (PAG) as a potential biomarker of chronic migraine (CM) and its association with levels of biomarkers related to migraine pathophysiology. Methods This case-control study included 112 patients with migraine (55 CM, 57 episodic migraine [EM]) and 25 headache-free controls. We analyzed iron deposition using 3T MRI and the NIH software platform ImageJ; we analyzed serum levels of markers of inflammation, endothelial dysfunction, and blood-brain barrier (BBB) disruption by ELISA in peripheral blood during interictal periods. Results Patients with CM showed larger iron grounds volume in RN compared to patients with EM (70.2 +/- 6.8 vs 25.5 +/- 7.3 mu L, p < 0.001) and controls (70.2 +/- 6.8 vs 15.1 +/- 10.8 mu L, p < 0.001), as well as larger iron deposits in PAG compared to patients with EM (360.3 +/- 6.5 vs 249.7 +/- 6.9 mu L, p < 0.001) and controls (360.3 +/- 6.5 vs 168.6 +/- 10.3 mu L, p < 0.001). In PAG, differences were also significant between patients with EM and controls. No significant differences were obtained for GP. Receiver operating characteristic curves showed that the optimal threshold for iron volume was 15 mu L in RN (80% sensitivity, 71% specificity) and 240 mu L in PAG (93% sensitivity, 97% specificity). Iron grounds volume in PAG was correlated with higher plasma levels of soluble tumor necrosis factor-like WEAK (r = 0.395, p = 0.005) and cellular fibronectin (r = 0.294, p = 0.040). Conclusions Patients with CM showed increased iron deposition in RN and PAG compared to patients with EM and controls. Iron grounds volume in PAG identified correctly patients with CM and was associated with elevated biomarkers of endothelial dysfunction and BBB disruption.

IF:8.69

Acute ischemic stroke due to painless long-segmental aortic dissection

期刊: NEUROLOGY, 2019; 92 (10)

A man in his 60s with no obvious trauma experienced sudden left-sided weakness and confusion. At a local hospital at 4.5 hours after onset, a cranial CT was unremarkable; the initial diagnosis was acute ischemic large artery stroke. He was transferred to our stroke center for mechanical thrombectomy within 6 hours. Digital subtraction angiography revealed no appearance of left common carotid artery and left subclavian artery, accompanied by suspicious protrusion of aortic false lumen (figure, A, arrows). Aortic dissection was suspected and confirmed by CT angiography (figure, B and C), with emergent surgery following. Painless long-segmental aortic dissection should be considered during evaluation for IV thrombolysis and mechanical thrombectomy.

IF:8.69

Increased dose of carbidopa with levodopa and entacapone improves "off" time in a randomized trial

期刊: NEUROLOGY, 2019; 92 (13)

Objective To investigate whether increased fixed carbidopa doses of 65 or 105 mg (ODM-101/65 and ODM-101/105) in combination with 75, 100, 125, or 150 mg of levodopa and 200 mg of entacapone might improve "off" time in fluctuating Parkinson disease (PD) compared to the standard combination of 4:1 levodopa/carbidopa with the usual 200 mg of entacapone (LCE) during a 4-week treatment period. Methods This was a randomized, double-blind, double-dummy, active-controlled, crossover, multicenter, phase II, proof-of-concept study in patients with fluctuating PD. Results One hundred seventeen patients were randomized into the study (mean age 67.0 years; daily "off" time 5.3 hours; mean daily levodopa dose 610 mg). Carryover-adjusted mean changes from baseline "off" times were during ODM-101/65, -1.53 hours (p = 0.02 vs LCE), during ODM-101/105, -1.57 hours (p = 0.01 vs LCE), and during LCE -0.91 hours. Changes in daily "on" time without dyskinesia were 1.54 hours (p = 0.005 vs LCE), 1.38 hours (p = 0.0214 vs LCE), and 0.69 hours, respectively. Changes in "on" time with troublesome dyskinesia were <0.1 hours and not significantly different between treatments. In patients with high-activity COMT genotypes Val/Met or Val/Val, "off" time was reduced more with ODM-101/65 and ODM-101/105 than with LCE (p = 0.015 and p = 0.006). No difference between the treatments was seen in safety and tolerability. The most common treatment-related adverse effects were nausea, dizziness, drug-effect decrease, and dyskinesia, which were in most cases mild or moderate in severity. Treatment-related serious adverse events were diarrhea (ODM-101/105 and LCE), and myocardial ischemia and blood creatine kinase increase (LCE). Conclusion Increasing the dose of carbidopa in combination with levodopa and entacapone should be considered in the treatment of fluctuating PD to improve daily "off" times. Genotyping patients with PD according to COMT activity may improve individual treatment strategies. ClinicalTrials.gov identifier NCT01766258. Classification of evidence This study provides Class II evidence that an increased dose of carbidopa improves motor fluctuations when administered with levodopa and entacapone.

IF:8.69

Clinical Reasoning: A teenager with persistent headache

期刊: NEUROLOGY, 2019; 92 (13)

A previously healthy 15-year-old boy with no relevant medical history presented with 3 weeks of severe, persistent, holocephalic pain associated with nausea and vomiting, without fever or alteration of consciousness. A brain MRI including magnetic resonance angiography and magnetic resonance venography (MRV) at a local hospital was normal. A lumbar puncture revealed a CSF pressure of 240 mm H2O. An intracranial infection was suspected based on clinical symptoms and signs. He received empiric antiviral therapy and rehydration for 1 week, but there was no relief of symptoms. He was subsequently transferred to our hospital.

IF:8.69

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