Case Vignettes in Neurology

PATIENT CASE STUDIES

Case 37 3/12/99 Patient
Case discussion This 33 year-old male was first referred to University of California at Davis in March of 1990, for fever, skin rash, malaise, intermittent chills, and a weight loss of 10 pounds in 12 days. The patient was a College of Veterinary Medicine resident who had two weeks previously autopsied an infected horse. He was treated for diffuse bilateral pneumonia and eventually had recovered well when he presented in September, 1990 with complaints of headache, fevers and chills. Examinations included negative head CT, MRI and lumbar puncture (LP). As his complaints worsened, a repeat LP showed increased pressure, (335 mm water,) but no evidence of infection. Because of continued fevers and increasing headache, he underwent a lateral cervical puncture to obtain CSF which showed an elevated WBC count of 1250 WBC (82% PMN, 18% mononuclears), glucose 31 mg/dL (normal 60 mg/dL; blood glucose 115 mg/dL), protein 70 mg/dL (normal <40 mg/dL) and (+) titer for infection. Over the following 1 1/2 years, he underwent multiple surgical placements of ventricular catheters for intrathecal antibiotic treatment and was clinically stable. In March of 1992, he presented with double vision, inability to swallow, and a left arm weakness for which he was treated with corticosteroids and more antibiotics. His condition was stable for 10 weeks while he was at a rehabilitation hospital. He returned to his physician, however, at the end of June with bronchospasm, pneumonia, paraplegia and severe thoracic back pain. Pathologic Case Correlate: Neuropath Case X3
Summarize the Case in 1-2 sentences. Patient presents with two episodes of constitutional symptoms and rash with diagnosis of infection and treatment with antibiotics. Two years later, patient presented with CNS symptoms localizing to the base of the brain, brainstem, and thoracic cord. Fairly good summary; Additional comments: Please provide age; also provide onset and course (time intensity profile) should be fleshed out more. (this is a 33 yr old male with a 2 year history , initially presenting with an acute or subacute course with headache, rash, fever and malaise after an horse autopsy and who 6 months later developed persistent aseptic meningitis and who 2 years later developed ... Discuss lesion localization on the basis of the physical examination. Episode / Physical Exam findings / Lesion localization March 90 / Pneumonia, skin rash / Lungs ok Sept 90 / LP: increased pressure and CP: infection / blocked communication between lumbar and cervical CSF Infection where ? the question is to localize the lesion! March 92 / Diplopia (CN 3,4 or 6), hard to swallow (CN 9,10), left arm weakness / Brainstem and Left cervical root your answer is unclear. what is your localization for the diplopia (is it the peripheral cranial nerve?) dysphagia ? are you suggesting a problem in the vagus or glossopharygeal nerve? what left cervical root would give rise to left arm weakness ? May 92 / Paraplegia (legs) and Thoracic back pain / Lower thoracic spinal cord compression good; why do you say lower thoracic spinal cord compression (vs upper or middle)? what type of cord compressive lesion is this do you think ? (epidural, subdural, intramedullary ?) Discuss underlying pathogenesis on the basis of clinical course. Longstanding infection developed over months (and years) good: course is chronic and recurrent and this suggests inflammation (likely chronic infection) Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses. Primary diagnosis: Coccidioidomycosis meningitis note coccidiomyocosis meningoencephalitis and epidural abscess Reasoning: primary pneumonia with EN and secondary spread to meninges. The usual variety of Coccidioidomycosis meningitis is inflammation which results in the encasement of the brain substance, particularly the brain stem, in a rigid, highly organized, granulomatous mass of tissue. This last sentence does not seem pertinent to the question. The reasoning is in your first sentence; that this is a chronic infection that enters the body via the respiratory system and can cause persistent chronic meningitis and meningoencephalitis. In addition it is a disease that can infect horses. Alternative diagnoses: meningial infection from TB, sarcoid or other fungi; space occupying lesions less likely: abcess or tumor. if you classify the alternative dx: 1. other chronic infections e.g. TB, fungi 2. other chronic inflammatory processes. eg. vasculitis, sarcoid, 3. other causes of cord compression eg tumor Indicate 2 ancillary tests that would assist in confirming or refuting the clinical diagnosis. Indicate the test results that would confirm the clinical diagnosis. Rule in cocci: Meningial biopsy looking for spherules; serology testing for IgM precipitans. Rule out TB, other fungi: culture CSF Rule out space occupying lesions: CT only 2 tests are asked for. the tests should be to confirm (or refute) your clinical diagnosis (note to r/o tb fungi etc) Indicate complications of the disease and ancillary tests that would help evaluate them. Spread to other tissues: Liver: LFT; Bones: x-ray looking for osteolytic lesions; Lungs: x-ray Hydrocephalus: CT Death: mortality with coccidioidomycosis meningitis is high. good what about strokes?? cranial nerve infarcts ?? encephalitis ? spinal block from cord compression; paraplegia from cord compression and spinal cord infarction Discuss how the underlying pathophysiology is relevant in the management of this patient. Infection causing blockage of CSF flow due to granuloma and increased viscosity from exudate makes antibiotic delivery difficult; intrathecal antibiotics may not even get there. Granulomatous inflammation may be reduced from corticosteriods caused a decreased compression of CNS tissues. note hydrocephalus may be arising because of adhesions in the ventricular system (obstructive) and also by inflammation in the arachnoid granulations causing decreased csf resorption with communicating hydrocephalus. note inflammation of blood vessels including arterioles and arteries with vasculitis is an important pathogenetic mechanism for complications in chronic infections. The vasculitis results in strokes of brain, spinal cord and cranial nerves. Loss of cerebral autoregulation also occurs.

