| Case 18 3/1/99 Parkinson’s disease |
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| Case discussion This 58-year-old right-handed woman was well until six weeks prior to her first hospitalization when she noted increasingly illegible handwriting followed by weakness and clumsiness of her right hand when typing. She began to experience brief episodes during which she could not recall the appropriate word or phrase while speaking or would completely lose her train of thought in a conversation. Symptoms steadily increased until the day of hospitalization when a seizure occurred, during which involuntary convulsive movements began in the right hand, then spread to involve the right arm and entire right half of the body. This march would progress over a few seconds. Head and eyes turned to the right and convulsive movements became generalized. On examination after admission to the hospital she was lethargic but rousable. She was mute. She did not follow appendicular or midline commands. Ophthalmoscopy revealed bilateral papilledema. Visual fields appeared intact to confrontational threat. There was right sided facial weakness sparing the forehead and marked weakness of the right upper and lower extremity (2-3/5) with decreased muscle tone. Weakness was most marked distally in right upper extremity. DTRS were brisk 2+ on the left and 3+ on the right. The right plantar reflex is extensor, the left flexor. Sensation was intact bilaterally to painful stimuli. After treatment, her symptoms and signs improved to some extent. Her right lower face and right extremities were mildly weak with right hyperreflexia and Babinski sign. Upon simultaneous presentation of visual stimuli in both lateral visual fields, stimuli on the right were extinguished. She had a profound right hand apraxia in that she was unable to perform simple manual tasks such as holding a pen or turning a coin over in her fingers. Her aphasia was both receptive and expressive; she was unable to follow complicated directions and could not find the right words to express herself. She became extremely frustrated with her speech problem and substituted long colloquial phrases when unable to find the correct single word. She was able to walk well except for difficulty turning suddenly. Decreased sensory perception to all modalities was noted in the right face and upper extremity. Over the next few months her condition progressed. She was re-hospitalized for increasing lethargy. Examination showed right homonymous hemianopsia, left pupil dilated and sluggishly responsive to light, paresis of the right lower-face, spastic quadriparesis more marked on the right, right hemi-sensory loss, and bilateral Babinski signs. She rapidly became comatose. Blood pressure rose to 240/140 and pulse slowed to 40/min. Both pupils became dilated and fixed to light. Pathologic Case Correlate: Neuropath Case 6 |
This is a 58 year old woman with a progressive six week course of right hemiparesis, lethargy with papilledema episodes of word finding difficulty and confusion with a right sided Jacksonian seizure with march and secondary generalization followed by postictal mutism,. Post treatment, there were multiple left cortical deficits and a rapid, progressive deterioration over the course of a few months. good Left cerebral hemisphere (right hemiparesis, language difficulties) Cortex of left frontal lobe (Right simple motor seizure) Left perisylvian cortex (mutism) Possible obstruction to CSF flow in ventricular system (papilledema) Left parietal cortex and left temporal cortex ·region of optic radiations (Right homonymous hemianopsia) Left occipital cortex or left hemisphere (RHH) Left parietal cortex or left hemisphere (Right hemisensory loss) Transtentorial herniation and or diencephalon (Contralateral motor pathway involvement, hypertension, coma, fixed pupils and bilateral corticospinal signs.) good Subacute crescendo course suggestive of an expanding mass lesion or infection. good
Glioblastoma multiforme left cerebral hemisphere good
a) MRI with IV gadolinium to increase the sensitivity of detection and
clarifying the site of origin of the tumor to confirm the diagnosis good As mentioned earlier, the mass effect of the lesion causes an increase in the intracranial pressure leading to papilledema and transtentorial herniation of the medial left temporal lobe leading to bilateral pupillary dilation. Herniation of the brain can also lead to hemorrhages in the brain stem that can lead to quadriparesis as seen in this patient. MRI scan will show us high resolution of the brain structure, localization and elucidation of pathological processes. Seizures due to disruption of the cortical neurons could be evaluated with an EEG (which may demonstrate a focal lesion.) Complications of treatment, in particular those relating to the steroid treatment would be monitored with follow-up physical exams and appropriate laboratory testing. good, on the long side The pathophysiology of a malignant astrocytoma is one of a neoplastic process. This process may begin due to a mutation in the DNA molecule, perhaps in a tumor suppressor, (e.g. p53) gene of an astrocyte leading to an abnormality in the growth regulation of the astrocytes. This unchecked growth leads to clonal proliferation of these malignant cells. Malignant astrocytomas/glioblastoma multiforme tumors carry a grave prognosis, irrespective of the selected treatment. The management of this patient should include emergent reduction of the intracranial pressure by performing a craniotomy along with corticosteroid therapy to reduce edema and inflammation. In addition, to limit the rapid growth of the tumor, radiation therapy should be initiated. Monoclonal antibodies have been used in the hope that they would serve as carriers, taking cytotoxic drugs, toxins, or radionuclides directly to the tumor site. The results of these trials are not as great as their expectations, however, due to the problems with access and delivery across the blood-brain barrier. Access may be improved by modification of the blood-brain barrier with mannitol or preliminary binding with liposomes to improve the passage of cytotoxic drugs and monoclonal antibodies to tumor tissue. The infiltration of the neoplasm disrupts the cortical neuronal connections and provides the opportunity for seizure activity. Monitoring the patientâs EEG activity and treating the patient with anticonvulsants to reduce the possibility of future seizures would be beneficial to her management care. good |
A 58 year-old right-handed woman presents with a 6 week progressive course of weakness right extremities with upper motor neuron signs, partial seizures starting with right hand which progressed to generalized seizures, bilateral papilledema, and expressive as well as receptive aphasia. She deteriorated over several months, with signs of increasing intracranial pressure, eventually progressing to coma. good right sensory and motor as well as language problems: left hemisphere seizures: indicates involvement of cortex: left precentral gyrus, hand area papilledema and altered mental state: obstruction of CSF flow, increased intracranial pressure, mass lesion good This is a subacute onset over weeks consistent with a neoplastic or infectious cause good
Left hemisphere neoplasm involving cortex
good
* MRI--mass lesion in left hemisphere, evidence of crossing of corpus
callosum good
* hydrocephalus, raised intracranial pressure leading to herniation,
alterations in consciousness good Tumors of the brain produce edema by altering permeability of vasculature, causing a mass effect and thus increasing intracranial pressure. Steroids have been shown to dramatically reduce the edema surrounding the tumor, leading to fewer complications. Exact management depends on the type of tumor as determined by biopsy, but in general surgical excision or radiotherapy, or a combination of both, is used to eliminate or reduce the tumor. Chemotherapy has been shown to prolong survival in malignant tumors, but problems with toxicity and penetration of the blood brain barrier have reduced its usefulness. good; note also likely role for underlying stem cell for origin of the tumor likely glioma; also likely role for oncogenes or tumor suppresor factors in initiation of tumor |