Internal Carotid Artery Dissection Presenting With Dysgeusia, Horner Syndrome, and Hypesthesia of the Fifth Cranial Nerve: A Case Report.

BACKGROUND: Carotid artery dissection (CAD) is a significant cause of ischemic stroke. Early recognition and treatment of CAD is important to prevent accompanying cerebral ischemia. This case report presents an atypical clinical presentation of CAD and emphasizes the diagnostic challenge for emergency physicians. CASE REPORT: A 54-year-old woman presented to the emergency department with a bilateral headache of 4 days' duration, hypesthesia of the left fifth cranial nerve, dysgeusia, and partial Horner syndrome on the left side. Magnetic resonance angiography showed a left-sided CAD without any signs of cerebral ischemic events. Antiplatelet therapy with clopidogrel was started, and the patient did not show any deterioration in the weeks thereafter. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: CAD can present with different combinations of cranial nerve palsies and should be in the differential diagnosis of dysgeusia and hypesthesia of the trigeminal nerve. Early recognition of CAD can be challenging in patients with rare cranial nerve involvement, but early treatment is crucial to prevent cerebral ischemic events.

[Back pain and radicular pain after subarachnoid haemorrhages]. / Rugpijn na een subarachnoïdale bloeding.

Subarachnoid haemorrhages (SAH) are acute life-threatening events that are frequently misdiagnosed. 4% of patients with SAH do not have the typical acute intense headaches, but present with other symptoms. Misdiagnosis leads to treatment delays and, consequently, higher morbidity and mortality. We describe two patients with atypical symptoms after SAH and delay in diagnosis. The first patient came to the emergency room with cervical, back and radicular pain that spread to both legs. An acute headache had started 11 days earlier. Physical examination showed signs of meningeal irritation. A cerebral CT scan revealed a subarachnoid haemorrhage. The second patient came to the outpatient clinic with pain in his lower back, apathy, apraxia and unsteady gait after an acute headache had started nine days before. When the patient visited our outpatient clinic, the headache had disappeared. A cerebral CT scan nevertheless revealed a subarachnoid haemorrhage. When diagnosing SAH, it is important not to miss the acute headache in the history, even though this headache may no longer be present at the time of presentation.

[Cerebral imaging in a delirious patient]. / Beeldvormend onderzoek bij een verwarde patiënt.

An 80-year-old woman attended the emergency department with sub-acute confusion and fever. On examination no focal signs were found. Cerebral CT showed an area of hypodensity suspected to be an infarction, but an MRI scan carried out 10 days later showed cerebral abscesses caused by a dental infection that had occurred four weeks previously. This case report shows that even if a patient with confusion does not show focal signs on examination, an organic brain disease - such as cerebral abscess - may still be present. Hence, when confusion is associated with fever or impaired consciousness, an MRI scan should be carried out.

[Acute treatment of hypertension in intracerebral haemorrhage]. / Acute hypertensiebehandeling na een hersenbloeding.

Thus far no effective treatment for an intracerebral haemorrhage has been available. A randomized clinical trial recently showed that treatment of hypertension in the acute phase of spontaneous intracerebral haemorrhage with a target systolic blood pressure of 140 mmHg is safe and improves prognosis. The effect of blood pressure reducing therapy was small and not statistically significant for the primary outcome (mortality or severe morbidity). Moreover, this effect was shown in one trial only. Therefore the clinical relevance of these study results remains debatable. Considering all available arguments, in patients with a systolic blood pressure >150 mmHg in the first 6 hours after spontaneous intracerebral haemorrhage the Dutch Neurovascular Working Group recommends lowering the blood pressure to a target systolic level of 140 mmHg within 1 hour and maintaining this target level for 1 week. When this strategy is not chosen, we recommend that a systolic blood pressure >180 mmHg should be treated anyway, with a target level of 160 mmHg.

[Neurological signs in aortic dissection]. / Neurologische verschijnselen bij een aortadissectie.

