Unilateral winged scapula: Clinical and electrodiagnostic experience with 128 cases,with special attention to long thoracic nerve palsy

Introduction: In this study we report a large series of patients with unilateral winged scapula (WS), with special attention to long thoracic nerve (LTN) palsy. Methods: Clinical and electrodiagnostic data were collected from 128 patients over a 25‐year period. Results: Causes of unilateral WS were LTN palsy (n = 70), spinal accessory nerve (SAN) palsy (n = 39), both LTN and SAN palsy (n = 5), facioscapulohumeral dystrophy (FSH) (n = 5), orthopedic causes (n = 11), voluntary WS (n = 6), and no definite cause (n = 2). LTN palsy was related to neuralgic amyotrophy (NA) in 61 patients and involved the right side in 62 patients. Discussion: Clinical data allow for identifying 2 main clinical patterns for LTN and SAN palsy. Electrodiagnostic examination should consider bilateral nerve conduction studies of the LTN and SAN, and needle electromyography of their target muscles. LTN palsy is the most frequent cause of unilateral WS and is usually related to NA. Voluntary WS and FSH must be considered in young patients. Muscle Nerve 57 : 913–920, 2018     

Cranial nerve palsies accompanying pituitary tumour

We reviewed 12 patients with pituitary tumour and cranial nerve palsy to analyse the clinical characteristics, the radiographic appearances, and the outcome after surgery. All patients had pathologically nonfunctioning macroadenomas with evidence of apoplexy. The third cranial nerve was the most frequently affected, followed by the sixth and fourth cranial nerves. Third cranial nerve palsy manifested as a symptom sequence comprising mydriasis, followed by limitation of gaze and ptosis. These symptoms recovered in reverse order of development. The time taken for recovery of cranial nerve palsy after surgery was significantly correlated with the length of time between the onset of symptoms and surgery. Pituitary apoplexy appears to be the primary cause of cranial nerve palsy with pituitary tumour. Early surgical intervention is most likely to bring about rapid recovery from cranial nerve dysfunction.     

Radial nerve paralysis--causes, site and diagnosis. Analysis of 103 cases]

The results of a retrospective analysis of 103 patients presenting with a radial nerve palsy are reported. Among the traumatic injuries of peripheral nerves the radial nerve is the one most frequently affected due to its exposed anatomic site with close neighbourhood to the long bones of the arm. Fractures of the shaft of the humerus (18%) and proximal radius (12%) are the main causes of traumatic lesions (55%). Among the non-traumatic peripheral nerve injuries the radial nerve is less frequently affected than the median and the ulnar nerves. The most frequent site of radial nerve lesions in these cases is the upper arm (22%), the most frequent cause is pressure palsy (21%) during unphysiological deep sleep ("Saturday night paralysis").     

A standardized ultrasound approach in neuralgic amyotrophy

Neuralgic amyotrophy (NA), also referred to as idiopathic brachial plexitis and Parsonage-Turner syndrome, is a peripheral nerve disorder characterized by acute severe shoulder pain followed by progressive upper limb weakness and muscle atrophy. While NA is incompletely understood and often difficult to diagnose, early recognition may prevent unnecessary tests and interventions and, in some situations, allow for prompt treatment, which can potentially minimize adverse long-term sequalae. High-resolution ultrasound (HRUS) has become a valuable tool in the diagnosis and evaluation of NA. Pathologic HRUS findings can be grouped into four categories: nerve swelling, swelling with incomplete constriction, swelling with complete constriction, and fascicular entwinement, which may represent a continuum of pathologic processes. Certain ultrasound findings may help predict the likelihood of spontaneous recovery with conservative management versus the need for surgical intervention. We recommend relying heavily on history and physical examination to determine which nerves are clinically affected and should therefore be assessed by HRUS. The nerves most frequently affected by NA are the suprascapular, long thoracic, median and anterior interosseous nerve (AIN) branch, radial and posterior interosseous nerve (PIN) branch, axillary, spinal accessory, and musculocutaneous. When distal upper limb nerves are affected (AIN, PIN, superficial radial nerve), the lesion is almost always located in their respective fascicles within the parent nerve, proximal to its branching point. The purpose of this review is to describe a reproducible, standardized, ultrasonographic approach for evaluating suspected NA, and to share reliable techniques and clinical considerations when imaging commonly affected nerves.     

