The patient was a 54-year old male retired Air Force pilot. He was in excellent
ID: 99022 • Letter: T
Question
The patient was a 54-year old male retired Air Force pilot. He was in excellent health until he discovered a chronic neurologic disorder which led to his death six months after the onset of symptoms. The initial manifestations were nonspecific with forgetfulness, subtle behavioral changes, headaches, and fatigue. Disequilibrium, a wide-based gait, and double vision developed, and he sought medical attention. There was no history of alcohol use or tick bite. He was never febrile. He was treated with penicillin and tetracycline for presumed Lyme disease or Rocky Mountain spotted fever (RMSF). His symptoms persisted. Serology tests for Borrelia burgdorferi and RMSF were negative. Evaluation by a neurologist revealed findings consistent with cerebellar and brainstem degeneration. Head computed tomogram (CT) scan revealed mild cerebral and cerebellar atrophy but no mass lesions. His social history was significant for his having been a U.S. Air Force pilot for 23 years. He spent several years in Guam and Vietnam. His eating habits were conventional. He had never been transfused, and he had no risk factors for HIV. Routine hematology and chemistry findings were normal; HIV and VDRL test were negative. Lumbar puncture revealed a normal opening pressure, and all tests on the CSF, including cultures, were within normal parameters. No brain biopsy was conducted. He had progressive decline in his neurological status and died. An autopsy was done. The neuropathological exam was pathognomonic for this patient’s diagnosis. Extensive spongiform changes were prominent in the cerebrum, cerebellum, and basal ganglia.17. Name and describe three viruses can cause chronic neurological disorders.
18. The etiology in this particular case is an unconventional agent which causes a non-inflammatory subacute degenerative neurological disorder. What is the name of the etiological agent? Give a description of the etiological agent. What is the disease in humans called?
19. A similar disease occurs in deer and sheep, describe these diseases. Describe an epidemic in cattle which has been associated with a variant of this disease in humans.
20. Describe three possible modes of person-to-person transmission of this agent. The patient was a 54-year old male retired Air Force pilot. He was in excellent health until he discovered a chronic neurologic disorder which led to his death six months after the onset of symptoms. The initial manifestations were nonspecific with forgetfulness, subtle behavioral changes, headaches, and fatigue. Disequilibrium, a wide-based gait, and double vision developed, and he sought medical attention. There was no history of alcohol use or tick bite. He was never febrile. He was treated with penicillin and tetracycline for presumed Lyme disease or Rocky Mountain spotted fever (RMSF). His symptoms persisted. Serology tests for Borrelia burgdorferi and RMSF were negative. Evaluation by a neurologist revealed findings consistent with cerebellar and brainstem degeneration. Head computed tomogram (CT) scan revealed mild cerebral and cerebellar atrophy but no mass lesions. His social history was significant for his having been a U.S. Air Force pilot for 23 years. He spent several years in Guam and Vietnam. His eating habits were conventional. He had never been transfused, and he had no risk factors for HIV. Routine hematology and chemistry findings were normal; HIV and VDRL test were negative. Lumbar puncture revealed a normal opening pressure, and all tests on the CSF, including cultures, were within normal parameters. No brain biopsy was conducted. He had progressive decline in his neurological status and died. An autopsy was done. The neuropathological exam was pathognomonic for this patient’s diagnosis. Extensive spongiform changes were prominent in the cerebrum, cerebellum, and basal ganglia.
17. Name and describe three viruses can cause chronic neurological disorders.
18. The etiology in this particular case is an unconventional agent which causes a non-inflammatory subacute degenerative neurological disorder. What is the name of the etiological agent? Give a description of the etiological agent. What is the disease in humans called?
19. A similar disease occurs in deer and sheep, describe these diseases. Describe an epidemic in cattle which has been associated with a variant of this disease in humans.
20. Describe three possible modes of person-to-person transmission of this agent. The patient was a 54-year old male retired Air Force pilot. He was in excellent health until he discovered a chronic neurologic disorder which led to his death six months after the onset of symptoms. The initial manifestations were nonspecific with forgetfulness, subtle behavioral changes, headaches, and fatigue. Disequilibrium, a wide-based gait, and double vision developed, and he sought medical attention. There was no history of alcohol use or tick bite. He was never febrile. He was treated with penicillin and tetracycline for presumed Lyme disease or Rocky Mountain spotted fever (RMSF). His symptoms persisted. Serology tests for Borrelia burgdorferi and RMSF were negative. Evaluation by a neurologist revealed findings consistent with cerebellar and brainstem degeneration. Head computed tomogram (CT) scan revealed mild cerebral and cerebellar atrophy but no mass lesions. His social history was significant for his having been a U.S. Air Force pilot for 23 years. He spent several years in Guam and Vietnam. His eating habits were conventional. He had never been transfused, and he had no risk factors for HIV. Routine hematology and chemistry findings were normal; HIV and VDRL test were negative. Lumbar puncture revealed a normal opening pressure, and all tests on the CSF, including cultures, were within normal parameters. No brain biopsy was conducted. He had progressive decline in his neurological status and died. An autopsy was done. The neuropathological exam was pathognomonic for this patient’s diagnosis. Extensive spongiform changes were prominent in the cerebrum, cerebellum, and basal ganglia.
17. Name and describe three viruses can cause chronic neurological disorders.
18. The etiology in this particular case is an unconventional agent which causes a non-inflammatory subacute degenerative neurological disorder. What is the name of the etiological agent? Give a description of the etiological agent. What is the disease in humans called?
19. A similar disease occurs in deer and sheep, describe these diseases. Describe an epidemic in cattle which has been associated with a variant of this disease in humans.
20. Describe three possible modes of person-to-person transmission of this agent.
Explanation / Answer
Answer:
17. Measles:
Measles can produce complications such as Subacute Sclerosing Panencephalitis (SSP) which is a rare progressive neurologic disease and begins 2-10 years after infection.
JC virus:
JC virus is activated in immunocompromised people and spreads to the brain, causing progressive multifocal leukoencephalopathy (PML).
HTLV-1:
HTLV-1 infected individuals develops neurological disorder called HTLV associated myelopathy/tropical spastic paraparesis.
18. The etiological agent is prion. Prions are proteinaceous infectious agent that effects the CNS and are inherited. They are transmissible by ingestion, transplant, and surgical instruments and causes spongiform encephalopathies.
The disease in humans is called Creutzfeldt-Jakob Disease.
19. (a) Scrapie, a prion disease in sheep is an invariable fatal and chronic neurodegenerative disease. The animals with scrapie suffer ataxia, convulsions, blindness, anorexia, and eventually death.
(b) The epidemic in cattle which has been associated with a variant of this disease in humans is Bovine Spongiform Encepholopothy (BSE) or mad cow disease. It is a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion.
BSE is often spread through feeding cattle with meats from slaughtered animals such as goats, sheep, and other cattle (ruminants). Prion that is linked to BSE is spread from the slaughtered diseased animal to a healthy one.
The symptoms of BSE are lack of coordination, trouble in walking and getting up, aggression towards other cattle and humans, kick while being milked, skin tremors, have reluctance to cross concrete or turn corners, high stepping, decreased milk production, and weight loss.
20. Three possible modes of person-to-person transmission are cannibalism, corneal transplant and eating infected meat.
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