The Status of Affected Infraorbital Nerve and Inferior Alveolar Nerve in Patients With Jaw Fibrous Dysplasia: A Clinical and Radiographic Evaluation.

The affected infraorbital nerve (IFBN) and inferior alveolar nerve (IFAN) status in patients with jaw fibrous dysplasia has not been definitely depicted. In this study, the authors try to explore the status of affected IFBN and IFAN in patients with jaw fibrous dysplasia. Ten patients with jaw fibrous dysplasia were included in this study. The complaints of numbness in the IFBA and IFAN innervated area were asked and recorded, and careful clinical examination was performed to evaluate the touch sense, pain sense, pressure sense, and temperature sense in the IFBA and IFAN innervated areas. Computed tomography scans also were performed to evaluate the imaging characteristics of affected IFBA and IFAN. The results showed that 1 patient with maxillary lesion showed complaints of slight numbness, and clinical examination showed that the patient exhibited slight insensitive in pain sense. In addition, 1 patient with mandibular lesion showed relative obvious complaints of numbness, and clinical examination showed that the patient exhibited slight insensitive in pain sense and temperature sense, but not serious. All other patients exhibited no numbness in the IFBA and IFAN innervated area. Although the position and morphology changed in some patients, all neural canal of affected IFBA or IFAN existed and showed no invasion of lesion. Taking these findings together, it further confirmed that evaluation of the function of IFBAN and IFAN is necessary for patients with jaw fibrous dysplasia, and the affected IFBAN and IFAN may should be reserved in most patients with jaw fibrous dysplasia when resecting or recontouring the lesion.

Wisdom tooth extraction causing lingual nerve and styloglossus muscle damage: a mimic of multiple cranial nerve palsies.

The combination of tongue hemianaesthesia, dysgeusia, dysarthria and dysphagia suggests the involvement of multiple cranial nerves. We present a case with sudden onset of these symptoms immediately following wisdom tooth extraction and highlight the clinical features that allowed localisation of the lesion to a focal, iatrogenic injury of the lingual nerve and adjacent styloglossus muscle.

Numb Chin Syndrome.

"Numb chin syndrome" (NCS) refers to new-onset numbness of the lower lip and chin within the distribution of the mental or inferior alveolar nerves. While this focal numbness may be downplayed or even overlooked by patients and clinicians, in the right clinical scenario this may be the presenting symptom of an underlying malignancy. In the absence of any obvious, temporally related dental cause, there are certain conditions that clinicians should consider including orofacial and systemic malignancies as well as several inflammatory disorders. Thorough diagnostic evaluation should always be performed when no clear cause is evident. This paper will discuss the differential, recommended evaluations, and the prognosis, for a patient presenting with NCS.

Congenital hypomyelinating neuropathy with lethal conduction failure in mice carrying the Egr2 I268N mutation.

Mouse models of human disease are helpful for understanding the pathogenesis of the disorder and ultimately for testing potential therapeutic agents. Here, we describe the engineering and characterization of a mouse carrying the I268N mutation in Egr2, observed in patients with recessively inherited Charcot-Marie-Tooth (CMT) disease type 4E, which is predicted to alter the ability of Egr2 to interact with the Nab transcriptional coregulatory proteins. Mice homozygous for Egr2(I268N) develop a congenital hypomyelinating neuropathy similar to their human counterparts. Egr2(I268N) is expressed at normal levels in developing nerve but is unable to interact with Nab proteins or to properly activate transcription of target genes critical for proper peripheral myelin development. Interestingly, Egr2(I268N/I268N) mutant mice maintain normal weight and have only mild tremor until 2 weeks after birth, at which point they rapidly develop worsening weakness and uniformly die within several days. Nerve electrophysiology revealed conduction block, and neuromuscular junctions showed marked terminal sprouting similar to that seen in animals with pharmacologically induced blockade of action potentials or neuromuscular transmission. These studies describe a unique animal model of CMT, whereby weakness is due to conduction block or neuromuscular junction failure rather than secondary axon loss and demonstrate that the Egr2-Nab complex is critical for proper peripheral nerve myelination.

Atypical botulism sparing palsy of extraocular muscles.