Tuberculous meningitis presenting as mesencephalic infarction and syringomyelia

Cerebral ischaemia is a serious complication of tuberculous meningitis (TBM) with the anterior circulation most commonly affected. Acute syringomyelia is a very rare complication of TBM. Here, we report an unusual presentation of TBM with a third nerve palsy as a result of posterior circulation stroke as well as a syringomyelia.     

Ultrasound of peripheral nerves in neuralgic amyotrophy

Introduction: Neuralgic amyotrophy (NA) can often be difficult to diagnose. Nerve ultrasound (US) is potentially useful, but it is operator-dependent, especially for small nerves. Methods: Fifty-one consecutive patients with NA (mean duration 16 months) and 50 control subjects underwent US of the brachial plexus and major nerves of the upper extremity at predefined sites. We compared cross-sectional areas (CSAs) of affected and unaffected sides with controls and sides within patients. Results: The median nerve and radial nerve at the level of the upper arm were enlarged on the affected sides compared with controls and the unaffected sides of patients. Enlargement was most pronounced for affected sides vs. controls (median 44%, radial 67%). Discussion: NA patients showed increased CSAs, especially in the major nerves of the upper limb, even after longer disease duration. This could make US a useful adjunct in diagnosing NA. Muscle Nerve 59 :55–59, 2019     

Evaluation of proximal facial nerve conduction by transcranial magnetic stimulation.

A magnetic stimulator was used for direct transcutaneous stimulation of the intracranial portion of the facial nerve in 15 normal subjects and in patients with Bell's palsy, demyelinating neuropathy, traumatic facial palsy and pontine glioma. Compound muscle action potentials (CMAPs) thus elicited in the orbicularis oris muscle of controls were of similar amplitude but longer latency (1.3 SD 0.15 ms) compared with CMAPs produced by conventional electrical stimulation at the stylomastoid foramen. No response to magnetic stimulation could be recorded from the affected side in 15 of 16 patients with Bell's palsy. Serial studies in two patients demonstrated that the facial nerve remained inexcitable by magnetic stimulation despite marked improvement in clinical function. In the patient with a pontine glioma, the CMAP elicited by transcranial magnetic stimulation was of low amplitude but normal latency. In six of seven patients with demyelinating neuropathy, the response to intracranial magnetic stimulation was significantly delayed. Magnetic stimulation produced no response in either patient with traumatic facial palsy. Although the precise site of facial nerve stimulation is uncertain, evidence points to the labyrinthine segment of the facial canal as the most likely location.     

Electrodiagnostic studies of the facial nerve in peripheral facial palsy and hemifacial spasm

Electrodiagnostic (EDX) assessment is one of the most important aspects in the evaluation of the two most common disorders of the facial nerve: facial palsy and hemifacial spasm. Facial palsy is usually an acute disorder that resolves in a few weeks but, in a number of cases, leads to a postparalytic facial syndrome featuring muscle synkinesis, myokymia, and involuntary mass contractions of muscles on the affected side. Hemifacial spasm is usually a chronic disorder characterized by paroxysms of involuntary, clonic, and synchronous twitching of all facial muscles on the affected side. EDX studies provide information on lesion location and severity, pathophysiology underlying the two disorders, and differential diagnosis between syndromes presenting with abnormal facial muscle activity. This monograph is intended to describe the most relevant EDX findings in the two disorders and the most appropriate timing for the examinations in order to provide useful information for prognosis and therapeutic decision-making.     

