The 20-year-old man fell to the ground and convulsed, and after this examination was completed, it was caused by rheumatism?

*For medical professionals only

Foreword Summary:
A young man in his 20s suddenly falls to the ground and convulses, and is admitted
to the emergency department with a "generalized tonic-clonic seizure".

History: Patients complain of progressive pain in both lower extremities for 6 weeks, recent weight loss of 6 kg, with gait instability, nonspecific headache, and mild swelling
of the ankle.

The patient had asthma when he was a child and did not receive special treatment; There is hypertension, up to 180/115 mmHg, taking three oral antihypertensive drugs
.

The patient smoked 10 cigarettes a day, drank alcohol occasionally, smoked marijuana regularly, and had used cocaine
.

Physical examination: pale, cachexia, unclear
consciousness.

The lymphadenopathy of the neck and groin is enlarged and non-tender
.

There are several smaller, irregular, well-demarcated lesions
on the instep and outer ankle.

Neurologic body examination shows impaired dorsal flexion of both feet, high gait while walking, decreased pinprick sensation in the ankle, impaired proprioception of the bilateral distal interphalangeal joint, normal reflexes, and negative Rhomberg test
.

Test: the patient's blood picture is elevated and liver function is abnormal
.

In addition, urine protein is elevated, creatinine ratio is elevated, urine electrolytes are elevated, and random troponin is elevated (without dynamic elevation).


Vasculitis screening results: negative
.

HBV serology: HBsAg, HBcAb, HBeAg positive, suggesting acute active infection, HBV viral load up to 17300000 copies IU/ml
.

Table 1: Blood biochemical results on the first day of admission

In addition, patients tested positive for COVID-19 nucleic acid but had no symptoms
.

A lumbar puncture 3 days after admission to the hospital shows normal
cerebrospinal fluid.

Examination: CT of the head shows suspected bilateral encephalitis
.

Seizures
were not recorded on the EEG.

Nerve conduction studies show a decrease
in the amplitude of sensory and motor responses at the distal lower extremities.

MRI shows high T2 signaling in the frontal, parietal, occipital lobes, and multiple regions along the cortical spinal tract, cerebellum, and ponidium, and is consistent with the diagnosis
of reversible posterior encephalopathy syndrome (PRES).

Figure 1 Cranial MRI

CT angiography of the chest, abdomen, and pelvis shows multiple intrarenal aneurysms
.

Abdominal venous scan shows a bilateral wedge-shaped low-density area of both kidneys, consistent
with multiple bilateral renal infarction.

Figure 2 Abdominal CTA

The process of solving the case

After reading the case profile, we will notice that in addition to seizures, the patient's neurological symptoms include nonspecific headaches, impaired consciousness, and acryomotor dysfunction
.

Obviously, epilepsy is not enough to explain these phenomena
.

So what kind of disease will it be?
The results of the cranial MRI tell us that the patient meets the diagnosis of pres
.

As a result, encephalopathy and epilepsy are the most common symptoms of PRES, and patients are often unconscious, and about 60% to 75% of patients may have seizures
.

Therefore, it is feasible to use PPRES to explain the encephalopathy symptoms, seizures and acrificial sensation, and motor dysfunction of the
case.

However, the PRES still cannot explain the significantly elevated inflammatory markers and severe hepatitis B infection in this case
.
Don't forget, abdominal CT angiography also gave us an important tip: this patient also had multiple intrarenal aneurysms and corresponding renal infarctions! Considering the rapid weight loss of patients, pain in both lower extremities, and hepatitis B infection, is it enough to diagnose polyarteritis nodular arteritis (PAN)?
Figure 3 PAN diagnostic criteria meet at least 3 of the above 10 criteria
to diagnose PAN
.


Eventually, the patient was diagnosed with PAN, and PAS was one of
its manifestations.

PAN often affects the nervous system and kidneys

PAN is a small and medium-vessel necrotizing arteritis characterized by the absence of glomerulonephritis/ANCA positive
.

The disease is rare and the pathogenesis is unknown, most cases are idiopathic, and a small proportion are associated
with HBV infection.

HBV-associated PAN usually occurs within the first 4 months of infection and the mechanism is unknown
.


PAN is pathologically manifested by inflammatory vascular wall infiltrates and fibrinoidous necrosis, which tend to occur at arterial branch points
.

This process can lead to microaneurysm formation, thrombosis, and tissue ischemia
.

Angiography shows that microaneurysms (approximately 1 to 5 mm in diameter) with stenosis support PAN diagnosis
.
The clinical presentation of PAN is diverse, but it is worth noting that the rate of peripheral nervous system involvement is as high as 50% to 75% and is usually the initial clinical presentation
.

Inflammatory infiltration of blood vessels can lead to ischemia and mononeuritis polyneuritis, most commonly motor and sensory nerve involvement
.

As the disease progresses, this may evolve into acrylosymmetric sensorimotor neuropathy, as shown in
this example.
At the same time, the rate of renal involvement in PAN is also high
.

Renal involvement with multiple intrarenal aneurysms and resulting infarction is the most common manifestation of PAN
.

Renal hypoperfusion and infarction lead to activation of the renin-angiotensin-aldosterone system, leading to hypertension and proteinuria, but renal function may be normal, which is also consistent
with this case.
However, central nervous system (CNS) involvement in PAN is much less common than peripheral, occurring in 2% to 10% of patients
.

However, a case of CNS involvement indicates a poor prognosis
.

The most commonly reported CNS manifestations regarding PAN are diffuse encephalopathy, followed by PPRES
.

Navinan et al.
have reported a case of PAN-associated pres in a woman in her 20s, manifesting as seizures, visual impairment, and episodic hypertension
.

Case reports from Arai and Stanzani et al.
further point to PAN as the cause
of PAS in this setting.

In this case, the authors also support this claim
.
In CNS involvement in PAN, stroke is common, occurring in 13% to 17%, and most are secondary to lacunar infarction in deep vessel-thrombotic microangiopathy
.

The incidence of stroke is highest 6 months after the first appearance of PAN and is not related to
disease activity.

However, it is inconclusive whether antiplatelet agents should be used as a primary prevention in patients receiving cortisol-treated PAN
.

Case Breaking

PAN is a rare disease and it is difficult to accurately assess the manifestations of neurologic involvement or the risk of
developing CNS complications.

Therefore, for young patients with hypertension and PRES, it is necessary to undergo a full body examination and PAN screening, and when proteinuria occurs in the absence of nephritis, renal angiography
should be performed in time 。 References: [1] Heartshorne R,Nwe M,Barakat A,et al Polyarteritis nodosa presenting with posterior reversible leukoencephalopathy syndrome BMJ Case Reports CP 2022; 15:e250867.
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Source of this article: Medical Rheumatic Immunization Channel

The author of this article: Xiao Codeda

This article reviewed: Chen Xinpeng, deputy chief physician

Editor-in-charge: Tangerine

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