Recent studies of patients with botulism found ophthalmoplegia as a characteristic clinical sign. Here we illustrate a very rare case of atypical foodborne botulism with multiple bilateral cranial nerve palsies sparing palsy of extraocular muscles. Therefore, the classical diagnostic pentad of botulism (dry mouth, nausea, vomiting, dysphagia, diplopia, fixed dilated pupils) may be of limited sensitivity in single cases.

Severe cranial nerve involvement in a patient with monoclonal anti-MAG/SGPG IgM antibody and localized hard palate amyloidosis.

We report a patient with severe cranial polyneuropathy as well as sensory limb neuropathy. Biclonal serum IgM-kappa/IgM-lambda gammopathy was found and serum anti-myelin-associated glycoprotein (MAG)/sulfoglucuronyl paragloboside (SGPG) IgM antibody was also detected. Immunofluorescence analysis of a sural nerve biopsy specimen revealed binding of IgM and lambda-light chain on myelin sheaths. No amyloid deposition was detected in biopsied tissues except for the hard palate, suggesting that the amyloidosis was of the localized type and had no relation to the pathogenesis of cranial neuropathy. Our observations indicate that the anti-MAG/SGPG IgM antibody may be responsible for this patient's cranial polyneuropathy, which is a rare manifestation in anti-MAG/SGPG-associated neuropathy.

Familial granulomatous synovitis, uveitis, and cranial neuropathies.

A family is presented that had what is believed to be a previously undescribed syndrome of granulomatous synovitis, bilateral recurrent uveitis, and cranial neuropathies. Affected members included the proband, his brother, father, and probably the decreased paternal grandmother. Disease onset was in childhood. Each had symmetric, boggy polysynovitis of the hands and wrists, resulting in nearly identical boutonniere deformities. Hand radiography in the proband and his brother revealed no erosions or joint destruction despite more than 20 years of disease. Synovectomy specimens in the proband and his brother showed granulomatous inflammation with giant cells. Recurrent, nongranulomatous, acute iridocyclitis with visual impairment afflicted the proband, brother, and father. Apparently corticosteroid-responsive bilateral neurosensory hearing loss occurred in the proband, and a transient sixth cranial nerve palsy in his brother. All members of the family were antinuclear antibody-, rheumatoid factor-, and HLA-B27-negative. Serum angiotensin-converting enzyme levels were within normal limits in all family members. The inheritance pattern of this syndrome is most consistent with an autosomal dominant mode.

Neurological manifestations of the oculodentodigital dysplasia syndrome.

Oculodentodigital dysplasia (ODDD) (MIM 164200) is a rare autosomal dominant inherited disorder affecting the development of the face, eyes, limbs and dentition. Neurological complications are thought to be occasional manifestations of the disorder. This report illustrates the neurological manifestations by a pedigree of two ODDD patients with spastic paraparesis, cerebral white matter hyperintensity and basal ganglia hypointensity. A systematic review of the English, French, German and Italian literature on ODDD is also provided to summarize the neurological manifestations of the disorder. 243 previously described ODDD cases presented a spectrum of neurological manifestation including spasticity (25), subcortical white matter lesions (9) and basal ganglia changes (6) on MRI. Additional findings consisted of gaze palsy and squinting (28), bladder and bowel disturbances (21), visual loss (20) and blindness (4), hearing loss (15), ataxia (11), nystagmus (9), muscle weakness (5) and paresthesias (3). Neurological manifestations, including spasticity associated with MRI changes, are an underrecognized feature in the ODDD phenotype. A clinical guide to the neurological manifestations of ODDD may assist in the assessment of patients with this condition.

Early correction of orbicularis oculi paralysis with an encircling silicone prosthesis.

Fifteen patients with paralysis of the 7th and 5th nerves or the 7th nerve alone were treated with an encircling silicone prosthesis. There has been no serious ocular complication observed over the last 3 years. The prosthesis has been removed in 9 of the 15 patients in whom good 7th nerve recovery has occurred. In the remaining 6 patients, the prosthesis is being well tolerated.

Facial sensibility in adolescents with and without clefts 1 year after undergoing Le Fort I osteotomy.