Hereditary amyloidosis of the Finnish type in a German family: Clinical and electrophysiological presentation

Hereditary amyloidosis of the Finnish type (HAF, or familial amyloid polyneuropathy type IV) is an autosomal dominant disease that has been described most commonly in the Finnish population but has also been found in some other countries. Herein we report the first German family whose members suffer from this condition. There are no known Finnish ancestors. We performed clinical and electrophysiological examinations in 22 members of this family. All symptomatic family members suffered from facial palsy, and most of them had peripheral neuropathy. One patient had confirmed corneal lattice dystrophy. Additional symptoms were hypoglossal nerve involvement in 5 patients and oculomotor nerve palsy in 1 patient. The lips of all older patients appeared thickened. The causative G654A mutation in the gelsolin gene was found in all affected family members. Muscle Nerve, 2010     

An alarming sign for serious diseases in children: bilateral facial paralysis

Facial paralysis in children is most often idiopathic, and isolated facial nerve palsy resulting from leukemic infiltration is a rare occurrence. We report a 13-year-old male with acute lymphoblastic leukemia presenting with bilateral facial palsy, who was previously diagnosed with idiopathic facial palsy and treated with steroids. This rare presentation of acute lymphoblastic leukemia should be kept in mind as a diagnostic possibility in a patient with bilateral facial nerve paralysis.     

Posttraumatic pneumocephalus-induced bilateral oculomotor nerve palsy

Bilateral third nerve palsy often points to the involvement of its nucleus. Third nerve palsy as a result of posttraumatic nuclear involvement is an extremely rare condition. A 23-year-old man presented with a depressed skull fracture after acute head trauma and had Glasgow Coma Scale Score of 9. The diameters of the pupils were 6.5 and 7.5 mm and they were not reactive to light stimulation. There was bilateral ptosis. Computed tomography (CT) relieved bilateral perimesensephalic pneumocephalus. We suggested that bilateral oculomotor nerve paresis might develop in association with posttraumatic bilateral perimesensephalic pneumocephalus, which affected the nucleus of the third nerve.     

Posttraumatic pneumocephalus-induced bilateral oculomotor nerve palsy

Bilateral third nerve palsy often points to the involvement of its nucleus. Third nerve palsy as a result of posttraumatic nuclear involvement is an extremely rare condition. A 23-year-old man presented with a depressed skull fracture after acute head trauma and had Glasgow Coma Scale Score of 9. The diameters of the pupils were 6.5 and 7.5 mm and they were not reactive to light stimulation. There was bilateral ptosis. Computed tomography (CT) relieved bilateral perimesensephalic pneumocephalus. We suggested that bilateral oculomotor nerve paresis might develop in association with posttraumatic bilateral perimesensephalic pneumocephalus, which affected the nucleus of the third nerve.     

Isolated Abducens Nerve Palsy: Update on Evaluation and Diagnosis

Abducens nerve palsy is a common clinical finding in neurology practice. In many instances, the origin is obvious and management straightforward; however, the list of possible etiologies and mimics is vast and diverse and diagnostic decisions can be challenging and even controversial. This is especially true when the abducens nerve is affected in isolation, since in the current era of cost-effective medicine, it is critical to accurately diagnose etiologies that may lead to major morbidity or mortality with efficiency. Topics for highlighted updates in this review include management of isolated abducens nerve palsy with a high likelihood of a microvascular ischemic etiology; common imaging pitfalls and current state-of-the-art neuroimaging; and abducens palsy mimics.     

致动眼神经麻痹脑动脉瘤的DSA研究

目的:探讨致动眼神经麻痹脑动脉瘤的DSA表现。方法:整理DSA证实为脑动脉瘤患者资料85例,对其临床DSA表现进行分析。结果:脑动脉瘤致动眼神经麻痹的发生率约占全部脑动脉瘤的16.5%,后交通动脉瘤最易导致动眼神经麻痹,约占全部后交通动脉瘤的40%。DSA可以真实的反映致动眼神经麻痹的脑动脉瘤的特征。结论:对于动眼神经麻痹的患者,应行DSA检查,以明确或排除脑动脉瘤的可能。     

Isolated sensory manifestations in neuralgic amyotrophy: report of eight cases

A series of eight patients with isolated clinical and electrophysiological sensory deficit related to neuralgic amyotrophy (NA) is reported. NA was diagnosed by clinical and electrodiagnostic features and disease course. Imaging and laboratory investigations excluded other disorders. The results showed mild to severe involvement of eight individual sensory nerves: lateral antebrachial cutaneous nerve lesions in three instances and partial lesions of the median nerve in five cases. Our findings suggest that isolated clinical and electrodiagnostic sensory involvement in NA is not exceptional but rather is unrecognized. The pattern of these nerve lesions agrees with the most typical pattern of NA, which is a mononeuritis or mononeuritis multiplex. The present study also shows that the spectrum of NA is diverse and may vary from pure motor to pure sensory deficit, according to the nature of the involved nerve fibers.     