A substantial number of patients suffering from aortic dissection will show neurological signs. These can dominate the clinical picture and hinder an accurate diagnosis of this life-threatening disease. We present a case of lower extremity pain and a case of transient global amnesia caused by aortic dissection. A third patient suffered from acute cerebral ischemia accompanied by hypotension and back pain, suggestive of aortic dissection. In this third case, aortic dissection was excluded before systemic thrombolytic therapy was administered, for the patient could have suffered disastrous complications caused by this emergency stroke therapy. Clinicians should be aware that a wide range of cerebral, spinal and peripheral neurological signs can be caused by aortic dissection. An unusual combination of symptoms can be a clue for underlying aortic disease. High-risk clinical features are predisposing factors in medical history, typical acute onset back or chest pain, and pulse deficit, blood pressure asymmetry or a new cardiac murmur on physical examination. These features should be explicitly evaluated in patients with an acute neurological deficit. If neurological symptoms and a high-risk clinical feature are present, immediate aortic imaging should be considered since early detection can be life saving.

Autosomal dominant restless legs syndrome maps to chromosome 20p13 (RLS-5) in a Dutch kindred.

Six chromosomal loci have been mapped for restless legs syndrome (RLS) through family-based linkage analysis (RLS-1 to RLS-6), but confirmation has met with limited success, and causative mutations have not yet been identified. We ascertained a large multigenerational Dutch family with RLS of early onset (average 18 years-old). The clinical study included a follow-up of 2 years. To map the underlying genetic defect, we performed a genome-wide scan for linkage using high-density SNP microarrays. A single, strong linkage peak was detected on chromosome 20p13, under an autosomal-dominant model, in the region of the RLS-5 locus (maximum multipoint LOD score 3.02). Haplotype analysis refined the RLS-5 critical region from 5.2 to 4.5 megabases. In conclusion, we provide the first confirmation of the RLS-5 locus, and we reduce its critical region. The identification of the underlying mutation might reveal an important susceptibility gene for this common movement disorder.

[Juxta facet cyst of the lumbar spine: an uncommon cause of lumbar radiculopathy]. / De juxta-articulaire facetcyste: een bijzondere oorzaak van het lumbosacraal radiculair syndroom.

Lumbar juxta facet cysts (JFC) are an uncommon cause of radiculopathy. Spontaneous regression of symptomatic JFC has not often been reported. We describe 2 patients, a 59-year-old man and a 55-year-old man, with radiculopathy of the 5th lumbar nerve root due to a JFC at L4-5. The first patient recovered spontaneously. After 8 months, the JFC had clearly reduced on MRI. In the second patient the JFC was surgically resected due to progressive pain, after which the patient remained without symptoms. In the literature it is suggested that surgical removal of the JFC should be the treatment of choice. However, of the 5 patients who were diagnosed with a JFC in our department, 3 recovered spontaneously and 2 after surgery. In our opinion further studies on the course and management of symptomatic lumbar JFC are warranted.

Episodic ataxia associated with EAAT1 mutation C186S affecting glutamate reuptake.

BACKGROUND: Episodic ataxia (EA) is variably associated with additional neurologic symptoms. At least 4 genes have been implicated. Recently, a mutation in the SLC1A3 gene encoding the glutamate transporter EAAT1 was identified in a patient with severe episodic and progressive ataxia, seizures, alternating hemiplegia, and migraine headache. The mutant EAAT1 showed severely reduced uptake of glutamate. The syndrome was designated EA6 and shares overlapping clinical features with EA2, which is caused by mutations in CACNA1A. OBJECTIVE: To test the role of the SLC1A3 gene in EA. DESIGN: Genetic and functional studies. We analyzed the coding region of the SLC1A3 gene by direct sequencing. SETTING: Academic research. PATIENTS: DNA samples from 20 patients with EA (with or without interictal nystagmus) negative for CACNA1A mutations were analyzed. MAIN OUTCOME MEASURES: We identified 1 novel EAAT1 mutation in a family with EA and studied the functional consequences of this mutation using glutamate uptake assay. RESULTS: We identified a missense C186S mutation that segregated with EA in 3 family members. The mutant EAAT1 showed a modest but significant reduction of glutamate uptake. CONCLUSIONS: We broadened the clinical spectrum associated with SLC1A3 mutations to include milder manifestations of EA without seizures or alternating hemiplegia. The severity of EA6 symptoms appears to be correlated with the extent of glutamate transporter dysfunction.

Assessment of feasibility of endovascular treatment of ruptured intracranial aneurysms with 16-detector row CT angiography.

