by Aireen Lacap
The student body and the faculty of St. Luke’s College of Medicine as well as doctors from the St. Luke’s Medical Center (SLMC) gathered for the General Student-Faculty Assembly for the Neurology to discuss a rare case of Ramsay-Hunt syndrome on February 4, 2015.
Diagnosing Ramsay Hunt Syndrome
The representative patient presented by Intern Ferron F. Ocampo was a 26-year-old male who presented with a chief complaint of right facial asymmetry, with accompanying severe right ear pain and dizziness.
Historyindicated he had chickenpox at three years of age. He was a non-smoker, an occasional drinker, a registered nurse, and has been a call center agent for one year, having worked the night shift three months prior to admission to the hospital. He had no recent travel, and indicated multiple homosexual partners.
Upon physical examination, he had normal vital signs but was febrile at 38.1 °C, had warm, moist skin, pink palpebral conjunctivae, and multiple vesicular lesions on the right ear. There was right peripheral facial palsy and loss of taste on the anterior two-thirds of the tongue.
Herpes Zoster Oticus and Ramsay Hunt Syndrome on the right were considered as admitting impression. Medications administered were hydrocortisone, valacyclovir and ampicillin-sulbactam. Upon MRI, there was noted enhancement of cranial nerves (CN) 7 and 8 on the right.
Brainstem encephalitis was considered due to extending infection; therefore, lumbar puncture CSF analysis was requested, as well as referral to an Infectious Disease Specialist, the latter refused by the patient. He was discharged on the eighteenth day with right peripheral facial paralysis but no other symptoms with the diagnosis of Varicella Zoster Virus (VZV) Brainstem Encephalitis and Herpes Zoster Oticus on the right.
Background of Ramsay Hunt Syndrome
According to intern Ocampo, the Varicella Zoster Virus, a member of the Human Herpes Virus 3 family, causes Ramsay-Hunt Syndrome. It is a DNA virus and is contracted via the respiratory tract.
Primary infection occurs as chickenpox, generalized vesicular lesions with a centripetal pattern, and is a self-limiting disease, while secondary infection presents with Herpes Zoster, reactivation of the latent virus, localized vesicular rashes with dermatomal distribution. The latter also occurs with increasing age and immunosuppression.
The disease was first documented by Dr. James Ramsay Hunt in 1907, with symptoms of right facial paralysis, vesicular rash on the external ear, and loss of taste and sensation on the anterior two-third of the tongue.
Weakening of the immune system, stress and surgical procedures may trigger reactivation of VZV and inflammation of ganglion and nerves in the narrow facial canal, which may spread to the facial nerve (CN 7) and vestibulocochlear nerve (CN 8). Anti-viral medications usually given are acyclovir, valacyclovir and famciclovir, which shorten duration of viral shedding, decrease formation of new lesions and accelerate recovery.
There is, however, lower rate of recovery for multiple cranial nerve involvement. Complications include myelitis, encephalitis, ventriculitis, but this occurs commonly with immunocompromised patients. Due to the spread of the viral infection to the brainstem, brainstem encephalitis may occur. This presents with inflammation and multiple cranial nerve involvement.
MRI is preferred over CT scan as imaging modality, but lumbar puncture CSF analysis is recommended if lesion or edema is not seen.
Open Floor Q & A
The floor was opened for questions and several doctors inferred the necessity of including actual figures in the presentation, instead of interpretations.
Dr. Geraldine Mariano inquired about the importance of the low (3%) but positive neutrophilia in the CSF, saying that “we should be more careful” in interpreting such findings. Dr. de Ramos, meanwhile, suggested there might have been a misdiagnosis for allergic sinusitis during the patient’s check-up before admission to SLMC.
Dr. Damian affirmed that it is necessary for antibiotic and antiviral treatment to be administered to counter both types of infection before the symptoms of either disease appears to treat the patient more effectively.
A few present doctors agreed that findings on the tympanic membrane should have been documented.
Reactor Dr. Tiu, an Infectious Disease specialist, recommends ordering all cultures from the patient, and notes the importance of diagnosis, prognosis, public health importance and prevention regarding disease. He emphasized that call center agents should not be stigmatized with contraction of HIV.
He also commended the use of acyclovir due to its distinct effect only on the Herpes family, as it is the most common type of virus. According to him, prevention is currently possible, since the VZV vaccine was approved by the CDC for patients fifty years old and above, but the effect lasts for only five years. There is also a vaccine available for shingles, when only Varicella vaccine was available before.
