Diabetic inferior division palsy of the oculomotor nerve

In this case report we present a diabetic patient with isolated, pupil-sparing, incomplete inferior division third cranial nerve palsy. Laboratory tests for the evaluation of thyroid function, infection, myasthenia gravis and autoimmune diseases were normal. Cranial computed tomography, magnetic resonance imaging and magnetic resonance angiography also showed normal findings. Accordingly, diabetes related vasculopathic third nerve palsy was suggested. The ocular signs of oculomotor palsy completely disappeared 2 months later. Although this clinical entity is rarely reported, differential diagnosis with pupil-sparing third nerve palsy of other etiologies such as compression by an aneurysm or tumor still need to be investigated.     

Polyneuritis of cranial nerves and acquired immunodeficiency syndrome (AIDS): 5 cases

Cranial neuropathies were present in 5 patients with positive serology for the human immunodeficiency virus. Two patients presented with abnormalities of ocular movements (3rd, 4th), two with an isolated unilateral facial nerve palsy and one with a lesion of the accessory nerve. Neurological symptoms and signs are present in approximately 60 to 80 p. 100 of cases of the acquired immunodeficiency syndrome, but cranial neuropathies affect only 2 to 3 p. 100 of the patients. The isolation of the human immunodeficiency virus from the nerve suggest a direct role, but an indirect immune mechanism may also be present. Some of the patients with aseptic meningitis or subacute encephalopathy have demonstrated involvement of cranial nerves, mainly 2nd, 7th and 8th. Systemic tumors (lymphoma) may involve the central nervous system by diffuse meningeal invasion with lesions of 3rd, 4th and 6th nerves. Opportunist infections like Herpes zoster or Cytomegalovirus may produce cranial neuropathies (2nd, 5th). Isolated mononeuropathies or cranial nerves palsies have also been reported in patients treated with chemotherapeutic agents like vinca alkaloids.     

Cranial nerve involvement in childhood polyarteritis nodosa.

Amongst a variety of neurological manifestations of childhood polyarteritis nodosa, cranial nerve involvement is unusual. We report 4 cases with cranial nerve palsies in a series of 36 biopsy-proven patients. Two cases presented with IIIrd nerve palsy alone, one with right IIIrd and left IVth nerve palsy, and one with peripheral VIIth nerve paresis. All 4 patients showed good response to prednisolone and cyclophosphamide treatment. Cranial nerve involvement in childhood polyarteritis nodosa seems not so rare when patients are followed on long term basis.     

Tolosa-Hunt syndrome preceded by facial palsy in a child

We report on a previously healthy 11-year-old boy with unilateral periorbital mild headache and facial nerve palsy, followed during the next 5 months by recurrent unilateral headaches and subsequent extrinsic paresis of the third cranial nerve and paresis of the sixth cranial nerve, each of which improved with steroids. Cranial magnetic resonance imaging revealed increased thickening of the left cavernous sinus and adjacent structures, with marked gadolinium enhancement. Eight months after the initial signs, he developed left retro-orbital headache that lasted 16 days and was relieved 24 hours after resuming steroid treatment. Recurrent multiple cranial neuropathies, neuroimaging findings, and long-lasting headache that responded to steroids indicated Tolosa-Hunt syndrome, further confirmed by extensive investigation and a long follow-up to exclude other causes. This patient illustrates the complexity of disorders with multiple cranial nerve palsies, and adds to the sparse literature on Tolosa-Hunt syndrome in children, describing the first pediatric case preceded by facial palsy.     

Microvascular cranial nerve palsies in an Arabic population.

OBJECTIVES: The incidence of microvascular ocular cranial nerve palsies may be increasing with the prevalence of diabetes in the developing world. We review this problem for the first time in an Arabic population. MATERIALS AND METHODS: This is a prospective nonrandomized study of all patients with the diagnosis of microvascular cranial mononeuropathy seen in the Neuro-ophthalmology Clinic at the King Khaled Eye Specialist Hospital between September 1997 and April 1998. RESULTS: Forty-seven patients with microvascular palsies of cranial nerves 3, 4, or 6 were seen in this 8-month period. Compared to previous studies, this group had a stronger association with previously diagnosed diabetes mellitus, more males affected, and a longer duration of the cranial nerve palsy before complete resolution. Five patients had an unusual clinical course that included a second microvascular cranial mononeuropathy before the first palsy completely resolved. CONCLUSIONS: Microvascular cranial nerve palsies may occur more frequently in this Arabic population than elsewhere and may have certain unusual features.     

Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement

A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.     

