Kimberly Harbaugh

Misdiagnosis of Symptomatic Cerebral Aneurysm Prevalence and Correlation With Outcome at Four Institutions

BACKGROUND AND PURPOSE It is not known what fraction of patients with symptomatic cerebral aneurysms are misdiagnosed at initial medical presentation. It is also not clear whether misdiagnosed patients more frequently deteriorate before definitive aneurysm diagnosis and therapy or achieve a poorer outcome than correctly diagnosed patients. METHODS We reviewed records of consecutive patients with symptomatic cerebral aneurysms managed by four tertiary-care neurosurgical services during a recent 19-month period. Clinical course and outcome were analyzed according to misdiagnosis or correct diagnosis at initial medical evaluation. RESULTS Fifty-four of 217 patients (25%) were misdiagnosed at initial medical evaluation, including 46 of 121 patients (38%) initially in good clinical condition (clinical grade 1 or 2). Forty-six of 54 patients (85%) in the misdiagnosis group were initially grade 1 or 2 compared with 75 of 163 patients (46%) with correct initial diagnosis (P < .01). Twenty-six of 54 misdiagnosed patients (48%) deteriorated or rebled before definitive aneurysm treatment compared with 4 of 165 correctly diagnosed patients (2%) (P < .001). Among patients initially presenting as clinical grade 1 or 2, overall good or excellent outcome was achieved in 91% of those with correct initial diagnosis and 53% of patients with initial misdiagnosis (P < .001). Deterioration before correct diagnosis accounted for 16 of 67 patients (24%) with poor or worse final outcome in this series. CONCLUSIONS Patients in good clinical condition with symptomatic cerebral aneurysms were commonly misdiagnosed. Misdiagnosed patients were more likely than correctly diagnosed patients to deteriorate clinically and had a worse overall outcome. Misdiagnosed cases accounted for a significant fraction of overall poor outcomes among consecutive cases of symptomatic aneurysms.

Shoulder numbness in a patient with suprascapular nerve entrapment syndrome: cutaneous branch of the suprascapular nerve: case report.

OBJECTIVE AND IMPORTANCEThe ability to diagnose peripheral nerve disorders is dependent on knowledge of the anatomic course and function of the nerves in question. The classic teaching regarding the suprascapular nerve (SScN) is that it has no cutaneous branches, despite the fact that a cutaneous branch was first reported in the anatomic literature 20 years ago. CLINICAL PRESENTATIONWe describe a case of a 35-year-old male patient who presented with right shoulder pain and atrophy and weakness of the right supra- and infraspinatus muscles. During the examination, he was also noted to have an area of numbness involving the right upper lateral shoulder region. Electrical study results were consistent with SScN entrapment at the suprascapular notch. INTERVENTIONThe patient underwent surgical decompression 7 months after the onset of his symptoms. The patient noted resolution of his shoulder pain immediately after the procedure, and his shoulder sensory disturbance had improved by 2 weeks. At 9 months after surgery, he remained pain-free, his shoulder sensation was normal, and his motor abnormalities had improved significantly. CONCLUSIONThis case provides clinical evidence for the presence of a cutaneous branch of the SScN, as described in cadaveric studies. Although shoulder numbness demands a search for alternative diagnoses, it does not necessarily exclude the diagnosis of SScN entrapment.

The muscular axillary arch: an anatomic study and clinical considerations.

OBJECTIVE The muscular axillary arch is a musculotendinous structure that arises from the latissimus dorsi muscle and crosses the axilla before inserting to the humerus, brachial fascia, or coracoid process. Case reports have described the neurovascular compression symptoms caused by this anatomic variant and have reported that the symptoms can be relieved by division of the muscle. However, there has been little information published regarding this topic in the neurosurgical literature. METHODS We evaluated 70 axillary dissections in 35 cadavers to assess for the presence of this anomaly. RESULTS The muscular axillary arch was identified unilaterally in 3 (8.6%) of the 35 cadavers. All 3 arches arose from the anterior border of the latissimus dorsi muscle and inserted at a point along a line extending from the coracoid process to the intertubercular groove deep to the insertion of the pectoralis major muscle. All 3 arches crossed over the neurovascular bundle in the axilla. CONCLUSION Compression by the muscular axillary arch should be considered in the differential diagnosis of patients with thoracic outlet and hyperabduction syndromes.

Fulminant Guillain-Barré syndrome after closed head injury: a potentially reversible cause of an ominous examination. Case report.

Fulminant Guillain-Barré syndrome (GBS) is a rapidly progressive form of polyneuropathy in which patients demonstrate eventual flaccid quadriplegia and an absence of brainstem function. Most patients present after a mild upper respiratory or gastrointestinal illness and have nondiagnostic cerebral imaging studies. The authors present a case of fulminant GBS that developed in a 55-year-old alcoholic man 1 week after admission for a closed head injury. The details of this case and a discussion of GBS will be presented. This case provides evidence for combined central and peripheral nervous system involvement in severe cases of GBS. Recognition of fulminant GBS is important to prevent inappropriate declaration of brain death or withdrawal of support in the face of a potentially reversible process.