Case 37 3/12/99 Patient

Case discussion

This 33 year-old male was first referred to University of California at Davis in March of 1990, for fever, skin rash, malaise, intermittent chills, and a weight loss of 10 pounds in 12 days. The patient was a College of Veterinary Medicine resident who had two weeks previously autopsied an infected horse. He was treated for diffuse bilateral pneumonia and eventually had recovered well when he presented in September, 1990 with complaints of headache, fevers and chills.

Examinations included negative head CT, MRI and lumbar puncture (LP). As his complaints worsened, a repeat LP showed increased pressure, (335 mm water,) but no evidence of infection. Because of continued fevers and increasing headache, he underwent a lateral cervical puncture to obtain CSF which showed an elevated WBC count of 1250 WBC (82% PMN, 18% mononuclears), glucose 31 mg/dL (normal 60 mg/dL; blood glucose 115 mg/dL), protein 70 mg/dL (normal <40 mg/dL) and (+) titer for infection. Over the following 1 1/2 years, he underwent multiple surgical placements of ventricular catheters for intrathecal antibiotic treatment and was clinically stable.

In March of 1992, he presented with double vision, inability to swallow, and a left arm weakness for which he was treated with corticosteroids and more antibiotics. His condition was stable for 10 weeks while he was at a rehabilitation hospital. He returned to his physician, however, at the end of June with bronchospasm, pneumonia, paraplegia and severe thoracic back pain.

Pathologic Case Correlate: Neuropath Case X3

Summarize the Case in 1-2 sentences.

Patient presents with two episodes of constitutional symptoms and rash with diagnosis of infection and treatment with antibiotics. Two years later, patient presented with CNS symptoms localizing to the base of the brain, brainstem, and thoracic cord.

Fairly good summary; Additional comments: Please provide age; also provide onset and course (time intensity profile) should be fleshed out more. (this is a 33 yr old male with a 2 year history , initially presenting with an acute or subacute course with headache, rash, fever and malaise after an horse autopsy and who 6 months later developed persistent aseptic meningitis and who 2 years later developed ...

Discuss lesion localization on the basis of the physical examination.

Episode / Physical Exam findings / Lesion localization March 90 / Pneumonia, skin rash / Lungs ok Sept 90 / LP: increased pressure and CP: infection / blocked communication between lumbar and cervical CSF

Infection where ? the question is to localize the lesion!

March 92 / Diplopia (CN 3,4 or 6), hard to swallow (CN 9,10), left arm weakness / Brainstem and Left cervical root

your answer is unclear. what is your localization for the diplopia (is it the peripheral cranial nerve?) dysphagia ? are you suggesting a problem in the vagus or glossopharygeal nerve? what left cervical root would give rise to left arm weakness ?

May 92 / Paraplegia (legs) and Thoracic back pain / Lower thoracic spinal cord compression

good; why do you say lower thoracic spinal cord compression (vs upper or middle)? what type of cord compressive lesion is this do you think ? (epidural, subdural, intramedullary ?)

Discuss underlying pathogenesis on the basis of clinical course.

Longstanding infection developed over months (and years)

good: course is chronic and recurrent and this suggests inflammation (likely chronic infection)

Indicate one likely clinical diagnosis. List (or classify) alternative diagnoses.

Primary diagnosis: Coccidioidomycosis meningitis note coccidiomyocosis meningoencephalitis and epidural abscess Reasoning: primary pneumonia with EN and secondary spread to meninges. The usual variety of Coccidioidomycosis meningitis is inflammation which results in the encasement of the brain substance, particularly the brain stem, in a rigid, highly organized, granulomatous mass of tissue.

This last sentence does not seem pertinent to the question. The reasoning is in your first sentence; that this is a chronic infection that enters the body via the respiratory system and can cause persistent chronic meningitis and meningoencephalitis. In addition it is a disease that can infect horses.

Alternative diagnoses: meningial infection from TB, sarcoid or other fungi; space occupying lesions less likely: abcess or tumor. if you classify the alternative dx:
1. other chronic infections e.g. TB, fungi
2. other chronic inflammatory processes. eg. vasculitis, sarcoid,
3. other causes of cord compression eg tumor

Indicate 2 ancillary tests that would assist in confirming or refuting the clinical diagnosis. Indicate the test results that would confirm the clinical diagnosis.

Rule in cocci: Meningial biopsy looking for spherules; serology testing for IgM precipitans. Rule out TB, other fungi: culture CSF Rule out space occupying lesions: CT

only 2 tests are asked for. the tests should be to confirm (or refute) your clinical diagnosis (note to r/o tb fungi etc)

Indicate complications of the disease and ancillary tests that would help evaluate them.

Spread to other tissues: Liver: LFT; Bones: x-ray looking for osteolytic lesions; Lungs: x-ray Hydrocephalus: CT Death: mortality with coccidioidomycosis meningitis is high.

good
what about strokes?? cranial nerve infarcts ?? encephalitis ? spinal block from cord compression; paraplegia from cord compression and spinal cord infarction

Discuss how the underlying pathophysiology is relevant in the management of this patient.

Infection causing blockage of CSF flow due to granuloma and increased viscosity from exudate makes antibiotic delivery difficult; intrathecal antibiotics may not even get there. Granulomatous inflammation may be reduced from corticosteriods caused a decreased compression of CNS tissues. note hydrocephalus may be arising because of adhesions in the ventricular system (obstructive) and also by inflammation in the arachnoid granulations causing decreased csf resorption with communicating hydrocephalus. note inflammation of blood vessels including arterioles and arteries with vasculitis is an important pathogenetic mechanism for complications in chronic infections. The vasculitis results in strokes of brain, spinal cord and cranial nerves. Loss of cerebral autoregulation also occurs.