Case Vignettes in Neurology

PATIENT CASE STUDIES

Case 24 3/5/99
Case discussion Patient Case discussions This 54-year-old man sustained at age 40 a compression fracture of the T12 and L1 vertebral bodies in a 1975 motor vehicle accident. This resulted in flaccid paraplegia with sensory loss below mid-thigh and loss of bowel and bladder control. He had multiple complications of this paraplegia including sacral decubitus ulcer and chronic urinary tract infection leading to nephrectomy. He was admitted on 8/25/89, after he had been found alone and unattended in his apartment with decreased attention and what appeared to be new weakness of the right upper extremity. Examination on admission revealed that he was lethargic but arousable.with BP 100/58, pulse 84, respirations 12. He was poorly attentive. He was oriented to hospital and to the month and year. He was aphasic with mild defects in comprehension and word finding difficulties. Strength in the upper extremities was normal in the left and decreased 4/5 on the right. There was a 5x2 cm sacral decubitus and flaccid paraplegia with absent sensation in both legs. Two days after admission he became poorly responsive with a temperature of 40, labored respirations, blood pressure 80/50, chills, nausea and vomiting. He was able to respond appropriately to commands and to give yes and no answers to several questions. Neurological examination revealed flaccid lower extremity paralysis with right upper extremity weakness. His decreased level of consciousness on admission improved with hydration, but he still remained somewhat obtunded. He continued to respond to pain. He moved his upper extremities, but 4 days after admission he was found to be unresponsive with pupils 6 mm and fixed. Oculocephalic reflexes were not present. Pathologic Case Correlate: Neuropath Case X1
Summarize the case in 1-2 sentences. This patients was admitted with to the hospital with a prior history of spinal cord damage which most likely masked the onset of his current problem. The patientÕs new problem consisted of a focal central neurologic signs of acute onset in the central nervous system consistent with the signs of a mass occupying lesion. include the age and the course: This is a 54 yr old male with a history of a traumatic cauda equina/conus medullaris syndrome with severe flaccid paraplegia and neurogenic bladder 14 years before who now presents with apparent sudden onset of inattention, aphasia, right upper extremity weakness and a long standing sacral ulcer. Course progressed with over a few days with persistent fever, hypotension, respiratory failure, and subsequent coma and absent brain stem reflexes. Remember: the summary is a summary of the physical signs and course; You do not indicate the onset and course of the new problem; you do not give the information one would need from the summary to localize the lesion Discuss lesion localization on the basis of physical exam. This patient has two separate problems. The first is a spinal cord lesion due to a motor vehicle accident in 1975, which resulted in loss of sensation below mid-thigh, flaccid paralysis, and loss of bladder and bowel control. This problem probably affected the diagnosis of this patients new problem. His current problem is a focal lesion in the CNS, most likely occurring on the left side of the brain based on his specific deficits in word and comprehension problems, as well as right upper extremity weakness. where is the site in the nervous system of the tramatic lesion; is it cns or pns? if so where? you are correct as regards the second lesion; aphasia suggests moreover involvement also of cortex not just left hemisphere Discuss underlying pathogenesis on the basis of clinical course. The course of this patients problem is rapidly progressive over a four day course after presenting with an acute onset. In addition, he has signs of specific localization and increased intercranial pressure. This is suggestive of a focal lesion of increasing size. This mass lesion most likely is localized in either the posterior fossa or near the foramen magnum and is likely to be blocking the cerebral aquaduct. remember sudden onset and course suggests trauma, or vascular (we are not told what the onset was, patient was "found" worsening over a few days suggests inflammatory or mass lesion; loss of brain stem reflexes (oculocephalics) and dilated pupils suggests involvement of brain stem and of third cranial nerves. suggestive of diencephalic or transtenorial herniation but could also arise as a consequence of anoxia Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses. A brain tumor, most specifically a meningioma, is the most likely diagnosis. A meningioma is most likely, because of its onset and the fact that it is occurring in a 40-year-old patient (most common age of onset is 40 to 60). It is probably difficult to observe the onset in this patient, because of his prior paraplegia. As he progresses over the course of four days, he progressively shows the signs of late stage increase in intercranial pressure going from nausea, vomiting, chills, high fever and lethargy, to unresponsive with bilaterally enlarged pupils. The differential diagnosis includes any mass occupying lesion in the brain including a primary or metastasizing brain tumor, brain abscess, subdural empyema, and a cerebral vascular accident (either ischemic or hemorrhagic), or a subdural hematoma. why do you suggest a meningioma as your main clinical dx; I know this is the pathological diagnosis; however the clinical diagnosis is not the one you do at autopsy; it is one that you do at the bedside on the basis of the clinical course and the physical signs. meningioma is a slow growing tumor and is not the one that you would have likely thought of when you initially evaluated the patient; your explanation is likely correct that the progressive nature of the tumor was missed over years because of his prexisting deficit, but there is not enough information inthe history and course for you to come up with this dx. Remember the clinical dx has to follow from the onset and course and the findings on exam. What explanation do you think was responsible for the apparent sudden worsening ? The last sentence of the first paragraph of this answer does not seem to be related to the question. You can say that the onset is unclear because of the circumstances; consequently the lesion may be one that caused an sudden or acute course (eg vascular including hemorrhage, or trauma), to one that is subacute (including tumor and inflammation) to one that has been chronic progressive with sudden worsening (eg bleeding in a slow growing tumor) I agree clinical course suggesting herniation, suggests a mass or space occupying lesion. The would include tumor, abscess or hemorrhage. Indicate 2 ancillary tests that would assist in confirming or refuting the clinical diagtnosis. Indicate the test results that would confirm the clinical diagnosis. plain skull XR Ð diagnostic for meningioma CT Ð most diagnostic for meningioma; shows space occupying lesion readily MRI Ð shows space occupying lesion readily LP Ð CONTRAINDICATED D/T INCREASED INTERCRANIAL PRESSURE the question calls for 2 tests only; Indicate the complications of the disease and ancillary tests that would help evaluate them. death respiratory and cardiovascular failure d/t increased intercranial pressure Ð monitor patient closely secondary metastasis of tumor Ð do appropriate tests such as a CT or CXR. complications also include focal deficits (weakness, aphasia, apraxia, etc) Discuss how the underlying pathophysiology is relevant in the management of this patient. The most imprtant pathophyaiologic feature in working with this patient is his increased intercranial pressure. This must be alleviated by surgical ressection. If removed, it is possible to recurr over time. With this patient however, it is unlikely that resuccetion would work, because he already expresses late stage signs, and the tumor is most likely advanced beyond management. Agreed; pathophysiology of ICP is important; what are the ways that a mass lesion cause raised icp? And if your clinical dx was a brain tumor than the pathophysiological discussion also revolves around the cell type the tumor arises from,the role of tumor suppressor factors and oncogenes in development of these tumors and the strategies of how treatment may be effective.