Clinical outcomes of aneurysmal subarachnoid hemorrhage patients treated with oral diltiazem and limited intensive care management

BACKGROUND Aneurysmal subarachnoid hemorrhage (SAH) patients are frequently treated with prophylactic nimodipine and undergo invasive monitoring of blood pressure and volume status in an intensive care unit (ICU) setting to decrease the incidence of delayed ischemic neurological deficit (DIND) and improve functional outcomes. The goal of this study was to examine the incidence of DIND and poor functional outcomes in a consecutive series of SAH patients treated with a different regimen of prophylactic oral diltiazem and limited use of intensive care monitoring. METHODS The study involved a consecutive series of 123 aneurysmal SAH patients treated by the senior author who were admitted within 72 hours of hemorrhage and who never received nimodipine or nicardipine. Functional outcomes were graded using the Glasgow Outcome Scale (GOS). RESULTS Of the 123 patients identified, favorable outcomes (GOS 4 and 5) were achieved in 74.8%. The incidence of DIND was 19.5%. Hypertensive, hypervolemic, hemodilutional (HHH) therapy was used in 10 patients (8.1%) and no patients were treated for DIND by endovascular means. Seven patients (5.7%) had a poor functional outcome or death because of DIND and two of these were related to complications of HHH therapy. These results were compared to contemporary series of SAH patients managed with other treatment protocols. CONCLUSIONS Functional outcomes of patients treated with a regimen of oral diltiazem, limited use of ICU monitoring and HHH therapy for DIND compare favorably with other contemporary series of SAH patients.

An interstitial deletion within 9p21.3 and extending beyond CDKN2A predisposes to melanoma, neural system tumours and possible haematological malignancies

Familial atypical multiple mole melanoma syndrome (FAMMM) is characterised by dysplastic naevi, malignant melanoma and pancreatic cancer. Given that large deletions involving CDKN2A (cyclin-dependent kinase inhibitor 2A) account for only 2% of cases, we describe a family that highlights the co-occurrence of both melanoma and neural system tumours to aid clinical recognition and propose a management strategy. A patient with multiple neurofibromas was referred with a provisional diagnosis of neurofibromatosis type 1 (NF1). Prior molecular testing, though, had failed to identify an NF1 mutation by sequencing and multiplex ligation-dependent probe amplification. His family history was significant for multiple in situ/malignant melanomas at young ages and several different cancers reminiscent of an underlying syndrome. A search of the Familial Cancer Database, FaCD Online, highlighted several families with cutaneous melanoma and nervous system tumours who were subsequently identified to have large deletions spanning CDKN2A. Although sequencing of CDKN2A and TP53 failed to identify a mutation, a heterozygous CDKN2A deletion was identified by targeted array comparative genomic hybridisation (CGH). Whole-genome oligonucleotide array CGH and SNP analysis identified an interstitial deletion of at least 1.5 Mb within 9p21.3 and spanning approximately 25 genes. Identification of the underlying molecular abnormality permits predictive testing for at-risk relatives. Given the young cancer diagnoses, a surveillance regimen was developed and a clinical team organised for ongoing management so that genetic testing could be offered to both adults and minor children. Surveillance recommendations addressed cancer risks associated with FAMMM, and other cancers exhibited by this family with a large contiguous gene deletion.

Pure neuritic leprosy presenting as ulnar nerve neuropathy: a case report of electrodiagnostic, radiographic, and histopathological findings

Hansens disease, or leprosy, is a chronic infectious disease with many manifestations. Though still a major health concern and leading cause of peripheral neuropathy in the developing world, it is rare in the United States, with only about 150 cases reported each year. Nevertheless, it is imperative that neurosurgeons consider it in the differential diagnosis of neuropathy. The causative organism is Mycobacterium leprae, which infects and damages Schwann cells in the peripheral nervous system, leading first to sensory and then to motor deficits. A rare presentation of Hansens disease is pure neuritic leprosy. It is characterized by nerve involvement without the characteristic cutaneous stigmata. The authors of this report describe a case of pure neuritic leprosy presenting as ulnar nerve neuropathy with corresponding radiographic, electrodiagnostic, and histopathological data. This 11-year-old, otherwise healthy male presented with progressive right-hand weakness and numbness with no cutaneous abnormalities. Physical examination and electrodiagnostic testing revealed findings consistent with a severe ulnar neuropathy at the elbow. Magnetic resonance imaging revealed diffuse thickening and enhancement of the ulnar nerve and narrowing at the cubital tunnel. The patient underwent ulnar nerve decompression with biopsy. Pathology revealed acid-fast organisms within the nerve, which was pathognomonic for Hansens disease. He was started on antibiotic therapy, and on follow-up he had improved strength and sensation in the ulnar nerve distribution. Pure neuritic leprosy, though rare in the United States, should be considered in the differential diagnosis of those presenting with peripheral neuropathy and a history of travel to leprosy-endemic areas. The long incubation period of M. leprae, the ability of leprosy to mimic other conditions, and the low sensitivity of serological tests make clinical, electrodiagnostic, and radiographic evaluation necessary for diagnosis. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