The student body and the faculty of St. Luke’s College of Medicine as well as doctors from the St. Luke’s Medical Center (SLMC) gathered for the General Student-Faculty Assembly for the Neurology to discuss a rare case of Ramsay-Hunt syndrome on February 4, 2015.
Diagnosing Ramsay Hunt Syndrome
The representative patient presented by Intern Ferron F. Ocampo was a 26-year-old male who presented with a chief complaint of right facial asymmetry, with accompanying severe right ear pain and dizziness.
Historyindicated he had chickenpox at three years of age. He was a non-smoker, an occasional drinker, a registered nurse, and has been a call center agent for one year, having worked the night shift three months prior to admission to the hospital. He had no recent travel, and indicated multiple homosexual partners.
Upon physical examination, he had normal vital signs but was febrile at 38.1 °C, had warm, moist skin, pink palpebral conjunctivae, and multiple vesicular lesions on the right ear. There was right peripheral facial palsy and loss of taste on the anterior two-thirds of the tongue.
Herpes Zoster Oticus and Ramsay Hunt Syndrome on the right were considered as admitting impression. Medications administered were hydrocortisone, valacyclovir and ampicillin-sulbactam. Upon MRI, there was noted enhancement of cranial nerves (CN) 7 and 8 on the right.
Brainstem encephalitis was considered due to extending infection; therefore, lumbar puncture CSF analysis was requested, as well as referral to an Infectious Disease Specialist, the latter refused by the patient. He was discharged on the eighteenth day with right peripheral facial paralysis but no other symptoms with the diagnosis of Varicella Zoster Virus (VZV) Brainstem Encephalitis and Herpes Zoster Oticus on the right.
Background of Ramsay Hunt Syndrome
According to intern Ocampo, the Varicella Zoster Virus, a member of the Human Herpes Virus 3 family, causes Ramsay-Hunt Syndrome. It is a DNA virus and is contracted via the respiratory tract.
Primary infection occurs as chickenpox, generalized vesicular lesions with a centripetal pattern, and is a self-limiting disease, while secondary infection presents with Herpes Zoster, reactivation of the latent virus, localized vesicular rashes with dermatomal distribution. The latter also occurs with increasing age and immunosuppression.
The disease was first documented by Dr. James Ramsay Hunt in 1907, with symptoms of right facial paralysis, vesicular rash on the external ear, and loss of taste and sensation on the anterior two-third of the tongue.
Weakening of the immune system, stress and surgical procedures may trigger reactivation of VZV and inflammation of ganglion and nerves in the narrow facial canal, which may spread to the facial nerve (CN 7) and vestibulocochlear nerve (CN 8). Anti-viral medications usually given are acyclovir, valacyclovir and famciclovir, which shorten duration of viral shedding, decrease formation of new lesions and accelerate recovery.
There is, however, lower rate of recovery for multiple cranial nerve involvement. Complications include myelitis, encephalitis, ventriculitis, but this occurs commonly with immunocompromised patients. Due to the spread of the viral infection to the brainstem, brainstem encephalitis may occur. This presents with inflammation and multiple cranial nerve involvement.
MRI is preferred over CT scan as imaging modality, but lumbar puncture CSF analysis is recommended if lesion or edema is not seen.
Open Floor Q & A
The floor was opened for questions and several doctors inferred the necessity of including actual figures in the presentation, instead of interpretations.
Dr. Geraldine Mariano inquired about the importance of the low (3%) but positive neutrophilia in the CSF, saying that “we should be more careful” in interpreting such findings. Dr. de Ramos, meanwhile, suggested there might have been a misdiagnosis for allergic sinusitis during the patient’s check-up before admission to SLMC.
Dr. Damian affirmed that it is necessary for antibiotic and antiviral treatment to be administered to counter both types of infection before the symptoms of either disease appears to treat the patient more effectively.
A few present doctors agreed that findings on the tympanic membrane should have been documented.
Reactor Dr. Tiu, an Infectious Disease specialist, recommends ordering all cultures from the patient, and notes the importance of diagnosis, prognosis, public health importance and prevention regarding disease. He emphasized that call center agents should not be stigmatized with contraction of HIV.
He also commended the use of acyclovir due to its distinct effect only on the Herpes family, as it is the most common type of virus. According to him, prevention is currently possible, since the VZV vaccine was approved by the CDC for patients fifty years old and above, but the effect lasts for only five years. There is also a vaccine available for shingles, when only Varicella vaccine was available before.