BACKGROUND: It is unclear whether 16-detector row CT angiography (CTA) can replace digital subtraction angiography (DSA) to assess the feasibility of endovascular treatment (EVT) in the acute phase after aneurysmal subarachnoid hemorrhage. METHODS: We studied 80 consecutive patients with aneurysmal subarachnoid hemorrhage, who underwent both CTA and DSA. Two interventional neuroradiologists independently scored CTA and, immediately thereafter, DSA with respect to feasibility of EVT. We determined whether CTA without DSA was sufficient for a definite judgment. We also assessed interobserver agreement. RESULTS: The 2 readers judged EVT to be feasible in 24 and 37 patients with CTA alone and not feasible in 34 and 20 patients. In these patients, DSA yielded additional information in 6 (reader 1) and 5 patients (reader 2), which did not affect treatment decision. In 19 and 7 patients, DSA was considered inferior to CTA. In the remaining patients (n = 22 and 23, respectively), feasibility of EVT could not be judged with CTA alone, and DSA results were required in addition for a treatment decision. Interobserver agreement on feasibility of EVT was just fair (kappa <0.40). CONCLUSIONS: In our series of patients, 16-detector row CTA was a reliable investigation to assess feasibility of EVT of ruptured intracranial aneurysms in most patients. Further, we found that interobserver disagreement on feasibility of EVT was considerable.

Temporary divergence paralysis in viral meningitis.

A 43-year-old woman who reported diplopia and headache was found to have comitant esotropia at distance fixation and normal alignment at reading distance (divergence paralysis). Eye movement, including abduction, was normal as was the rest of the neurologic examination. Brain MRI was normal. Lumbar puncture showed an elevated opening pressure and a cerebrospinal fluid formula consistent with viral meningitis. The patient was treated with intravenous fluids and analgesics and with a temporary prism to alleviate diplopia. Within 3 weeks, she had fully recovered. This is the first report of divergence palsy in viral meningitis.

Cerebral hypoperfusion and clinical onset of dementia: the Rotterdam Study.

Cerebral blood flow (CBF) velocity is decreased in patients with Alzheimer's disease. It is being debated whether this reflects diminished demand because of advanced neurodegeneration or that cerebral hypoperfusion contributes to dementia. We examined the relation of CBF velocity as measured with transcranial Doppler with dementia and markers of incipient dementia (ie, cognitive decline and hippocampal and amygdalar atrophy on magnetic resonance imaging) in 1,730 participants of the Rotterdam Study aged 55 years and older. Cognitive decline in the 6.5 years preceding CBF velocity measurement was assessed with repeated Mini-Mental State Examinations in nondemented subjects (n = 1,716). Hippocampal and amygdalar volumes were assessed in a subset of 170 nondemented subjects. Subjects with greater CBF velocity were less likely to have dementia. Furthermore, in nondemented subjects, greater CBF velocity was related to significantly less cognitive decline over the preceding period (odds ratio per standard deviation increase in mean CBF 0.74 [95% confidence interval, 0.58-0.98]) and larger hippocampal and amygdalar volumes. A low CBF is associated with dementia, but also with markers of incipient dementia. Although we cannot exclude that this is caused by preclinical neurodegeneration leading to hypoperfusion, it does suggest that cerebral hypoperfusion precedes and possibly contributes to onset of clinical dementia.

Cerebral haemodynamics in the elderly: the rotterdam study.

Transcranial Doppler ultrasonography allows the non-invasive assessment of cerebral haemodynamics. Data on the frequency distribution of these parameters in the oldest old are scarce, which makes a distinction between physiological and pathophysiological ageing difficult. We studied the relation between cerebral haemodynamic parameters and age and sex in 1,720 participants of a population-based study by means of transcranial Doppler ultrasonography. The end-diastolic, peak systolic and mean cerebral blood flow velocity, and cerebrovascular CO2 reactivity declined significantly with increasing age up to 90 years (per year -0.6 cm/s; -0.3 cm/s; -0.5 cm/s, and -0.6%/kPa, respectively). The pulsatility index increased with age (per year 0.01). End-diastolic, peak systolic and mean cerebral blood flow velocities were lower in men compared to women (age-adjusted difference 1.6 cm/s; 4.1 cm/s, and 2.5 cm/s, respectively). Cerebrovascular CO2 reactivity was higher in men compared to women. Adjusting for carotid atherosclerosis did not change the observed sex differences. These findings provide insight into physiologic changes of haemodynamics during ageing and may serve as a starting point for investigations on determinants of pathophysiologic changes in cerebral haemodynamics in the elderly.