Neurology in Question

Chapter 2

Dr. James represents the second patient.

“Patient Michael Brad, Alex Brad’s father.”

Dr. James calls Marie.

“Marie, why did your husband not come?”

“He’s been diagnosed with Parkinsonism.”

“I heard Parkinson disease only occurs in old men.”

“No. His neurologist told him that it was probably caused by antiemetics. He developed Parkinsonism after taking them. He has bradykinesia, which makes him walk with small steps.”

Dr. James remembers his consultation with Michael.

“Sir, you have hypokinetic Parkinsonism. It is one of the main extrapyramidal syndromes. It is associated with dysfunction of the basal ganglia and dopaminergic pathways involving the substantia nigra. You have decreased facial expression, or ‘face figée,’ rigidity, and bradykinesia.”

“My baby’s emergency required a lot of running.”

“I get you. It must have been hard.”

The memory fades.

Back on the phone, Marie continues.

“My husband stopped the antiemetic, is taking L-Dopa, and is getting better.”

I am Dr. James, a humble neurologist at BS Hospital. This morning handover has concluded.

A 20-year-old woman comes to me holding her head, looking like she is in severe pain.

“It hurts so much. I puke a lot, too.”

I take her temperature using a non-contact infrared thermometer.

It shows 39°C.

“You have a fever. I suspect meningitis. Please lie down.”

I go to her right side.

As I gently flex her neck, the patient’s face wrinkles from the pain.

“She has neck stiffness. We need to investigate meningitis urgently.”

I turn to the extern beside me.

“Do urgent blood tests and blood cultures. If bacterial meningitis is suspected, we need to start appropriate empiric antibiotics immediately. If there is no contraindication to a lumbar puncture, we will perform one to analyze the cerebrospinal fluid.”

“Do we need a brain scan?”

“Only if she has an indication for brain imaging before the lumbar puncture, such as altered consciousness, a focal neurological deficit, papilledema, a new seizure, or significant immunosuppression. If imaging is necessary, we must not unnecessarily delay antibiotics.”

“What does the brain scan show?”

“The scan may show complications or another cause of her symptoms, but a brain scan alone does not diagnose meningitis.”

“And surgery?”

“Uncomplicated meningitis does not require surgery. We treat it with appropriate antimicrobial therapy and supportive care. Neurosurgery is only needed if there is a complication requiring surgical treatment, such as hydrocephalus or a brain abscess.”

The extern nods.

“Understood.”

I look at the patient.

“Let’s treat the emergency.”

The patient suddenly becomes confused.

Her skin develops dark purple patches.

“Doctor...”

I look at her carefully.

“She is developing sepsis. Those purpuric lesions are concerning for purpura fulminans.”

Her condition deteriorates rapidly.

“Start the emergency treatment. Give the appropriate empiric antibiotics immediately and begin fluid resuscitation. Monitor her blood pressure, oxygen saturation, urine output, and organ function.”

Ten minutes later, her condition suddenly worsens.

Her lips turn blue.

“Her SpO₂ is 80%!”

“She’s severely hypoxemic. Give her oxygen immediately.”

The nurse looks at the medication tray.

“Are you sure the injections you prepared contain the prescribed antibiotics?”

“No. They contain gentamicin and quinine.”

I freeze.

“What?”

“The new infirmary told me to prepare these.”

“Oh no. Those are the wrong medications for this protocol.”

I immediately check the prescriptions.

“This is the wrong medication. Stop it and prepare the correct antibiotics. Notify the senior doctor about the medication error immediately.”

“My bad! Should I check her blood pressure?”

“Please do.”

“Her blood pressure is 50/40.”

“She is in septic shock.”

Her temperature is still 39°C.

“She has a fever. Continue the resuscitation and monitor her closely.”

“What about surgery?”

“Not for uncomplicated meningitis. There is no inflammatory liquid that we routinely drain with surgery. Neurosurgery is only necessary if a complication such as hydrocephalus, an abscess, or another structural lesion requires it.”

The extern nods.

“Understood.”

The next patient arrives.

Serene, 40 years old, walks with a cane supporting her right leg. Her pupils are bilaterally dilated.

She was brought to the neurology emergency department after being hit by a car.

“She’s incompatible with life,” a first-year resident says.

I pinch her hand.

She moves it.

“She responds to pain. We cannot determine her Glasgow Coma Scale from this finding alone, but she is clearly not completely unresponsive.”

I look at her eyes.

“Could she be conscious?”

Her cousin answers.

“She visited an ophthalmologist before the accident.”

“No wonder.”

“What do you mean?”

