A Case of Rapid Deterioration: Acute Multiple Sclerosis of the Marburg Type.
Multiple sclerosis (MS) is an inflammatory disorder that selectively affects the myelin sheaths of the conduction pathways while preserving axis cylinders of the central nervous system (CNS). It is thought to he caused by repeated episodes of immune-mediated damage to the CNS and often leads to chronic, progressive neurologic deterioration. MS generally follows a relapsing-remitting course that, with the accumulation of multiple lesions over time, results in progression of symptoms late in the disease. Death early in the course of MS is uncommon with less than 4% of patients dying within five years of onset.
There are acute forms of MS, however, which are virulent, fulminant and associated with high morbidity and mortality. Marburg type (acute multiple sclerosis), Balo' type (concentric sclerosis) and Schilder type are three acute variants of MS which may lead quickly to death; they are differentiated on the bases of clinical, radiographic and pathologic features. The pathological characteristics of the classical relapsing-remitting form of MS are also found in these acute subtypes in association with a variable necrotizing or hemolytic component.
This article focuses on the form of MS referred to as acute MS of the Marburg type. It is rightly considered a "fulminant MS," because it characteristically presents suddenly and with great intensity. Marburg wrote about this type of demyelination in 1906. He described a 30-year-old woman who was previously healthy but suddenly became confused. She had headaches, vomiting, gait unsteadiness, left hemiparesis, hyperreflexia and a left Babinski response.[9,10, 11] She deteriorated rapidly and died 26 days after onset. At autopsy, her brain white matter showed changes of acute demyelination.
In recent years, several case studies describing patients with symptoms described by Marburg have been reported.[7,11,12,20] All patients in these case reports were young (22-49 years old) and all had rapidly progressing CNS symptoms. Only one survived, while the others died 19-72 days from the onset of symptoms.
The following case report is an example of MS of the Marburg type. While this situation occurred several years ago, the purpose of this case presentation is to heighten the awareness of nurses to the possibility of fulminant MS causing rapidly progressive neurological symptoms. The example points out the difficulty in diagnosis and challenges to the caregiver to meet the rapidly changing needs of patient and family.
A 29-year-old Caucasian woman was admitted to the hospital with a three-day history of rapidly progressing left hemiparesis. Upon awakening three days prior to admission she was "feeling drunk and off balance." The next day she noted left arm and leg weakness. Computed tomography (CT) at this time was negative (Figs 1A and 1B). Prior to admission to the hospital, the patient also complained of headache, nausea, fatigue and "vision blackouts" which were described as episodic tunnel vision.
[Figure 1 ILLUSTRATION OMITTED]
The past medical history included migraine headaches following involvement in a motor vehicle accident three years earlier. Two months prior to admission, the patient underwent MRI of the spine to explore complaints of occipital headache. This was reported as normal. The patient was married, a smoker and had three children, one of whom was born four months prior to admission.
On admission, the patient was afebrile with normal vital signs. Neurological examination revealed a drowsy woman with mild confusion. Cranial nerves were grossly intact except for asymmetric, dilated, sluggishly reactive pupils and weakness of cranial nerve VII (facial nerve) resulting in dysarthria. There was dense left-sided spastic hemiparesis with hypalgesia to light pinprick of the left upper and lower extremities. However, she could perceive pinprick on her face and neck. The patient was unable to walk. Reflexes were exaggerated on the left; the plantar response was extension on the left and flexion on the right. The differential diagnosis at this time included inflammatory demyelination, progressive multifocal leukoencephalopathy and primary neoplasm.
The patient underwent testing for syphilis and a full metabolic work-up including calcium, toxicology and heavy metal screens; all were normal. A cerebrospinal fluid (CSF) examination and chest x-ray were performed. CSF examination demonstrated an opening pressure of 10 cm [H.sub.2]O, with 87% lymphocytes, 1% neutrophils, 0 erythrocytes, protein 46 mg/dL, glucose 73 mg/dL, IgG 10.1 mg/dL and oligoclonal bands 2. Gram stain and cultures were negative. Chest x-ray revealed multiple noncavitary and noncalcified pulmonary nodules consistent with metastatic carcinoma or granulomas.