Bell's palsy - part of a polyneuropathy?

Some authors have suggested that Bell's palsy should be considered a part of a polyneuritis cranialis and others, reporting Bell's palsy in patients with diabetes mellitus, proposed that it is an overt sign of a subclinical polyneuropathy. To study whether nerves other than the facial nerve were affected, the clinical findings of 112 patients with Bell's palsy were correlated with a quantitative EMG done on an asymptomatic leg muscle. The electromyographic results obtained from these leg muscles showed changes similar to those occurring in peripheral nerve lesions, and the changes correlated well with the degree of facial palsy. The results indicate that the patients with symptomatic facial dysfunction have an acute subclinical polyneuropathy.     

Predisposing factors in Bell's palsy: a case-control study

Summary The frequency of diabetes mellitus reported in subjects affected by Bell's palsy varies widely. In this investigation, a case-control study, we encountered a frequency of 24.8%. In addition, arterial hypertension and lipid disturbances were found to affect subjects with Bell's palsy more frequently than controls. These findings appear to suggest a primarily ischaemic pathogenesis for most cases of idiopathic peripheral facial paralysis. Furthermore, the finding of significantly lower taste impairment in diabetics than in non-diabetics with Bell's palsy may support the hypothesis of a vascular rather than a metabolic pathogenesis in these cases also. In fact, the vessels supplying the distal portion of the facial nerve, probably more affected in the diabetic patients in order to preserve taste sensation, have such a particular anatomical configuration that this might favour the onset of a diabetic small vessel disease which, in turn, would represent a factor of easier decompensation.     

A case of multiple cranial nerve palsy with severe dysphagia due to herpes zoster infection]

A case of multiple cranial nerve palsy by herpes zoster was reported. A 79-year-old man showed fever, sore throat, and dysphagia. No vesicle was noted at ear and pharynx. The patient developed, later, left peripheral facial nerve palsy. The cerebrospinal fluid revealed pleocytosis with increased protein. The viral antibody titer of herpes zoster was significantly elevated both in cerebrospinal fluid and in serum. The left facial palsy was slightly improved. But his dysphagia didn't improve during at least 10 months after the onset. Among the cranial nerves, trigeminal and facial nerves are the most commonly affected by herpes zoster. But there are a few cases of the 9th and 10th cranial nerve involvement in the literature. However, dysphagia has rarely been reported in these previous cases, only four cases developed severe dysphagia like the present patient. All of these cases including our case were over sixty years old, while cases with slight dysphagia were under sixty years old. No other differentiating factor is noted between these two groups with regard to sites of vesicles, findings of cerebrospinal fluid and mode of therapy.     

Complete oculomotor palsy caused by persistent trigeminal artery

Primitive trigeminal artery (PTA) is the most frequent embryonic communication between the carotid and vertebro-basilar system. PTA is a pathophysiology phenomenon which has been implicated as a rare cause of cranial nerve dysfunction. We report the case of a 40-year-old woman who developed a complete oculomotor nerve palsy caused by a persistent ecstatic trigeminal artery. Brain MRI and MRA studies documented a neurovascular conflict between the oculomotor nerve and a PTA. To the best of our knowledge there is no report about complete third cranial nerve palsy NC due to a PTA. A role of this rare vascular condition is discussed.     

Lymphoma as a cause of isolated oculomotor nerve palsy

We report two patients with diffuse large B-cell lymphoma with isolated oculomotor nerve palsy. This was the initial manifestation of lymphoma in one patient but in a second, the palsy appeared as a part of a generalized recurrence of lymphoma. In addition, we reviewed the clinical findings of 12 previously reported patients. Isolated oculomotor nerve palsy was most frequently associated with the large B-cell lymphoma cell type, and was not frequently associated with periorbital pain. The pupil was spared in half the patients irrespective of infiltration of the oculomotor nerve. MRI and cerebrospinal fluid cytological examinations are helpful in determining invasion of lymphoma to the oculomotor nerve, cavernous sinus, and surrounding leptomeninges.