Primary Sj?gren's syndrome manifesting as multiple cranial neuropathies: MRI findings

We report a case of primary Sjögren''s syndrome presenting with multiple cranial nerve palsies and radiological evidence of cranial pachymeningitis and hypophysitis. A 47-year-old woman developed right sensory neural hearing loss followed, 2 months later, by right facial palsy. Cranial magnetic resonance imaging showed features of pachymeningitis and pituitary gland infiltration. The diagnosis of primary Sjögren''s syndrome was confirmed by demonstrating positive SS-A and SS-B antibodies and histological evidence of lymphocytic infiltration of the sublabial salivary gland. During the 2-year follow-up, the patient had transient VI^th, IX^th, X^th, and XII^th cranial nerve palsies. Sjögren''s syndrome should be considered in the differential diagnosis of patients presenting with multiple recurrent cranial nerve palsies, even if prominent sicca symptoms are absent.     

Delayed hypoglossal palsy following occipital condyle fracture--case report.

Occipital condyle fractures are rare. When present, they produce lower cranial palsies and/or brainstem dysfunction. A 32 year old man sustained multiple injuries. At the time of admission the patient had no neurological deficits. Three weeks after the accident, the patient complained of slurring of speech. Clinical examination revealed an isolated hypoglossal palsy. Radiological evaluation revealed an occipital condyle fracture. The patient was treated with a rigid collar. Eighteen months after the injury, the patient noted slight improvement in his speech. However, clinical examination showed a persistent hypoglosssal palsy. Occipital condyle fractures are rare. They may be associated with lower cranial nerve palsies. As demonstrated by this case, this entity should be included in the differential diagnosis of hypoglossal palsy. Since occipital condyle fractures can exist without neurological deficits, special attention should be paid to imaging of the craniovertebral junction in patients with head injury.     

Spontaneous carotid dissection presenting lower cranial nerve palsies

Cranial nerve palsy in internal carotid artery (ICA) dissection occurs in 3--12% of all patients, but in 3% of these a syndrome of hemicranias and ipsilateral cranial nerve palsy is the sole manifestation of ICA dissection, and in 0.5% of cases there is only cranial nerve palsy without headache. We present two cases of lower cranial nerve palsy. The first patient, a 49-year-old woman, developed left eleventh and twelfth cranial nerve palsies and ipsilateral neck pain. The angio-RM showed an ICA dissection with stenosis of 50%, beginning about 2 cm before the carotid channel. The patient was treated with oral anticoagulant therapy and gradually improved, until complete clinical recovery. The second patient, a 38-year-old woman, presented right hemiparesis and neck pain. The left ICA dissection, beginning 2 cm distal to the bulb, was shown by ultrasound scanning of the carotid and confirmed by MR angiogram and angiography with lumen stenosis of 90%. Following hospitalisation, 20 days from the onset of symptoms, paresis of the left trapezius and sternocleidomastoideus muscles became evident. The patient was treated with oral anticoagulant therapy and only a slight right arm paresis was present at 10 months follow-up. Cranial nerve palsy is not rare in ICA dissection, and the lower cranial nerve palsies in various combinations constitute the main syndrome, but in most cases these are present with the motor or sensory deficit due to cerebral ischemia, along with headache or Horner's syndrome. In the diagnosis of the first case, there was further difficulty because the cranial nerve palsy was isolated without hemiparesis, and the second case presented a rare association of hemiparesis and palsy of the eleventh cranial nerve alone. Compression or stretching of the nerve by the expanded artery may explain the palsies, but an alternative cause is also possible, namely the interruption of the nutrient vessels supplying the nerve, which in our patients is more likely.     

Volume of focal brain lesions and hippocampal formation in relation to memory function after closed head injury in children

OBJECTIVES: (1) A study of verbal learning and memory in children who had sustained a closed head injury (CHI) at least 3 months earlier. (2) To relate memory function to focal brain lesion and hippocampal formation volumes using morphometric analysis of MRI. METHODS: A group of 245 children who had been admitted to hospital for CHI graded by the Glasgow coma scale (GCS), including 161 patients with severe and 84 with mild CHI completed the California verbal learning test (CVLT) and underwent MRI which was analysed for focal brain lesion volume independently of memory test data. Brain MRI with 1.5 mm coronal slices obtained in subsets of 25 patients with severe and 25 patients with mild CHI were analysed for hippocampal formation volume. Interoperator reliability in morphometry was satisfactory. RESULTS: Severity of CHI and age at study significantly affected memory performance. Regression analysis showed that bifrontal, left frontal, and right frontal lesion volumes incremented prediction of various learning and memory indices after entering the GCS score and age into the model. Extrafrontal lesion volume did not contribute to predicting memory performance. CONCLUSIONS: Prefrontal lesions contribute to residual impairment of learning and memory after severe CHI in children. Although effects of CHI on hippocampal formation volume might be difficult to demonstrate in non-fatal paediatric CHI, further investigation using functional brain imaging could potentially demonstrate hippocampal dysfunction.     