Static two-point discrimination, pressure, and vibratory threshold values were measured at standard coordinates in the area corresponding to the infraorbital nerve distribution in 59 adolescents 1 year after undergoing LeFort I osteotomy. The patients were divided into three groups: those born with unilateral cleft lip and palate (n = 30; mean age, 18 years; SD = 2); those with bilateral cleft lip and palate (n = 12; mean age, 19 years; SD = 3); and those without a cleft (n = 17; mean age, 19 years; SD = 3). One year after the osteotomy, none of the patients complained of altered sensibility in the maxillary vestibular mucosa, gingiva, or upper lip and cheek regions. No significant differences were found between the mean postoperative sensibility values of patients with and without clefts, indicating that the presence of clefting did not affect sensory recovery. Mean postoperative static two-point discrimination values were higher than preoperative values in all areas tested and in all patient groups. These results suggest that the sensory recovery, although incomplete, was strong enough to mask any subjective feelings of changes in sensory function.

Synergistic divergence and jaw-winking phenomenon.

Synergistic divergence is a congenital syndrome of monocular adduction deficit, with simultaneous abduction of both eyes on attempted gaze into the field of action of the paretic medial rectus muscle. Based on electromyographic data, it has been speculated that it is a neural miswiring disorder related to Duane's retraction syndrome. The association between Duane's retraction syndrome and the Marcus Gunn jaw-winking phenomenon, another neural miswiring disorder, has been previously reported. The authors describe a patient with both bilateral synergistic divergence and Marcus Gunn jaw-winking phenomenon.

Chronic trichloroethylene intoxication: a new approach by trigeminal-evoked potentials?

Trigeminal nerve impairment is frequently seen in chronic trichlorethylene intoxication (TRI). A total of 104 occupationally exposed subjects were selected for study because they were employed at a highly exposed workplace. They were studied by clinical examination and by trigeminal somatosensory evoked potentials (TSEP). Normal values for TSEP were obtained from the study of 52 healthy nonexposed subjects. Facial hypoesthesia, when present, was global and predominant in the mandibular and maxillary nerve areas, associated or not with absent reflexes. A disturbed TSEP was found in 40 subjects which was predictable from their clinical symptoms. Correlation with exposure parameters (duration of exposure, trichlorethanol, and trichloracetic acid urinary rates) was mainly observed in subjects presenting both clinical and electrical alterations. Electrical alterations alone were less predictive. An abnormal TSEP may thus provide objective determination of risk assessment in the absence of clinical signs.

The origin of R2 of the blink reflex recorded on the affected side of patients with complete facial nerve paralysis.

The possible mechanisms contributing to the generation of R2-like response were investigated in 19 patients with complete facial nerve paralysis, where the efferent limb of the reflex arc is absent. The first possibility that potentials produced by the unaffected orbicularis oculi (0.0c) are conducted to the reference electrode taped over the nose was confirmed as the amplitude of R2 on the affected side was significantly reduced when the reference electrode was removed from the nose and taped on the ear lobule. The second possibility of volume conducted potentials produced by muscular generator in temporalis and masseter muscles through trigemino-trigeminal reflex was suggested in some patients based on three reproducible observations: (1) EMG activity of high amplitude could be recorded with electrodes taped over the affected 0.0c muscle during voluntary teeth clenching, (2) R2-like responses were recorded in patients with bilateral complete facial paralysis, (3) R2-like responses were recorded from temporalis and masseter muscles. The contribution of extraocular muscles could be discounted as R2 could be recorded from patients with Möbius's syndrome who had complete bilateral facio-ocular paralysis. The results of the present study further support the greater value of R1--rather than R2--in predicting clinical outcome of patients with peripheral facial nerve palsy.

Incidence of subclinical trigeminal and facial nerve involvement in diabetes mellitus.

We investigated the frequency of subclinical trigeminal and facial nerve involvement in 40 patients with diabetes mellitus and without clinical signs of cranial nerve lesions. 60% of the patients had distal symmetric sensory polyneuropathy which was confirmed by nerve conduction studies. Trigeminal and facial nerve functions were evaluated electrophysiologically using the blink-reflex R1 component (BlinkR-R1), masseter reflex (MassR), the first exteroceptive suppression of the masseter muscle (Mass-ES1), and distal motor latency of the facial nerve (DML VII). Latencies were significantly prolonged for the BlinkR-R1 (p < 0.0001), the Mass-ES1 (p < 0.05), and DML VII (p < 0.005) in diabetics compared with controls. No significant difference was found for the MassR. Prolonged latencies (> mean + 2.5 SD of age-matched controls) were demonstrated for the Mass-ES1 in 12.5%, BlinkR-R1 in 10%, DML VII in 6.2%, and MassR in 5% in individual of patients. Our findings indicate that trigeminal and facial nerve involvement is not infrequent in diabetics, although it is significantly less frequent than limb nerve involvement.