The angular course of the median nerve in the distal forearm and its anatomical importance in preventing nerve injury in a modern era of carpal tunnel release

OBJECTIVE A thorough understanding of anatomy is critical for successful carpal tunnel release. Several texts depict the median nerve (MN) as taking a course parallel to the long axis of the forearm (LAF). The authors report on their attempt to formally assess the course of the MN as it travels to the carpal tunnel in the distal wrist and discuss its potential clinical significance. METHODS The width of the wrist, the distance from the radial wrist to the MN, and the distance from the distal volar wrist crease to the point where the MN emerges between the flexor carpi radialis (FCR) tendon and the flexor digitorum superficialis (FDS) tendons were recorded during cadaveric dissection of 76 wrist specimens. The presence or absence of palmaris longus was documented. Finally, the angles between the MN and FCR tendon and between the MN and the LAF were measured using ImageJ. RESULTS The relative position of the MN at the distal wrist crease, as determined by the ratio of the distance from the MN to the radial wrist divided by wrist width, revealed a mean value of 0.48, indicating that the nerve was usually located just radial to midline. The mean distance between the distal wrist crease and the MNs emergence was 34.6 mm. The mean angle between the MN and the FCR tendon was 14.1°. The angle between the MN and the LAF had a mean value of 8.8° (range 0.0°-32.2°). The nerve was parallel to the LAF in only 10.7% of the studied wrists. Palmaris longus was absent in 14 (18.4%) of the 76 wrists. CONCLUSIONS The MN takes an angular approach to the carpal tunnel in the distal wrist in the vast majority of cases. This newly described finding will be useful to both clinicians and anatomists.

Fracture related ulnar and sciatic nerve transections: a report of two cases and literature review

IntroductionCase reports, case series, and case control studies have looked at the incidence of complete nerve transection in the setting of fracture and the need for surgical exploration dating back to the 1920s. We present two cases of nerve laceration accompanying traumatic fracture with a thorough review of the literature.MethodsWe used the following search terms: “ulnar nerve” OR “sciatic nerve” AND “laceration” OR “transection” AND “fracture.” Results were reviewed and included for discussion if they specifically reported ulnar or sciatic nerve laceration accompanying traumatic fracture.ResultsOur search yielded 15 papers reporting a total of 10 ulnar nerve lacerations and nine sciatic nerve lacerations. We present two additional cases. The first is a patient with a humerus fracture and complete ulnar nerve transection. The second case is a patient who suffered a femur fracture and complete transection of the sciatic nerve.ConclusionNerve laceration accompanying traumatic fracture is rare. We review the reported cases of nerve laceration and present two cases treated at our institution. Though uncommon, nerve laceration should be considered in the setting of traumatic fracture with neurological injury, particularly open fractures.

Evaluating the evidence: is neurolysis or neurectomy a better treatment for meralgia paresthetica?

BackgroundMeralgia paresthetica is a mononeuropathy of the lateral femoral cutaneous nerve (LCFN). Surgical treatment involves transection or decompression of the LCFN. There is no clear consensus on the superiority of one technique over the other. We performed a systematic review of the literature to answer this question.MethodsEligible studies included those that compared neurolysis versus neurectomy for the treatment of meralgia paresthetica after failure of conservative therapy. Our outcome of interest was resolution of symptoms. We performed a computerized search of MEDLINE (PubMed; all years) and of the Cochrane Central Register of Controlled Trials. Eligible studies had to include the words “meralgia paresthetica” and “surgery.” All patients regardless of age were included, and there was no language restriction. We then reviewed the articles’ titles and abstracts. All studies that compared neurolysis to neurectomy were included in the analysis.ResultsOf the studies identified, none were randomized controlled trials. There were two German language articles that were translated by a third researcher.Each study was evaluated by two independent researchers who assigned a level of evidence according to American Association of Neurologist algorithm and also performed data extraction (neurolysis vs. neurectomy and resolution of pain symptoms). Each study was found to be level four evidence.ConclusionAfter reviewing the data, there was insufficient evidence to recommended one method of treatment over the other. This highlights the importance of keeping a national registry in order to compare outcomes between the two methods of treatment.