Tensor Tympani and Stapedial Tendon Reconstruction for Postoperative Hyperacusis after Tendon Lysis for Middle Ear Myoclonus.

OBJECTIVE: To present a method for repair of the stapedial and tensor tympani tendons in a patient with hyperacusis after a tendon lysis procedure. PATIENTS: A 71-year-old professional musician who presented to clinic with debilitating hyperacusis following a tensor tympani and stapedial tendon lysis procedure to treat middle ear myoclonus. INTERVENTIONS: A novel procedure for reapproximation of the tensor tympani and stapedial tendons into their native insertion points using periosteal grafts and nitinol wire. MAIN OUTCOMES MEASURES: Stapedial reflex measurements, uncomfortable loudness level, and subjective patient experience. RESULTS: Postoperatively, the patient had objective improvement in hyperacusis with return of acoustic reflexes in the affected ear and durable improvements in their frequency-specific uncomfortable loudness levels. CONCLUSIONS: This case describes the debilitating complication of hyperacusis following tendon lysis and highlights the importance of maximizing behavioral and medical measures prior to undergoing surgical intervention for middle ear myoclonus.

Spinal segmental myoclonus following spinal surgery.

A 44-year-old male was admitted with L5/S1 spondylodiscitis complicated by a posterior epidural abscess that was compressing the thecal sac with severe narrowing of the canal and compression of the cauda equine. He underwent computed tomography (CT) guided drainage followed by L5/S1 decompression laminectomy and was started on a 6-week course of intravenous antibiotics with good response. He remained well and afebrile with inflammatory markers showing improvement. During this period, he developed intermittent myoclonic movements of right lower limb with severe pain over the back radiating to the gluteal region which hindered his rehabilitation potential. He was diagnosed with spinal segmental myoclonus based on clinical findings and history of recent spinal surgery. He was successfully treated with a course of clonazepam and continues to make functional improvements during his rehabilitation program.

Progressive Encephalomyelitis with Rigidity and Myoclonus Resolving after Thymectomy with Subsequent Anasarca: An Autopsy Case.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an autoimmune disorder involving the brainstem and spinal cord and is sometimes associated with thymoma. We encountered a 75-year-old woman with typical PERM features, glycine receptor antibody, and thymoma. Her neurologic symptoms improved after thymectomy, but she unexpectedly developed anasarca with massive pleural effusions and hypoalbuminemia and finally succumbed to death. The autopsy showed edema and mononuclear infiltration in the pleura but no neuropathological findings typical of PERM. Effective treatment of PERM can reverse the neuropathological signs of encephalomyelitis. The autoimmune nature of anasarca is possible but not proven.

Middle ear myoclonus cured by selective tenotomy of the tensor tympani: strategies for targeted intervention for middle ear muscles.

OBJECTIVE: To describe a case of middle ear myoclonus that was successfully cured by selective transection of the tensor tympani (TT) without sectioning the stapedius tendon (ST) and to review previously reported cases, elucidating precipitating factors for interventions targeting middle ear muscles. DATA SOURCES: One case we encountered and a recent systematic review published in 2012. STUDY SELECTIONS: In addition to our case, 23 cases identified by the previous systematic review regarding middle ear myoclonus in which surgical interventions were conducted. DATA SYNTHESIS: Outcomes for selective tenotomy of TT or ST were analyzed focusing on the following 6 preoperative factors: 1) history of facial palsy, 2) provoking factors for tinnitus, 3) auscultation of the ear, 4) movement of the ear drum, 5) complication with palatal myoclonus, and 6) confirmation of myoclonus during surgery. Among these, the first 2 factors represented significant factors for selective tenotomy of ST (p < 0.05 and p < 0.01, respectively). Furthermore, no auscultation of the ear was significant for selective tenotomy (p < 0.01), specifically for ST. Confirmation of muscle contraction during surgery contributed significantly (p < 0.01) to targeted intervention, but selective tenotomy of TT was successfully performed in 3 cases without such confirmation by confirming variations in compliance with tympanometry CONCLUSION: Assessment of the history of facial palsy, provoking factor of tinnitus, auscultation of the ear, and confirmation of myoclonus during surgery appear helpful in predicting which middle ear muscle is undergoing myoclonus. Furthermore, long-time-based tympanometry offers objective information for planning targeted intervention for middle ear muscles and clarifying clinical outcomes.