Summarize the Case in 1-2 sentences. This 54 year old male was admitted with to the hospital with an acute onset of right upper extremity weakness, lethargy, inattention, mild deficits of comprehension and word finding with aphasia. The course was progressive over a couple of days with a high fever, difficulty breathing, hypotenison, and eventual coma without reflexes. His case was aggrevated by a prior history of spinal cord damage which most likely masked the onset of his current problem. summary is on the long side. you dont know the course to be progressive so why do you say it is? Discuss lesion localization on the basis of the physical examination. This patient has two separate problems. The first is a traumatic lesion involving the CNS, specifically the spinal cord, a cauda equina or conus medullaris syndrome, due to a motor vehicle accident in 1975. It resulted in loss of sensation below mid-thigh, flaccid paralysis, and loss of bladder and bowel control. This problem probably affected the diagnosis of this patients new problem. His current problem is one of a focal lesion in the CNS, most likely occurring on the left side of the brain based on his specific deficits in word and comprehension problems, as well as right upper extremity weakness. Furthermore, his bout of aphasia most likely involves the cortex as well, possibly due to increased intercranial pressure. good Discuss underlying pathogenesis on the basis of clinical course. The patients problem is slowly progressive over a course of several days, thus it is suggestive of a space-occupying lesion or inflammatory lesion. The loss of the occulocephalc reflexes and the dialated pupils indicates that the brain stem is invloved and there is likely to be increased intercranial pressure leading to either diencephalic or transtentorail herniation. Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses. good; the exact course preceding the terminal deterioration is unclear. perhaps you should say that. Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses. Likely clinical diagnosis: primary brain tumor Note: It is probably difficult to observe the onset in this patient, because of his conus medullaris syndrome. This diagnosis may be further supported by the fact that the sudden worsening may be due to an intercranial hemorrhage because of a ruptured tumor blood vessel. The differential diagnosis includes any mass occupying lesion in the brain including a primary or metastasizing brain tumor, brain abscess, subdural empyema, and a cerebral vascular accident (either ischemic or hemorrhagic), or a subdural hematoma. I think you can say tumor if you say mass lesion; but you dont have the data to say primary brain tumor; differential is good; Indicate 2 ancillary tests that would assist in confirming or refuting the clinical diagnosis. Indicate the test results that would confirm the clinical diagnosis. 1) CT shows space occupying lesion readily, especially a brain tumor, possibly with evidence of fluid accumulation around the lesion due to blood or edema. 2) MRI shows space occupying lesion readily, especially a brain tumor, possibly with evidence of fluid accumulation around the lesion due to blood or edema. good Indicate complications of the disease and ancillary tests that would help evaluate them. 1) death 2) respiratory and cardiovascular failure d/t increased intercranial pressure monitor patient closely 3) secondary metastasis of tumor do appropriate tests such as a CT or CXR. 4) Further focal deficits of progressive weakness, aphasia, apraxia. good Discuss how the underlying pathophysiology is relevant in the management of this patient. The most important pathophyaiologic feature in working with this patient is his increased intercranial pressure. This must be alleviated by surgical ressection. If removed, it is possible to recur over time. With this patient however, it is unlikely that ressection would work, because he already expresses late stage signs of hypotension, high fever, coma, and loss of occulocephalic reflexes. Thus, the tumor is most likely advanced beyond management. A mass lesion may increase the intercranial pressure by several means. First, it may block off the cerebral aquaduct and cause hydrocephalus. Secondly, if a tumor exists, it may cause an intercranial bleed, and if slow enough, then its course may occur over several days. Furthermore, simple expansion of the tumor may increase intercranial pressure over time. Brain tumors arise from a variety of cell types; these include the astrocytoma, oligodentroma, ependymomas, germinomas, meduloplastoma, meningioma, or a schwanomma. It however is likely to be an astrocytoma, in particular a high-grade astrocytoma, for several reasons. First, astrocytomas are the most common primary neoplasms, are usually supratentorial in origin, and that he experiences motor weakness, and aphasia, and problems with comprehension suggesting infiltration into adjacent tissue. This type of tumor typically has defective p53, RB1, NF1, ras, and c-myc gene changes among other things. Treatment for such a patient would include surgery to obtain a pathological diagnosis, as well as, removing an aggressively enlarging lesion. In addition to surgery, radiation and chemotherapy (such as nitrosurea) available. There is no cure for an astrocytoma, but the length, and quality of the patient’s life may be lengthened. Average survival with treatment is 1 year and 25% at 2 years. good Remember the object is to develop a strategy based on the patient's presentation and not to work backwards from what you know the diagnosis to be!