Early nursing priorities were to provide physiologic support and patient comfort, as well as to monitor and gather data to assist with the medical diagnosis and treatment. The nursing diagnoses included acute confusion, impaired verbal communication and impaired physical mobility.
The patient deteriorated. The left-sided spastic hemiparesis worsened and she developed a left hemianopsia and left gaze preference. Pupils continued to be dilated, asymmetric and sluggishly reactive to light. Extraocular movements were normal. The positive left Babinski response remained. A repeat CT scan revealed a white matter lesion in the right parietal lobe, with no edema or mass effect. Magnetic resonance imaging (MRI) revealed a large area of abnormality in the white matter of the right parietal lobe.
The differential medical diagnosis now included a neoplasm, an acute inflammatory, infectious or demyelinating process. The CSF examination pointed to a demyelinating process in light of elevated IgG index and 2 unique oligoclonal bands. HIV serology was negative. Nursing diagnosis at this point also included sensory-perceptual alterations: visual and tactile.
The patient's condition was relatively stable. Vital signs were stable, she was afebrile and her only complaint was of "heartburn." Neurological examination was unchanged, however, pupils were now equal in size, but remained widely dilated. A five day course of plasma exchange was instituted as treatment for possible fulminant MS.
The first plasma exchange was well tolerated, nevertheless, the patient complained of a moderate headache and hiccoughs. On the second day of plasma exchange, the patient reported an improvement in the numbness in the left lower extremity. However, she developed bifrontal headache and vomiting. Neurological examination was unchanged. Analgesics and antiemetics were ordered. Her plan of care now included measures to deal with the nursing diagnosis of pain.
The patient experienced an acute deterioration in her neurologic status preceded by a tonic seizure. The right pupil increased in size and became nonreactive to light. At this point, the patient was transferred to the intensive care unit where intubation and sedation with propofol (Diprivan) were instituted to manage intracranial pressure. She also received a loading dose of phenytoin (Dilantin). Her fixed pupil improved with hyperventilation. Within two hours, the patient responded to voice but was unable to follow commands for some 13 hours. An electroencephalogram revealed a pattern consistent with general anesthesia and no epileptiform abnormalities. Plasma exchange was discontinued after completion of four exchanges, and a five-day course of methylprednisolone, one gram per day for five days, was instituted. A CT scan demonstrated an increase in edema and mass effect involving the right temporal, frontal and parietal lobes. There was effacement of the right lateral ventricle and shift of midline structures to the left. Mannitol (Osmitrol) 12.5 g every eight hours was ordered.
The nurses in the intensive care unit were now dealing with several new nursing diagnoses. These Included altered tissue perfusion: cerebral; decreased intracranial adaptive capacity; risk for trauma: seizures; and impaired breathing pattern.
The patient was more arousable, followed commands with her right side and nodded her head appropriately to questions. She was extubated and made attempts at verbalization.
However on the same day, the patient's condition again deteriorated. The right pupil was fixed and dilated, the left side was completely paralyzed. She moved her right side only in response to stimulation. Both plantar responses were extensor, dolls eyes phenomenon was present and corneal reflexes were intact. The patient was re-intubated and hyperventilated to a p[CO.sub.2] of 25-30 torr. Mannitol 100 g and furosemide (Lasix) 40 mg were given. The mannitol order was 25 g every 6 hours. MRI demonstrated a large confluent area of increased T2 signal within the fight parietal, posterior frontal and temporal white matter (Figs 2A, 2B). Compared to the MRI of three days earlier, there had been a mild increase in the mass effect and slight dilation of the temporal horns. A tiny new lesion involving the posterior right thalamus and a slight increase in periventricular T2 signal adjacent to the atrium and occipital horn of the left lateral ventricle appeared. A CT scan at this time demonstrated an increase in the mass effect and edema which now involved the right occipital lobe (Figs 3A, 3B).
