A 58-year-old man is referred , with worsening headache and cognition.
The patient was in his usual state of health until 4 months earlier, when he began to develop bitemporal headaches. Mild and intermittent at first, the headaches became progressively more severe and constant. Several over-the-counter medications, but with little effect. Two months later, he was admitted for persistent symptoms, which now also included mild neck stiffness, occasional nausea, and a burning sensation in his scalp. At this time, the patient noted that his headaches worsened upon coughing, sneezing, or bending over. He also felt that his thinking was "slower" and he complained of memory difficulties. In addition, he was sleeping up to 18 hours a day, which was a marked change from his usual 7 to 8 hours of nightly sleep. Magnetic resonance imaging (MRI) of the brain was read as normal. He was discharged .His symptoms continued to worsen during the next 2 months, at which point he was referred again.
Past Medical History
The patient has hypertension, chronic sinusitis, and a remote history of bleeding gastric ulcers.
Social History. He is a professional videographer, but unable to work currently because of his condition. He denies any tobacco, alcohol, or illicit drug use.
Family History: Multiple family members are known to have coronary artery disease and hypertension, but no family history of headaches, unexplained encephalopathy, or other neurologic conditions is known.
Medications: Currently takes clopidogrel, hydrochlorothiazide, lisinopril, hydromorphone, and prochlorperazine.
Review of Systems: The patient complains of a dry cough but denies any fever, chills, night sweats, or weight loss. He reports no contacts with other sick individuals and no recent travel outside his hometown. His one pet, a dog, passed away of "old age" several months earlier.
Physical Exam The patient is a tall, well-appearing gentleman who seems comfortable in bed. He is afebrile, with stable vital signs. normal temporal artery pulsations bilaterally, absence of oral ulcerations, no neck stiffness, no lymphadenopathy, and no rashes or splinter hemorrhages. Fundoscopic exam is unremarkable.
His neurologic exam is notable for his abnormal mental status. He is somnolent though quite easily arousable, and oriented to person and date but not to the location. Although he can register 3 objects, he does not recall any of them after several minutes despite prompting. His long-term memory appears to be relatively spared, but he cannot recall most of the events over the past few months, including the death of his dog. He follows multistep commands, albeit slowly; his speech, too, is slow but not dysarthric. The rest of his neurologic exam is unremarkable except for some mild bradykinesia throughout. He demonstrates no cogwheeling, tremor, or postural instability.
Imaging Studies
The patient was transferred to repeat MRI of the brain.
T1 sequence after gadolinium administration reveals diffuse pachymeningeal enhancement
Laboratory Investigations
Complete blood count, electrolytes, and hepatic panel were normal. TSH, B12, and ammonia level were unremarkable.ESR was normal, and the serum rapid plasma reagin (RPR) was negative, as were HIV, hepatitis, and Lyme serologies. Autoimmune investigations including antinuclear antibodies (ANA), anti-neutrophil cytoplasm antibodies (ANCA), anti-Ro/La, C3, C4, serum protein electrophoresis (SPEP), and urine protein electrophoresis (UPEP) were normal or negative.
Lumbar puncture demonstrated an opening pressure of 9 cm H2O. Cerebrospinal fluid (CSF) analysis revealed 3 white blood cells with 95% mononuclear cells, 0 red blood cells, glucose of 65 mg/dL, and a protein of 85 mg/dL. Gram stain and bacterial culture were negative, as were fungal and mycobacterial cultures. Viral polymerase chain reactions of the CSF were negative. CSF VDRL test and Lyme titer were negative. No oligoclonal bands were detected and the IgG index was normal. Cytopathology and flow cytometry were unremarkable
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