“He probably administered mydriatic eye drops. They can temporarily dilate the pupils and cause blurred vision and photophobia. Bright light can make the visual discomfort much worse. The bilateral mydriasis does not mean that she is comatose.”

“So she’s not blind?”

“Her visual symptoms may be reversible if they are caused by the mydriatic drops, but we need an ophthalmological examination to determine that.”

I turn toward the interns.

“We can’t assess her gait yet. Her right leg could be fractured or injured, which would interfere with the neurological examination.”

I turn toward Serene.

“You don’t appear to be comatose. I’m going to write a referral for orthopedics. You’ll have a leg radiograph to check for a fracture. The orthopedic doctor will assess and treat the injury. Then you’ll come back here so I can assess your gait.”

The orthopedic doctor, Dr. Mark, examines her.

“Madam, your leg is not fractured. However, you have a ligament sprain around the knee.”

He examines the knee carefully.

“Take paracetamol 1,000 mg every six hours if you have pain, provided you have no contraindication. Do not exceed the recommended daily dose.”

He gives her a knee brace.

“Here’s a brace for your knee. Wear it as instructed.”

“How soon? The neurologist said he’ll reassess my gait afterward.”

“About 15 days should be enough for an initial reassessment, depending on your symptoms. Follow the instructions and come back in 15 days.”

Fifteen days later.

Dr. Mark moves her right leg back and forth, then sideways.

He rotates it carefully.

“Does it hurt?”

“No.”

“Try walking on it without the cane.”

She walks.

“I don’t feel pain.”

“It’s reassuring. You can go see the neurologist now.”

He pauses.

“By the way, did you notice any swelling or pain in your legs?”

“Yes, on my thighs. I was afraid to walk with the cane when I was having trouble seeing, so I mostly lay down.”

“You’ve been immobile for fifteen days. We need to rule out deep vein thrombosis. I’ll request a compression ultrasound of the leg.”

Later, Dr. Mark calls me.

“Her ultrasound shows a deep vein thrombosis.”

“Start therapeutic anticoagulation.”

“Lovenox?”

“Yes, if there is no contraindication. It must be given by subcutaneous injection, and the dose is weight-based. We don’t use a fixed 250 mg twice-daily dose.”

“Do we need an INR?”

“Not to monitor enoxaparin. If we use warfarin, then we monitor the INR. For a patient treated with enoxaparin alone, INR is not the test we use to measure its anticoagulant effect.”

“Understood.”

Serene returns to the neurology department.

“You don’t have mydriasis anymore. See? It was probably due to the mydriatic drops. We can assess your gait now.”

The interns gather around me.

“Before we examine her gait, let’s clarify something.”

I point toward Serene.

“If a patient is already unsteady with the eyes open and remains unsteady when the eyes are closed, think about cerebellar ataxia or another cause of gait ataxia. Cerebellar ataxia is not corrected simply by visual input.”

I continue.

“In a pyramidal syndrome, we look for weakness, increased muscle tone, hyperreflexia, and pathological reflexes such as a positive Babinski sign.”

I raise my finger.

“And remember: a positive Babinski sign is extension of the great toe, often with fanning of the other toes. Downward flexion of the toes is the normal response.”

The residents nod.

“Falls mainly when the eyes are closed suggest a positive Romberg test. That points toward sensory ataxia, particularly impaired proprioception, although vestibular disorders can also produce a positive Romberg sign.”

I look at Mira.

“But don’t automatically call it Chagas disease. Chagas disease is an infection caused by Trypanosoma cruzi. It is not simply another name for proprioceptive loss.”

Serene looks confused.

“My brother has Chagas.”

“That is relevant to your family history, but we still need evidence that you have the infection. Your gait abnormality could have many causes.”

I ask her questions.

“Where do you live?”

“A. State.”

“Does your family have any medical history?”

“Mom has diabetes. Dad has hypertension. My little brother has Chagas. My maternal uncle has COPD and pneumothorax. My paternal aunt has atrial fibrillation and a mechanical valve after having mitral stenosis.”

“Any medications you’re taking?”

“Mydriatic eye drops and antihistamines for rhinitis.”

“Any surgical history?”

“I had my gallbladder removed. Here.”

She gives me the document.

“Okay. Any illnesses you had before?”

“None, except for my eyes.”

I examine her gait.

“Did you notice her swaying back and forth with her eyes open?”

“Yeah.”

“You’re the resident in charge of her, Mira. Ask her.”

Mira turns toward Serene.

“Do you feel vertigo?”

“Sometimes.”

I intervene.