[Foix-Chavany-Marie syndrome in a child caused by a head trauma]. / Syndrome de Foix-Chavany-Marie d'origine traumatique chez l'enfant.

We report the case of a traumatic Foix-Chavany-Marie syndrome (SFMC) which is the cortico-subcortical type of suprabulbar palsy. A 10-year-old boy was brain injured in a traffic accident in August 1996. He was found comatous (initial GCS = 6) without any focal neurological deficit. The hemodynamic situation was stable even though he presented two wounds of the scalp and a hemoperitoneum that required intensive perfusions. The initial CT scan elicited a frontal fracture, ischemo hemorrhagic lesions of the right frontopolar and anterior temporal cortex. On the second day, he developed on the left side a subdural collection and a extradural hematoma which was surgically withdrawn. The comatous state ended on the ninth day. On examination, The child was awake and alert, able to understand spoken and written language but unable to speak. There was masticatory diplegia: the mouth was half open, the patient was drooling, chewing was impossible. The most striking feature was the automatic voluntary dissociation which might be observed on laughing, crying and yawning. The patient was unable to initiate swallowing but reflex swallowing was preserved once food was placed into the pharynx. The child had a deficit of voluntary control of muscles supplied by nerves V, VI, IX, X, XI. These clinical features are the hallmarks of SFMC. The first case was reported in 1837 by Magnus. The syndrome was described by Foix Chavany et Marie in 1926, and called SFMC by Weller (1993). His literature review of 62 SFMC allowed the differentiation of five clinical types: the classical and most common form associated with cerebrovascular disease, a subacute form caused by central nervous system infections, a developmental form, a reversible form in children with epilepsy and a rare type associated with neurodegenerative disorders. Bilateral opercular lesions was confirmed in 31 of 41 patients who had CT or MRI performed, and by necropsy in 7 of 10 patients. As previously reported, the outcome was poor for this boy who recovered very limited orofacial motor abilities. The medical functional readaptation was long et tedious and took in consideration the fact that the speech disturbance was anarthria and not an aphasic or an apraxic one and the age of onset of this acute acquired syndrome.

[Damage to cranial and peripheral nerves following patency restoration of the internal carotid artery]. / Uszkodzenia nerwów czaszkowych i obwodowych po udroznieniu tetnicy szyjnej wewnetrznej.

UNLABELLED: The aim of the study was an assessment of the incidence of injury to cranial and peripheral nerves as complication of patency restoration of the internal carotid artery, and analysis of the effect of peripheral nerve injury on the results of carotid patency restoration. From Oct 1987 to Sept 1999 543 procedures were carried out for restoration of patency of the internal carotid artery. After the operation hypoglossus nerve injury was found in 7 cases (1.4%), vagus injury in 9 (1.8%). Signs of exclusively recurrent laryngeal nerve damage were found in 6 cases (1.2%). Glossopharyngeus nerve was damaged in 2 cases (0.4%), transient phrenic nerve palsy as a result of conduction anaesthesia was noted in 2 cases (0.4%). Damage to the transverse cervical nerve was found in 96 cases (60%). In 2 patients (1.2%) lower position of mouth angle was due to section of the mandibular ramus of the facial nerve. In another 2 cases skin sensation disturbances were a consequence of lesion of the auricularis magnus nerve and always they coexisted with signs of transverse cervical nerve damage. IN CONCLUSION: damage to the cranial nerves during operation for carotid patency restoration are frequent but mostly they are not connected with any health risks and often they regress spontaneously.

Congenital trigemino-abducens synkinesis in a neonate.

Congenital ocular synkinesis syndromes involve aberrant innervation of extraocular and eyelid muscles in a variety of patterns. A rare iteration is trigemino-abducens synkinesis, with only three published cases to date. Here the authors report (with video documentation) the earliest documented age of trigemino-abducens synkinesis and congenital ocular synkinesis in general. A 13-week-old (40-week postmenstrual age) girl presented with rhythmic abduction of the left eye that coordinated with sucking, likely resulting from abnormal embryologic development, causing activation of the lateral rectus by motor fibers of the mandibular branch of the trigeminal nerve.