Parenchymal brain injury in the preterm infant: comparison of cranial ultrasound, MRI and neurodevelopmental outcome

AIM: Magnetic resonance imaging (MRI) is increasingly being used in high-risk preterm neonates. Cranial ultrasound (US) was compared with MRI in preterm patients with parenchymal injury and related to neurodevelopmental outcome. PATIENTS AND METHODS: Studies were performed in 61 patients. Twelve infants with normal US (Group 1) had an MRI within the first 4 weeks of life (early MRI), and 10 also at term age (late MRI). Eight out of 20 infants with intraventricular haemorrhage with parenchymal involvement (IVH + PI) (Group 2) had an early as well as a late MRI and 12 a late MRI. Of the 20 patients with cystic-periventricular leukomalacia (c-PVL) (Group 3), 7 had an early MRI, 1 had an MRI on both occasions and 12 had a late MRI. All 9 children with focal infarction (FI) (Group 4) had a late MRI. RESULTS: MRI was conform with cranial US in Group 1. Early MRI in Group 2 showed contralateral c-PVL in one infant and an additional contralateral occipital parenchymal haemorrhage and blood in the posterior fossa in another infant. Late MRI showed an asymmetrical posterior limb of the internal capsule (PLIC) (n=6), which predicted later hemiplegia. Early MRI in Group 3 showed more cysts (n = 5), punctate white matter lesions (n = 6), lesions in the basal ganglia (n = 1) and once involvement of the cerebellum. Late MRI showed involvement of the centrum semiovale (n = 2) lesions in the basal ganglia (n = 2) and bilateral abnormal signal intensity of the PLIC in 7 infants who all went on to develop cerebral palsy. In Group 4 MRI showed signal intensity changes suggestive of cystic lesions compared to persisting echogenicity on US (n = 3) and an asymmetrical PLIC (n = 5), which predicted hemiplegia in 4. CONCLUSION: Early MRI especially provided additional information in those with c-PVL. MRI at term age could assess the PLIC, which was useful in children with unilateral parenchymal involvement, for prediction of subsequent hemiplegia and, to a lesser degree, in bilateral c-PVL for prediction of diplegia or quadriplegia.     

Infantile progressive spinal muscular atrophy with ophthalmoplegia and pyramidal symptoms

Two siblings presented with identical features of progressive peripheral paralysis of the lower motor neuron type, pyramidal signs, cranial nerve palsy which included external ocular palsy and deafness, and internal ocular palsy; both died before 1 year of age. Pathologic examination of the central nervous system in both patients revealed degeneration and loss of spinal and cranial nerve motor nuclei, including the oculomotor nucleus. In addition, there was degeneration of the Edinger-Westphal nuclei and demyelination of the corticospinal tract under the midbrain. Although spinal cord lesions were indistinguishable from those of Werdnig-Hoffmann disease, the 2 patients are not considered to have Werdnig-Hoffmann disease from the clinicopathologic findings.     

Isolated cranial nerve palsy secondary to carotid dissection

Cranial nerve palsy has a variety of causes such as cerebral ischemia, nerve ischemia in diabetes, infectious and noninfectious meningitis, subarachnoid hemorrhage, malignant tumors of the skull base, neck, or upper mediastinum, aortic aneurysm, surgery of the thyroid,and many more. We report two cases of spontaneous carotid dissections leading to cranial nerve palsies, which is an uncommon cause of isolated cranial nerve palsies.ICA dissection must therefore be included in the differential diagnosis of lower cranial nerve palsy and should be assessed by duplex ultrasound and MRI as is demonstrated in our cases.     

Peripheral nerve neurolymphomatosis: Clinical features,treatment, and outcomes

Introduction: This series characterises nine patients with neurohistopathologically proven peripheral nerve neurolymphomatosis. Methods: A search of the hospital neuropathology database from 2002 to 2019 identified biopsy proven cases. Clinical data, investigation modalities, treatments, and outcomes were collated. Median age at neuropathy onset was 47 y, the neuropathy commonly as the initial lymphoma disease manifestation. Most (8/9) presented with painful asymmetrical sensory disturbance, with additional cranial nerve involvement in three. Neurophysiology typically demonstrated multiple axonal mononeuropathies. Cerebrospinal fluid protein was often raised (6/8). Magnetic resonance imaging suggested peripheral nerve infiltration in 6/9 and positron emission tomography CT in 4/9. Bone marrow biopsy was abnormal in 6/8. Treatment involved systemic or intrathecal chemotherapy and radiotherapy. Median survival was 23 mo. Discussion: Neurolymphomatosis is a rare but important cause of neuropathy, particularly in those lacking systemic evidence of lymphoma as correct aggressive treatment can prolong survival. Nerve biopsy is essential to classify lymphoma type and rule out alternatives.     