“Good question. But remember that vertigo is a vestibular symptom. It is not a sign of proprioceptive loss.”

I examine her eye movements.

“She has horizontal nystagmus.”

The intern looks at me.

“Is that always a central lesion?”

“No. Horizontal nystagmus can occur in both peripheral and central vestibular disorders. The direction, whether it suppresses with visual fixation, associated neurological signs, and other features matter. Vertical nystagmus is much more concerning for a central lesion.”

I turn to Serene.

“Stand with your feet together.”

She stands.

“Now close your eyes.”

She begins to sway.

“She’s falling!”

I hold her.

“That’s a positive Romberg sign.”

“So she has a pyramidal disease?” a first-year resident asks.

“No. A positive Romberg points toward a sensory or vestibular problem, not specifically a pyramidal lesion.”

Mira examines the plantar response.

She strokes the lateral sole of the foot.

All the toes flex downward.

“Her Babinski sign is negative.”

“Correct. That’s a normal plantar response. But a negative Babinski alone does not prove that she has a peripheral nervous system lesion.”

I turn toward the interns.

“Neurological examination is about combining findings, not assigning a diagnosis to one sign.”

Serene looks at me.

“Do I have Chagas disease?”

“We need to investigate the cause of your sensory ataxia first. If Chagas disease is suspected because of your history or epidemiological exposure, we will order the appropriate tests.”

“How do we treat Chagas disease?”

“Treatment depends on the stage and clinical manifestations. Antiparasitic treatment uses drugs such as benznidazole or nifurtimox. Benzodiazepines and L-Dopa do not treat Chagas disease.”

The resident looks surprised.

“And L-Dopa?”

“L-Dopa is used mainly for Parkinsonian symptoms. It does not treat a peripheral nerve lesion.”

Serene suddenly shifts in her chair.

“Is something wrong?”

“My butt hurts.”

“What type of pain?”

“Electrical discharge.”

“It’s neurologic. Where does it start and where does it extend to?”

“It starts in my back and goes down my buttock, posterior thigh, posterior calf, lateral foot, and little toe.”

I nod.

“That distribution is compatible with an S1 radicular pattern.”

“What caused it?”

“It could be related to a spinal cause, such as nerve-root compression. We’ll evaluate it clinically.”

“Corticosteroids?”

“Systemic corticosteroids are not routinely recommended for uncomplicated sciatica because their benefit is limited. We’ll treat the pain appropriately and investigate further if necessary.”

“Do you drive?”

“No, but it started after I lay down for the last fifteen days.”

“That’s relevant. Did your orthopedic doctor request a Doppler ultrasound?”

“Yes.”

“Good. Tell me the result.”

Later, Dr. Mark returns with the ultrasound.

“It shows a deep vein thrombosis.”

“Continue therapeutic anticoagulation as prescribed. No need to repeat an INR if she remains on enoxaparin alone.”

“Understood.”

One month later.

Serene returns.

“My leg feels much better.”

Dr. Mike examines her.

“Can you walk?”

“Yes.”

“Can you run?”

She runs a few steps.

“All the better.”

I examine her eyes again.

“The nystagmus is gone.”

I assess her gait.

“Your gait is much better. Now let’s repeat the Romberg test.”

Serene stands with her feet together.

“Close your eyes.”

She remains standing.

The interns stare.

“I didn’t need to hold you.”

Serene chuckles.

“You stood by yourself.”

The next patient is a 45-year-old woman.

She complains of headaches that occur around her menstrual period.

She also reports hot flashes.

“Your headaches may be catamenial migraines. At 45, hot flashes can also occur during perimenopause.”

Suddenly, her headache changes.

“It started brutally. It is the worst headache I’ve ever had.”

I become concerned.

“She needs urgent imaging.”

The CT scan is performed without contrast.

It shows hyperdensity in the basal cisterns and around the Circle of Willis.

“This is a subarachnoid hemorrhage.”

The extern looks at the scan.

“Isn’t that a meningeal hemorrhage?”

“Subarachnoid hemorrhage is bleeding into the subarachnoid space. The subarachnoid space lies between the arachnoid mater and pia mater.”

I point at the scan.

“The blood can collect around the basal cisterns and the Circle of Willis.”

“How is the Circle of Willis formed?”

“Two anterior cerebral arteries are connected by the anterior communicating artery. The internal carotid arteries give rise to the anterior and middle cerebral arteries. The posterior communicating arteries connect the internal carotid circulation to the posterior cerebral arteries. The basilar artery is formed by the two vertebral arteries and then divides into the posterior cerebral arteries.”