[Figures 2, 3 ILLUSTRATION OMITTED]
The patient, although slightly improved, was drowsy. She moved her right sided limbs to command; her Glasgow Coma Scale score was 11. Later that day, she developed chest pain. An electrocardiogram and chest x-ray were normal. A transient drop in [0.sub.2] raised suspicion of a pulmonary embolism. Full leg stocking and sequential compression were applied. The nursing diagnosis of impaired gas exchange was added.
The patient's neurologic condition deteriorated again. Early in the morning, the patient did not respond to verbal stimulation. Right-sided weakness was now detected, and she had a right gaze preference. No papilledema was present. Later in the day, the patient's right pupil again became widely dilated and nonreactive to light, but improved following hyperventilation and mannitol. The patient was now agitated with suctioning and when visitors were in the room. After a day of fluctuating neurologic status and aggressive measures, both pupils became fixed and dilated.
CT scan at this time was consistent with progressing mass effect with uncal and cerebellar tonsillar herniation (Figs 4A, 4B). Despite efforts to control intracranial pressure, brain death ensued.
[Figure 4 ILLUSTRATION OMITTED]
Caring for this patient was an emotional challenge for all involved. The physicians and nurses were faced with responding to a patient whose condition was very labile and unrelenting. Her neurological status fluctuated dramatically. Although the diagnosis of fulminant MS was highly suspected early in the course of hospitalization, it was not until the seventh day of hospitalization that this became generally accepted.
A multidisciplinary team enhanced our abilities to manage the patient's symptoms and optimize the treatment plan. Members of the team included neurologists, neurosurgeons, critical care physicians, dietitians, nurses, chaplains, pulmonologists and social workers. Providing emotional support for the patient, and her family, in the face of an unknown and potentially fatal diagnosis was particularly difficult for the nursing staff members. The nurses were challenged to support the patient and family in coping with anxiety, fear, hopelessness, powerlessness and the grief process.
The patient's inconsistent response to treatment made it difficult for the team to foresee the outcome for this patient. Therefore, it was almost impossible to prepare the family for what quickly became the fatal consequences of this disease. The family was kept informed of the patient's changing condition. Family visiting time was unlimited. Clergy were involved in supporting the patient's family and were extremely helpful when her condition deteriorated to the point of brain death.
Pathologic examination of the brain indicated an acute demyelinating process, compatible with Marburg variant of multiple sclerosis (Fig 5). There were massive, confluent areas of active demyelination involving right parieto-occipital to right fronto-temporal regions, with extension into the right splenium of corpus callosum. Generalized moderate cerebral edema with early bilateral uncal herniation and herniation through the left Kernohan's (cerebral peduncle) notch were present. Microscopic examination demonstrated severe myelin loss (Fig 6).
[Figures 5-6 ILLUSTRATION OMITTED]
The Marburg variant of MS usually follows a rapidly progressive or stepwise downhill course. Focal neurological signs are similar to those seen in chronic MS, accompanied in some cases by hemiplegia, dysphasia, hallucinations, confusion or seizures. These cases must be differentiated from other diseases including cerebral tumors,[5,6,18] stroke, cerebral abscess, parasitic infections, viral encephalitis and acute disseminated encephalomyelitis.[8,15,19] In contrast to acute disseminated encephalomyelitis, fever and meningeal signs are absent although headache may be present, and there is no history of preceding infection or immunization.[8,15,19] The CSF protein level and lymphocyte count are normal or moderately elevated, with elevated levels of IgG and oligoclonal bands present less often than in chronic MS.