Clinical and neurophysiological improvement of SGCE myoclonus-dystonia with GPi deep brain stimulation.

Myoclonus-dystonia (M-D) is characterized by early onset myoclonus and dystonia. It is thought to be subcortical in origin. Response to oral medications may be incomplete, such that deep brain stimulation (DBS) surgery to the globus pallidum interna (GPi) or ventral intermediate thalamic nucleus (VIM) may be considered. The optimal site is not known. The physiology and surgical response for a 63-year-old woman who underwent GPi DBS for M-D with onset at age 2 and related to a mutation in the epsilon-sarcoglycan gene (SGCE) is described. She showed excellent clinical and neurophysiological improvement of both myoclonus and dystonia, suggesting that modulation by DBS is effective even after long disease duration and only partial response to oral medications.

Cerebellum can be a possible generator of progressive myoclonus.

A 19-month-old girl presented with progressive myoclonic jerking of both proximal lower extremities. On her brain magnetic resonance imaging (MRI), the authors found an ill-defined mass involving cerebellar vermis and the right middle cerebellar peduncle. ( 11)C-methionine positron emission tomography (PET) showed no abnormalities, but (18)F-fluorodeoxyglucose ((18)F-FDG) PET revealed a well-defined hypermetabolic focus. Depth electrodes were inserted deep into the mass, which recorded focal slow waves associated with the clinical myoclonus. Following the removal of the tumor, the myoclonus was completely resolved with no neurological deficit. Here, the authors present a case showing progressive myoclonus associated with a cerebellar ganglioglioma with the electrophysiological data, which provides strong supportive evidence that the cerebellum can be a myoclonus generator.

Treatment for severe palatoclonus by occlusion of the eustachian tube.

PURPOSE: Surgical blocking of the eustachian tube is presented as an ultimate treatment option in a 11-year-old suicidal boy with a therapy-resistant, persistent clicking tinnitus caused by myoclonus of the levator veli palatini. PATIENT: An 11-year-old boy decompensated psychologically as a result of loud and objective tinnitus. The tinnitus could be heard easily by an examiner by bringing his own ear at a distance of approximately 20 to 30 cm to the left ear of the patient. No neurologic etiology for the tinnitus could be traced. Pediatric psychiatric evaluation resulted in a recommendation to perform, as a last resort, an experimental surgical option like blockage of the eustachian tube. INTERVENTION: Treatment with Tegretol (Novartis, The Netherlands) had no effect. Treatment with Dysport (Ipsen) botulin toxin with 30 to 60 U was temporarily effective. Finally, 60 U were not effective anymore. As last refugium, a surgical blockage of the eustachian tube has been performed, first with bone cement and later by a more conventional surgical blockage of that bony tube. OUTCOME: After surgical blockage of the bony part of the eustachian tube, the objective tinnitus disappeared. Blockage of the protympanum by bone cement resulted in only 1 year of successful blocking. After recurrence of the tinnitus combined with aeration of the middle ear, a second surgical transcanal approach was successful in blocking the eustachian tube. With a grommet, the hearing level remained within 10 dB for 0.5 to 8.0 kHz.

Electrophysiological and positron emission studies in a patient with cortical myoclonus, epilepsia partialis continua and motor epilepsy.

A patient is described who had a combination of stimulus-sensitive cortical myoclonus, epilepsia partialis continua, and Jacksonian motor epilepsy. He eventually required surgery because of the severity of his seizures. Electrophysiological recordings made before and during surgery, and PET scans performed before surgery identified an abnormal area of cerebral cortex in the postcentral parietal region. It is suggested that the stimulus-sensitive myoclonus arose because input into this region from peripheral sensory afferents produced an abnormal discharge which was fed forwards via cortico-cortical connections to the precentral motor cortex, to produce a reflex muscle jerk. The epilepsia partialis continua may have been caused by spontaneous discharges arising in the same region of parietal cortex. Both forms of jerking disappeared after resection of this part of the cortex.

[The problem of spinal myoclonus (author's transl)]. / II problema delle mioclonie spinali

A case of the so called "spinal myoclonus" in a 71 year-old-man affected by lung carcinoma is reported. Clinical manifestations and comparison with similar previously described in literature induce to believe in the existence of a myoclonic syndrome, whose pattern seems to give support to the attribute of "spinal", at least as conventional term. An involvement of intercalated neurons is advanced as pathogenic ground. Anyhow, possible suprasegmental implications are also considered.