Acute motor axonal neuropathy following anti-rabies human diploid cell vaccine: A rare case and review

Rationale: Guillain Barre syndrome (GBS) is an acute neurological illness leading to quadriparesis with respiratory involvement. It can be triggered by infections, vaccinations, surgery, trauma, transplantation and drugs. Anti-rabies cell culture vaccines introduced to overcome the high rate of neurological complications associated with tissue based rabies vaccine, can be very rarely associated with GBS. Patient concerns: A 50-year-old female presented with acute severe upper back pain evolving into pure motor quadriparesis following administration of human diploid cell vaccine for rabies. Diagnosis: Acute motor axonal neuropathy variant of GBS following anti-rabies human diploid cell vaccine. Interventions: Intravenous high dose steroids. Outcomes: Patient recovered completely within 1 month. Lessons: Although anti-rabies cell culture vaccines are highly immunogenic and safe, they are rarely associated with GBS. Clinicians should be aware of this link because prompt diagnosis and treatment can result in complete recovery and avoid complications.

Hemi masticatory spasm with facial hemi atrophy and localized scleroderma: Report of a case with bilateral involvement

@#We report a rare case of a 45 year old female with 15 year history of progressive left facial thinning with frequent episodes of involuntary jaw closure and almost continuous rippling movements over her left sided masticatory muscles. There was localized scleroderma, left facial hemi atrophy and left hemi masticatory spasm. Localized scleroderma was proven histopathologically. Electrophysiological studies revealed normal blink reflex on both sides. Her masseter inhibitory reflex was absent bilaterally and surface electromyogram showed spontaneous bursts of high frequency activity over bilateral masseter and left temporalis muscles. The patient responded remarkably with bilateral botulinum toxin injection. This case highlights presence of rare bilateral involvement of HMS especially on EMG and excellent response to botulinum toxin.

Transfer Dysphagia Due to Focal Dystonia

OBJECTIVE: The inability to propel a bolus of food successfully from the posterior part of the oral cavity to the oropharynx is defined as transfer dysphagia. The present case series describes the varied presentation of transfer dysphagia due to focal dystonia and highlights the importance of early detection by following up on strong suspicions. METHODS: We describe seven cases of transfer dysphagia due to focal dystonia. Transfer dysphagia as a form of focal dystonia may appear as the sole presenting complaint or may present with other forms of focal dystonia. RESULTS: Four out of seven patients had pure transfer dysphagia and had previously been treated for functional dysphagia. A high index of suspicion, barium swallow including videofluoroscopy, associated dystonia in other parts of the body and response to drug therapy with trihexyphenidyl/tetrabenazine helped to confirm the diagnosis. CONCLUSION: Awareness of these clinical presentations among neurologists and non-neurologists can facilitate an early diagnosis and prevent unnecessary investigations.

Deep brain stimulation of subthalamic nucleus helps in improving late phase motor planning in Parkinson's disease.

OBJECTIVE: Deep brain stimulation of subthalamic nucleus (DBS-STN) is a well-accepted treatment for Parkinson's disease (PD) but its effect on motor planning in the disease is yet unclear. This study examines the effect of switching the stimulation ON and OFF on components of bereitschaftspotentials in PD. PATIENTS AND METHODS: Scalp bereitschaftspotentials were recorded during self-paced right wrist extensions at Fz, Cz, Pz, C3 and C4 sites in patients on DBS-STN plus medications (DBS-STN group) as treatment modality or on medications only (Med group) and compared with age matched healthy controls. In DBS-STN group, the potentials were recorded in stimulation ON, stimulation OFF, and again after re-switching stimulation ON-2. Offline analysis of potentials was done to calculate peak amplitude, late slope (-500 to 0ms) and early slope (-1500 to -500ms). RESULTS: We observed that the two components of bereitschaftspotentials in stimulation ON state were comparable to those in age matched controls. The late slope was found to be significantly reduced during stimulation OFF as compared to stimulation ON at Cz (p<0.001), C3 (p<0.001) and C4 (p<0.01) electrode sites. This parameter failed to improve on re-switching stimulation ON at Cz (p<0.01). No significant change was observed in early part of bereitschaftspotentials among any of the conditions. CONCLUSION: Our study shows that DBS-STN along with anti-parkinsonian medications helps in improving both components of bereitschaftspotentials in PD. Switching stimulation OFF for fifteen minutes principally affects the late component i.e. the execution part of motor planning; which cannot be reversed by re-switching ON. Thus the chronic and acute effects of switching DBS-STN ON are different and principally affect the later part of motor planning.