The diagnosis of acute, fulminant MS is suggested by evidence of multiple, irregularly enhancing lesions in the periventricular and subcortical white matter areas on MRI. There is often a pattern of diffuse, nonperivenous demyelination on microscopic examination of the brain tissue. The microscopic differences between the fulminant and typical forms of demyelination in patients with MS are that in the fulminant forms, the plaques are all of the same age, and there is a tendency toward confluence of the areas of demyelination resulting in large plaque formation.[7,12] Pathologic examination in acute cases of the Balo' type have shown concentric lamellar demyelination similar to rings in the trunk of a tree.[2,6,9]
While a biopsy is rarely necessary for diagnosis in typical relapsing remitting MS, fulminant demyelinating disease may present as a solitary, rapidly expanding mass lesion with radiographic enhancement and features suggestive of a neoplasm and biopsy is often necessary for definitive diagnosis.[5,13,16] The pathologist must he vigilant for the possibility of demyelination and avoid an incorrect diagnosis of brain tumor. An incorrect diagnosis of brain tumor may lead to inappropriate treatment with radiotherapy which may lead to serious long-term consequences particularly in younger patients.
Research into the causes of Marburg type of MS continues. A recent report suggests that a developmentally immature myelin basic protein may be implicated in the disease. There may be an associated genetic factor influencing the changes seen in this protein.
The use of plasma exchange in the treatment of MS is an experimental therapy. While it has been reported of benefit in some series,[3,17] research is yet ongoing. Mayo Clinic is conducting a National Institute of Health supported study to determine if plasma exchange can improve recovery from severe acute attacks of demyelination in patients with MS or other demyelinating conditions which fail to respond to traditional high dose intravenous steroids. The mechanism of action of plasmapheresis in acute MS is not known. It is speculated that it removes soluble factors including antibodies, pro-inflammatory cytokines and immune complexes that acutely damage or facilitate damage of oligodendrocytes or myelin sheaths, and perhaps influence blood brain barrier permeability.[3,17]
Solumedrol use in the acute types of MS has also shown some positive results.[1,6,12] This treatment is now widely regarded as the treatment of choice for attacks of all forms of MS; this was not the case when the patient in the case study was treated. Hopefully the new millennium will see causes and treatments identified for all types of this disease.
The diagnosis of MS is generally made in conjunction with MRI, CSF studies, neurological examination, meticulous history taking and after ruling out other neurologic diseases. In the typical course of MS, making the diagnosis quickly is not nearly as important as making the correct diagnosis. However, when the course of MS is rapid and unrelenting, as it is in Marburg's type of MS, making the correct diagnosis is essential to early initiation of treatment and efforts to prevent a cascade of devastating neurologic events.
The authors would like to acknowledge Drs. Jimmy Fulgham, John Noseworthy, Joseph Parisi and Brian Weinshenker for reviewing this article for medical accuracy. Leann Scroggins, MSN, RN, Joyce Nelson, MS, RN and Jenny Kennedy, RN are thanked for their critique of the nursing aspects of this manuscript.
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[20.] Wood DD, Bilbao JM, O'Connors P, Moscarello MA: Acute multiple sclerosis (Marburg type) is associated with developmentally immature myelin basic protein. Ann Neurol 1996; 40(1):18-24
Questions or comments about this article may be directed to: Jan Jasperson, MA, RN, Saint Mary's Hospital, A Mayo Foundation Hospital, 1216 2nd Street SW, Rochester, Minnesota 55905. She is a nurse manager in the Neuroscience Intensive Care Unit.
Anne G. Jones, MSN, RN, is a neuroscience clinical nurse specialist at Saint Mary's Hospital, A Mayo Foundation Hospital in Rochester, Minnesota.
Copyright [c] American Association of Neuroscience Nurses 0047-2603/96/3006/0350$1.25
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|Author:||Jasperson, Jan; Jones, Anne G.|
|Publication:||Journal of Neuroscience Nursing|
|Date:||Dec 1, 1998|
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