Posttraumatic cranial neuropathies.

Injury to cranial nerves is a common sequela of blunt head trauma. The olfactory, facial, and audiovestibular nerves are damaged most often, followed by the optic and ocular motor nerves. The trigeminal and lower cranial nerves are rarely involved. Chances of recovery are greatest for the facial nerve, intermediate for the ocular motor nerves, and least likely for the olfactory, optic, and audiovestibular nerves. Treatment is usually symptomatic, although steroids or surgical decompression of the optic and facial nerves can lead to dramatic results in selected patients.     

Peroneal neuropathy after weight loss

The objectives of this study were to evaluate the clinical and electrophysiological findings in peroneal mononeuropathies following a weight-reduction diet. Thirty patients with acute peroneal palsy and weight loss were studied. Complete nerve conduction studies (NCS) were performed in upper and lower limbs. NCS showed conduction block (CB) of the peroneal nerve at the fibular head that recovered in 29 patients within 3 weeks to 3 months. Severity of CB was correlated with clinical weakness. Three patients had abnormalities consistent with polyneuropathy (PNP). NCS in asymptomatic relatives confirmed familial neuropathy. Nerve biopsy and molecular study were consistent with hereditary neuropathy with liability to pressure palsies (HNPP). One of these peroneal palsies (6 months) recovered after neurolysis. Weight loss might be a risk factor in peroneal mononeuropathies. NCS is a tool in the diagnosis of the site and severity of the nerve injury. Testing should be considered for relatives of patients with PNP because peroneal mononeuropathies may be the first expression of HNPP.     

MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature

Background: Miller Fisher syndrome (MFS) is a rare demyelinating condition which may have involvement of cranial nerves. There are a few case reports of optic pathway involvement in children. We describe 3 patients with optic pathway enhancement in pediatric patients with MFS. Case series: We retrospectively reviewed brain imaging findings in 17 pediatric patients with of Guillain–Barré syndrome (GBS) meeting Brighton criteria who had brain MRIs performed during their acute illness. Cranial nerve enhancement was seen in 6/17 patients and optic nerve/chiasm enhancement was seen in 3 patients. Conclusion: Cranial nerve enhancement and optic pathway in particular, can be seen in patients with MFS. Imaging findings do not always correlate with clinical manifestations of cranial nerve involvement.     

Cavernoma of the trochlear nerve

Here we present the case of a 53-year old man with progressive double vision due to isolated left trochlear nerve palsy. Cranial magnetic resonance imaging (MRI) showed a small tumor within the left quadrigeminal cistern that did not increase in size after several months. Explorative neurosurgical intervention revealed a left trochlear nerve cavernoma. The lesion was microsurgically excised followed by end-to-end anastomosis of the trochlear nerve. After a one-year follow up, double vision totally disappeared and cranial MRI showed no recurrence. Cerebral cavernous malformations usually become symptomatic in seizures or focal neurological deficits after intracerebral hemorrhage. Rarely, cavernomas arise from cranial nerves. To the authors' knowledge, this is the first report on a symptomatic cavernous malformation arising from the trochlear nerve and on its successful surgical management.     

Isolated bilateral abducens nerve palsy due to an inflammatory process within the sella and parasellar regions

Isolated bilateral abducens nerve palsy raises concern about a serious intracranial condition. Abducens nerve palsy is a common isolated palsy due to its susceptibility to injury along its long course. Non-traumatic isolated abducens nerve palsy is often caused by a mass that indirectly stretches and compresses the nerve. Pathological processes directly causing bilateral isolated involvement of the abducens nerve are rare. We describe a 24-year-old man who presented with isolated bilateral abducens nerve palsy. Radiological imaging and laboratory tests were consistent with an aggressive bacterial infectious process located in the sellar region with parasellar extension. If promptly addressed, sixth cranial nerve palsy appears to be reversible with aggressive medical therapy and endoscopic sinus surgery.     

Management of non-motor complications in Parkinson’s disease

The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener’s granulomatosis. One patient developed de novo 6^th nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging with CT and MRI and the laboratory provided no clues suggesting active vasculitis. However, in both patients the neuropathies fully recovered in response to standard induction protocols of vasculitis. In the absence of organspecific proof of vasculitis, these treatment decisions were guided by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs.